Immune thrombocytopenia (ITP) is a disease caused by the destruction of platelets or thrombocytes by the immune system itself. It is therefore an autoimmune disease.

There are different levels of severity and symptoms of ITP depending on the platelet count. The disease is characterised by an increased risk of bleeding. Platelet levels of between 150,000 and 400,000 per microlitre are considered normal.

When these levels decrease temporarily or persistently, without known triggering factors, then we speak of primary ITP. If the platelet count is below these levels, but above 50,000, it is normally asymptomatic and does not cause bleeding. In these cases, the disease will be difficult to diagnose.

If the platelet count is between 30,000 - 50,000, small hemorrhages often occur when there is trauma, blow, etc. If the count is between 10,000 - 30,000, petechiae, metrorrhagia, epistaxis, gingivorrhagia, etc. may occur spontaneously.

In this situation we speak of moderate thrombocytopenia. If the levels fall below 10,000 platelets per microliter, we are dealing with severe thrombocytopenia in which internal bleeding can occur in vital organs with serious consequences.

The origin of primary immune thrombocytopenia is unknown; although there are other forms of secondary thrombocytopenia associated with certain diseases, infections or the taking of certain medications.

Why does immune thrombocytopenia occur?

The decrease in platelet levels is due both to an increased destruction of platelets in the blood and to a defect in their production in the bone marrow. People who suffer from this disease possess certain antibodies, usually of the IgG type (although in some cases IgM and IgA are also involved), which are capable of recognizing certain proteins on the surface of these cells and marking them as if they were foreign elements to the body itself (antigens).

Consequently, platelets and their precursors in the bone marrow are recognized and eliminated by cells of the immune system, such as macrophages.

What precautions should a person with TPI take?

Physical activity should be controlled and free of dangers such as risky or contact sports, which can cause trauma. It is advisable to ask the doctor which activities should be avoided according to the person's lifestyle.

There are medications that can alter platelet activity, which is why it is always a good idea to ask your doctor before taking any medication.

Keeping the immune system in good condition is important to ensure that its response is balanced and that hypo and hyperactivity situations do not occur. In this regard, microimmunotherapy can be a support within a global strategy for treating immune diseases.

Bibliography

1. Karam D. Echevarría S. Diagnosis and treatment of immune thrombocytopenic purpura. Clinical practice guide. Mexican Social Security Institute.
2. Mayo Clinic. Idiopathic thrombocytopenic purpura. Patient Care and Health Information. Diseases & Conditions. Available at: https://www.mayoclinic.org/diseases-conditions/idiopathic-thrombocytopenic-purpura/symptoms-causes/syc-20352325.

  Rheumatoid arthritis (RA) is a chronic autoimmune disease characterized by inflammation of the joints. It is sometimes confused with osteoarthritis, but they are different inflammatory diseases.

It affects women to a greater extent, possibly due to the influence of estrogens in the generation of inflammation. In addition, women are more susceptible to autoimmune diseases due to methylation of the X chromosome. There is also a certain heredity with regard to connective tissue diseases, so having a mother with a history of the disease is a risk factor.

Rheumatoid arthritis causes pain, stiffness, swelling, and can even cause joint deformity and loss of normal joint movement. It can also be accompanied by fatigue, occasional episodes of fever, or other symptoms such as heart complications, osteoporosis, or lung complications.

The exact causes of this disease are still not fully understood, although it has been shown that the immune system plays a key role in its development and progression. A combination of genetic and environmental factors triggers an abnormal immune response that results in the production of autoantibodies and chronic activation of the immune system. Let's see what happens to the immune system.

The Adaptive Immune Response in Arthritis

The adaptive response  plays a crucial role in this joint disease. Both B cells and T cells, specialized lymphocytes, are altered and are key in the development of the pathology.

Specifically, an increase in CD4+ T cells and a decrease in Regulatory T cells, responsible for maintaining tolerance and preventing immune reactivity to structures and tissues, have been observed in the affected joints. CD4+ T cells produce proinflammatory cytokines such as interleukin 6, tumor necrosis factor alpha (TNF-α), and interferon gamma. All contribute to chronic inflammation and joint destruction.

B cells also play an important role. Immune complexes consisting of autoantibodies and antigens have been found in the joints of people with this disease. Especially B cells producing anti-citrullinated peptide antibodies (ACPA), are closely associated with disease severity and joint destruction in RA.

In addition, B cells can activate T cells and give rise to a vicious circle that perpetuates the inflammatory response.

Proinflammatory environment and cytokines in arthritis

Chronic inflammation is a hallmark symptom of rheumatoid arthritis. The synovial inflammation typical of arthritis has been shown to be mediated by excessive production of proinflammatory cytokines. These cytokines include IL-1β, IL-6, TNF-α, and IL-17, among others.

They promote the migration of inflammatory cells to the joints and stimulate the production of enzymes that degrade cartilage and bone. TNF-α is one of the most studied cytokines in this disease and has been identified as an important therapeutic target to reduce inflammation and disease progression in these patients.

Solving the Immunological Puzzle

Rheumatoid arthritis is a complex autoimmune disease that involves a dysfunctional adaptive immune response and chronic inflammation of the joints. It must be treated by specialists to prevent it from progressing to other locations and to maintain joint functionality.

Many of the available treatments usually help to relieve symptoms, although they do not always treat the root of the problem. In this regard, it is also worth considering approaches that allow the mediators involved to be modulated simultaneously, trying to redirect the immune response both locally and systemically, so that it works optimally, depending on each person's situation.

Immunomodulatory treatments are an option to consider in this case, since they can offer complementary support to symptomatic relief, helping to rebalance inflammatory signaling.

This is precisely the goal of microimmunotherapy. Doctors who use it in patients with rheumatoid arthritis seek to counteract the deregulation that exists in the inflammatory response and its persistence, in order to limit the deterioration of the affected tissue.

It should be noted that microimmunotherapy is based on the use of the same type of molecules and immunological mediators that the immune system uses, in low and very low doses, to work from a physiological point of view, coming as close as possible to the natural functioning of the body.

For example, it can be aimed at down-regulating the action of cytokines such as IL-1 or IL-6, involved in the inflammatory reaction.

In the end, in rheumatoid arthritis as in other autoimmune diseases, it is not just a matter of slowing down immunity, but of returning it to a state of balance.

Bibliography

1. Miguel-Lavariega D, Elizararrás-Rivas J, Villarreal-Ríos E, Baltiérrez-Hoyos R, Velasco-Tobón U, Vargas-Daza ER, Galicia-Rodríguez L. Epidemiological profile of rheumatoid arthritis. Rev Med Inst Mex Seguro Soc. 2023 Sep 4;61(5):574-582. Spanish. doi: 10.5281/zenodo.8316427.
2. van Delft MAM, Huizinga TWJ. An overview of autoantibodies in rheumatoid arthritis. J Autoimmun. 2020 Jun;110:102392. doi: 10.1016/j.jaut.2019.102392. Epub 2020 Jan 3.
3. Radu AF, Bungau SG. Management of Rheumatoid Arthritis: An Overview. Cells. 2021 Oct 23;10(11):2857. doi:10.3390/cells10112857.

 The term lupus means "wolf" in Latin (in German "wolf"). In consulting the medical writings of the ancients, it was noted that this term was used to characterize various skin conditions whose marks are reminiscent of wolf bites. It appears for the first time in medical literature in 916 AD concerning the illness of the Bishop of Liège, Eraclius, lupus, from which he was miraculously cured on the occasion of a pilgrimage to the tomb of St Martin in Tours.

History of Lupus

The observation is reported by Herbert of Tours: "He Eraclius, whom he calls Hildricus, suffered from an ulcerous disease, lupus, which manifested itself by a red line on the forehead." This type of skin condition certainly did not go unnoticed by ancient physicians. The frescoes of ancient Egypt give a fairly accurate reflection of the skin condition of the inhabitants of the Nile Valley. The study of medical papyri, dating from 1000-1700 BC, has made it possible to identify the semiological descriptions of many skin diseases.

Hippocrates, who lived on the island of Kos between 460-375 BC, described ulcerative lesions of the skin that he called Herpes Esthiomenos (Herpes spreading on the skin, Esthiomenos gnawing). Claudius Galen, a Greek physician practicing in Rome between 131-201 AD, used the name Herpes in a less vague sense to designate superficial ulcerations of the skin.

Persian physicians such as Raazes (900 AD) and Avicenna (1000 AD) described the condition under the name "formica corrosiva" which was retained by Paul Aegina, a Greek physician from the island of Aegina, and by Galen. In the writings of the famous School of Medicine of Salerno of the Middle Ages, we find the writings of Rogerius Frugarti who describes lupus characterized by swellings of the extremities. Rolandus devotes the term "noli me tangere" (do not touch me) for diseases affecting the face.

Bernard of Gordon, physician of the School of Montpellier (1305) also mentions the ulcerative form of herpes which he calls lupus. In 1500, Paracelsus introduces the term consolida lupi to describe a disease different from estthiomenos, fistula and cancer. He thus uses the term lupus vorax. Girolamo Mercurialis gives a description of lupus in his treatise "de Morbei-Cutanei" of 1572, the first work of dermatology published in Europe.

In 1750, the name lupus appeared for the first time in an American medical work "A method of Physics" by Philips Barrough. The use of the word lupus will be reserved for red eruptions of the face by Hans van Gersdorf of Strasbourg in 1577 and taken up by Jean Dolaeus in 1684.

In 1790, the British Robert Willan established a first classification of skin diseases in which we find the description of lupus which he clearly separates from "noli me tangere" and herpes. Willan wrote the first atlas of dermatology "Manual on Skin Diseases" which includes many color illustrations entirely drawn by hand, which will greatly delay its publication.

In 1808, a new edition was published in which Willan reserved the name lupus for a nodular eruption of the face that was complicated by ulcers. Two types of lupus were described, tuberculous lupus and lupus vulgaris. After Willan's premature death, his student Thomas Bateman continued his work. A new atlas of skin diseases was published in 1810 that would influence the history of dermatology. In France, the St Louis hospital in Paris developed a dermatology department in 1801, the direction of which was entrusted to Jean-Louis Alibert.

In 1832, he published a work entitled "Descriptions of Skin Diseases observed at St Louis Hospital" with 50 color engravings. In it, he describes "tartars" within which he isolates the reddening scab corresponding to the condition described by his predecessors under the name of lupus. Alibert specifies that esthiomene corresponds to Paracelsus' lupus vorax and Willan's lupus which are associated with tuberculosis.

In 1815, Alibert left the service of the St Louis hospital to Laurent Théodore Biett, a doctor of Swiss origin, who had studied dermatology with Bateman in London and who would therefore apply the classification of dermatoses according to his English masters. His collaborators, Alphée Cazenave and Henri-Edouard Schedel published in 1828 the first edition of the "Practical Abbreviations of Skin Diseases" in which the authors separated different forms of lupus: lupus which destroys on the surface, which destroys in depth and lupus with hypertrophy. They demonstrated that "noli me tangere" is of cancerous origin and therefore clearly separates this condition from lupus.

In the second edition of 1833, the chapter devoted to lupus is supplemented by a particular form which is described under the name of "Erythema centrifugum".

In 1851, Cazenave extended the description of centrifugal erythema by noting skin lesions with atrophy, telangiectasia, fixed erythema and he modified the name to "Lupus Erythematosus". Work on lupus then continued in Vienna, a center of Austro-Hungarian medicine. Ferdinand von Hebra was put in charge of the Dermatology clinic in this city in 1841.

In 1846 he described a condition affecting the face that he called "seborrhea congestiva". He described the very particular appearance of the malar rash as a "butterfly wing".

In 1866, Von Hebra would specify that the condition was identical to that which Cazenave had described under the name of Lupus erythematosus. He therefore rallied to Cazenave's thesis and definitively accepted this term which would be universally retained. The first illustration of lupus erythematosus appeared in 1856 in Von Hebra's Atlas of Skin Diseases which included numerous hand-painted illustrations by his collaborator, the Swiss Anton Elfinger.

In 1866, a young Hungarian doctor, Moriz Kohn, joined Von Hebra's service. A brilliant doctor, polyglot, talented orator, Kohn would perfect the teaching of his master Von Hebra.

In 1869, he published his first article on lupus erythematosus.

In 1871, Moriz Kohn obtained permission to change his surname to KAPOSI and it was under this identity that he continued to publish numerous works.

In 1872, in a detailed treatise, he described the existence of two types of lupus: discoid lupus, exclusively cutaneous, and a disseminated form associating systemic visceral complications including subcutaneous nodules, arthralgia, lymphadenopathy, fever, weight loss, anemia. He called this form "disseminated and aggregated lupus erythematosus". Confusion arose about the adjective disseminated which is related to the cutaneous evolution and not to the multivisceral (systemic) character of the condition.

In 1902, Sequira and Baleau in London published a review of 71 cases of lupus, including 60 discoid and 11 disseminated. They noted the existence in this latter group of a frequency of acroasphyxia (which would later be better known as Raynaud's phenomenon), renal damage as well as pleurisy (pericarditis. Jadasshon, a German dermatologist practicing in Berne, Switzerland, contributed in 1904 to the replacement of the term "disseminated lupus erythematosus" by that of "systemic lupus erythematosus" or better "lupus disease". Sir Williams Osler would confirm the concept of systemic lupus thanks to numerous publications between 1895 and 1904. In 1936, CK Friedberg described the existence of lupus disease without cutaneous manifestations.

Systemic lupus seems to have existed since ancient times. Indeed, a group of researchers under the direction of Marvin Alison and Alejandro Pezza from the Inca Museum in Peru were able to carefully examine a mummy of a 14-year-old girl, dating from 890 BC, whose examination revealed alopecia, pleurisy and pericarditis, glomerulonephritis compatible with systemic lupus. Reproductions of lupus disease are also found in paintings by old masters.

Thus, Rembrandt in 1634 painted the portrait of Maria Bockenolle, the wife of Pastor Elison, in which one can notice the red rash on the face and the joint deformation of the hand.

At the Louvre Museum, one can admire the painting by the French painter Simeon Chardin from 1740, a copy of which from 1746 is in the Hermitage Museum in St. Petersburg, the "Benedicité" on which one can observe the erythema of the little girl's face.

The 20th century opened the era of lupus biology. In 1910, Hank reported the positivity of the Wasserman complement fixation reaction with the serum of lupus patients. In 1846, Hargraves discovered, in the sternal marrow of lupus patients, the presence of particular cells consisting of neutrophil polymorphonuclear cells having phagocytosed the nucleus of another cell which were called LE cells. The following year, Haserick showed that the serum of lupus patients was capable of causing the formation of LE cells with cells from the marrow of normal subjects.

In 1954, Peter Miescher, a Swiss immunologist, succeeded in absorbing serum factor LE with thymus cell nuclei, thus demonstrating that the factor was an anti-nuclear antibody.

In 1957, it was shown that these antibodies reacted with nucleoproteins, that is, the constitutional subunit of chromatin. The same year, Maxime Seligman in Paris showed that the serum of lupus patients causes a precipitate with DNA. This was confirmed by the German Deicher in Henri Kunkel's laboratory and by the Italian Ceppelini. Thus anti-DNA became the specific serological markers of lupus. An important discovery was to revolutionize the practice of immunology.

This is the development by Coons in 1953 of the immunofluorescence technique.

Friou applied it in 1957 to the search for anti-nuclear antibodies but it would not become widespread until 1968 when the first microscopes equipped with UV lighting became widespread in laboratories. From 1975 onwards, the search for anti-nuclear antibodies was carried out on Hep-2 cells, a substrate still used today.

In 1961, Anderson in Glasgow, will show that the antibodies present in the serum of lupus patients precipitated soluble extracts of thymus nuclei. This is the start of work on anti-ENA antibodies for "Extractable Nuclear Antigens", i.e. soluble antigens of the nucleus. In 1966, Tan identified in a lupus patient, Mrs Smith, a first anti-ENA, which was called anti-Sm. Other specificities will be identified in the following years. The sophistication and standardization of anti-nuclear antibody detection techniques will lead to genuine diagnostic progress in lupus disease.

Dermatological lupus has been the subject of treatment attempts since antiquity. At the beginning of the 15th century, the German physician Johan Tollat ​​von Vorchenberg wrote "for lupus caprifolin", that is to say honeysuckle. Medicine at the time used a very rich pharmacopoeia borrowed from the plant and mineral kingdoms. Jonathan Hutchinson, 1880, proposed cod liver oil, arsenic, zinc chloride and mercury nitrate. Anderson added the application of iodine. Gold salts were introduced in 1913 for the treatment of discoid lupus. For that of systemic lupus, quinidine was introduced by JF Payne in 1894, aspirin was used in 1899 by Radcliffe-Crocker and quinine by Mac Lead in 1908.

In 1956, plaquenil was introduced, which is still widely used today.

In 1935, Edward Kendall isolated cortisone, which was used to treat lupus by Hensch. From 1952, immunomodulators such as cyclophosphamide, mycophenolate, azathioprine, and then monoclonals such as rituximab were used. Other therapies are under development.

The first descriptions of lupus focused on dermatological manifestations. Various varieties of cutaneous lupus have been described by dermatologists.

At the end of the 19th century, it was realized that some lupus could be complicated by diffuse visceral manifestations and the dermis of systemic lupus erythematosus replaced that of disseminated lupus erythematosus. Thanks to the development of our knowledge in immunology, it was demonstrated at the end of the 20th century that lupus disease is an autoimmune disease. Immunological tests were developed that allow a precise diagnosis of systemic lupus. On the other hand, the knowledge acquired about the etiology of the disease will allow the implementation of increasingly effective targeted therapies.

Prof. René-Louis HUMBEL

Laboratory of Immunopathology, Luxembourg

BIBLIOGRAPHY:

• 1. Virchow R. Historical Notes on Lupus. Arc Pathol Anat 1865; 32:169-143.
• 2. Russel B. The History of Lupus Vulgaris: Its recognition, Nature, Treatment and Prevention.Pro Royal Soc Med 1954; 48: 127-132.
• 3. Smith CD The History of Lupus Erythematosus: from Hippocrates to Osler.Rheum Dis North Am 1988; 14:1-14.
• 4. Hoshberg MC. The History of Lupus Erythamotosus. Md Med J 1991; 40:871-873.
• 5. Gamarra A. An historical review of Systemic Lupus Erythematosus in Latin America.Med Sci Monit 2004; 10:171-185.
• 6. Mallavaapu RK, Grimsley EW. The History of Lupus Eythematosus. South Lad j 2007; 100:896-898.

I am a student, I work at night for the town hall of my city, with kids in "school failure". In 2 weeks, Saturday, May 29, we have a party, a kind of carnival, where we are supposed to parade, dance, walk in the streets of the city, from 12h to 19h.

Big problem: how am I going to do? How can I keep more than 1 hour to walk? From experience, I know it's useless to talk to my boss, because I work with one of my friends, who was supposed to know since 2002, when I told him about it, that I suffer from an LED. However, she "forgot" it, and when it was about a month ago she asked me what I had in the face (mask of the wolf), and that I replied that it was a lupus, she looked surprised that I did not speak to her before. Thank you for being my friend, it's great (pseudo) yupi friends!

So talk to the superior, even if I get along well, so that he understands nothing: no thank you.

People in general react to me as my father: According to him, I am in good health because: I get up in the morning, I shower, I feed myself, I have a social life. He does not understand anything, although he has been aware of my state of health since 1996. For him and for many people, since I found a normal platelet count following my removal of the spleen : all is fine But we know that no. The purpura was just one problem among many others, the e having platelet today, does not make me a miracle lupus.

May 29, I'm going to be broken, KO, crumbling, this fucking shit Saturday that's already getting on my nerves, because I know I'm not going to hold more than 1h, will sign my death sentence, oh yes it's going to be unsustainable, I can already see the back farting, limping, with my bones that will crack everywhere, ohlala...!

I have a cane, I use it from time to time, but I have never used it before them. I am afraid that my position and my skills will be challenged with respect to my health, especially that in June, we will have a meeting to find out who is left, and who will leave next year. I would be in my last year of BTS, a job of 2h / evening would be top, just to make me money but move a little too, but if they realize how much I can be limited in my movements, they may not want me anymore :(

It is unfair that the disease is so misunderstood. I do not even mention the fact that no one knows it, but those who know it, like my father for example, or some of my friends, ignore it. They have nothing to wank! (It's not vulgar! It's totally realistic, and therefore justified.

News and a sport to practice, even for us!

It is said that money is not happiness and it is true! Nothing would make me happier than spending a whole day in good health: by that I mean without pain or fatigue. It happens to me from time to time, but it's so rare! Once or twice a year...

Ahh when I hear some people say: "I'm tired, I spent the day to move in all directions, I'm exhausted", how I would like to be tired too for the same reasons and tired for doing things, and not dead "for nothing"! 

I have no friends, no one to talk to, talk about futile things like most girls of my age, no girlfriends with whom to talk to dudes etc. I'm not bad, I'm not so bad that nobody wants me. So what? The girls I was hanging out with do not want to go to school anymore because I have become too much of a watcher for the guys on the street, since I went from a 44 to a 36 (and 60mg of corticoids / day, at 2mg). I've "melted", and it bothers them: "Do you think you're at the beach to put on a tank top?", "Do you believe where to put a dress?". Frankly, if it is to hear such comments completely derogatory and useless, I still prefer to stay alone. I would say that it's people to whom I explained my health problems six years ago, and that except "and that's it, it's nothing like that!", I'm entitled to nothing other. Today, they do not even remember it.

You do not find that a little bit abused? lol it's stupid and foolish to be rejected because we're 55kg, whereas when I was 75kg, everyone liked me. My character has not changed so far, I am even more friendly and open today.

I'm ready to "lie", basically not to shout, to walk "properly" in public, people are stupid and suddenly are embarrassed by some attitudes that I could have , I never complained to my friends. But it does not happen! I must believe that I am not made to befriend people!

If I can not have friends, I must find myself a new occupation, something: cool, not tiring, which is an interesting minimum (at least for me). And I found! Fast no!

Did you see the ad for this new style of golf, the street golf, made in Decathlon? I cracked for 2 things: it's colorful lol, and: no need to bend down, nor farting back : just take golf ball with the club: there's a place provided for this purpose, nickel for people like us lol.

It's called "Ygolf". I tested, and I honestly found it fun, fun, and there are two clubs (to play 2), 4 balls, and a target / hole. It is light lol other good point, it is transported without being burst so far. Play this, in a large park with hills, in the early evening when it is always a bit warm, but the sun starts to leave: it is super nice and relaxing. It is a style of golf made to relax and laugh, very far from the classic golf! 

No need for superhuman strength, the balls are not rigid, the clubs are light, the carrying bag is practical. In short, I am happy, I will be able to practice a funny sport: street golf, within the limits of my state of health of course! 

I who complained a while ago of not having an occupation, I found one, which replaces a lot of useless things, and which fills my free time. If you have other ideas, just as tiring, I listen to you :-)

Back to school after the holidays was not easy! I missed a lot of my blog, and also my facebook page. In fact, I'm pushing, suddenly, I'm sick H24. For 1 month, I did not stop coughing, coughing. Accompanied by fever (once a week, it's accurate lol) and cramps with or without fever. Currently, the cough is almost gone, the fever less rarely (once a month), but the pain is more present than ever. About once a week I am stuck with pain, I can not even move a finger! If I lie down, only I can not get up. If they help me up, I walk like a granny, step by step, back all bent and it's far from glamorous! 

And it's a big vicious circle: I lie down to rest, but the more I rest, the more it gets worse. On the contrary, if I try to move, walk, it passes over the hours alone. But you have to have a lot of courage to get up and move while suffering martyrdom.

And if no one sees that I am alone, in my room, in the vegetable state that does not move, well I can spend 24 hours without giving news and no one worries XD! These months it's not going at all! So I'm hyper late course and homework, I missed the job too, it's pretty chaotic! 

I had not really had a big push since 2004, and here I must say that I am served! When I'm in remission I do a little anything. I go out, I'm having fun, I sleep if I have the time or so much worse, I rest more, basically I do not care at all : - / ben, we pay dearly after -_- "it will teach me..!!! I hope that you are well, that there are not too many thrusts in the air ;-) Give your news!

It's all arranged...

Since the beginning , I chained: a cold that lasts 2/3 months, an otitis that lasts 1 month, and more recently, a loss of 8 kgs in 10 days, I hallucinated. Things seem to be better now: otitis almost gone, the cold causing me more fever, I do not care, and I lose more weight! not at the rate of almost 1kg / day lol anyway!

That was the news "health"! In a completely different field: school, studies are going well. But it's a year more than stressful: exams in May loool I still have not 5 months of revisions ... finally manage to combine homework (there are 30 in all, I have already returned 13. ..) + internship + reviews + my job and it's not going to be easy! 

For the moment I'm just homework + job loool down revisions in the middle of December, and the internship, I should start it (if all goes well) from January: creation of an event (neighborhood party ) + creation of a "mini-journal". I would say more next time ;-)

Soon the holiday season: my friends, I would be absent from Facebook from Friday, and until next year lol January what: p because I'm going to Lisbon, for the end of the holidays year, even in case of snow, I think my flight should not be canceled, priority being given to departures. Departures on airports not "blocked" by the snow 

Systemic lupus in children and adolescents in ten key points: Editor: Dr. B. Bader-Meunier, Reference Center "Juvenile Arthritis", Necker Hospital, Paris) Editors: Drs Quartier (Reference Center "Arthritis Juveniles", Paris), Dr. Ranchin (Pediatric Nephrology, Lyon)

1 What is Systemic Lupus? How is Lupus treated and supervised in children and adolescents?

SLE is an autoimmune disease that can affect one or more organs, especially the skin, joints, blood cells and kidneys. SLE is a chronic disease which means it can last a long time. Autoimmune means that it is a disease of the immune system that will cause an attack on the patient's own organs. Systemic means that it can reach several organs of the body. The word lupus comes from the Latin word wolf because of the characteristic aspect of the cutaneous involvement of the face with a red plaque in the shape of the mask which is called "wolf".

The management of pediatric lupus should be coordinated by a specialized hospital center in connection with the local treatment and / or hospital physician. The treatment aims to treat the outbreaks of the disease, to prevent the occurrence of thrust and the occurrence of sequelae, to treat pain when it is present. The background treatment most often comprises platinum, sometimes combined with treatment with nonsteroidal anti-inflammatory drugs, corticosteroids and / or other immunosuppressants (imurelR, cellceptR, endoxanR in particular), and analgesics.

Scheduled consultations should be done at least every 2 to 3 months depending on the nature of the events. In particular, they will make it possible to ensure that Lupus is well controlled, to possibly adapt the treatment, and to look for complications, in particular renal, by researching the presence of proteins in the urine. You will find a more detailed description of the manifestations and treatments of Systemic Lupus in references 1 and 2.

2 What are the signs that should lead to prompt medical advice?

Unusual signs may result from an outbreak of the disease, a side effect of treatment or infection (especially in case of treatment with corticosteroids and / or immunosuppressants), and should lead to a rapid medical examination. This may include, for example, fever, headache or severe stomach pain, pallor, easy bruising or bleeding through brushing, behavior change (but this list is not exhaustive).

3 What to avoid

1. Sex exposure: It can cause an eruption, sometimes serious, on the areas of the skin exposed to the sun, and sometimes also cause a relapse of the disease. The wearing of long sleeves and a hat, and the regular application (every 2 hours and after each bath) of powerful sunscreen creams (index at least equal to 50) is therefore necessary in all children and adolescents with a Lupus.
2. Smoking: It decreases the effectiveness of PlaquenilR and increases the risk of cardiovascular complications in adulthood.
3. The abrupt discontinuation of treatment, especially cortisone can lead to a serious complication (acute adrenal insufficiency). Stopping other treatments may result in Lupus flare.
4. The cessation of specialized follow-up, especially during adolescence or when transferring Pediatric care to an adult medicine service. Specific care for each medical team can then be useful.
5. Take oestroprogestative oral contraception (the most usual) without discussing it with the doctor responsible for the management of Lupus.

4 Is a diet necessary?

A diet is necessary in case of treatment with corticosteroids. A plan sheet will be given for this. A diet low in sugar is essential to avoid excessive weight gain if cortisone doses are important. A low salt diet may be helpful depending on your child's condition and cortancyl dose.

5 Which vaccines are recommended? What are the contraindicated vaccines?

The usual vaccination schedule should be followed according to the vaccination recommendations in force. However, in case of treatment with an immunosuppressant, biotherapy and / or high-dose corticosteroids live vaccines (measles, rubella, mumps, yellow fever, oral polio, BCG vaccine) are contraindicated.

Vaccinations against influenza, pneumococcus and vaccination against cervical papillomavirus infections in adolescents before the first sexual intercourse are recommended according to the usual pattern. Vaccine efficacy may be decreased by concomitant immunosuppressive therapy, and booster may be necessary after discontinuation of immunosuppressive therapy. Vaccination should be avoided during an outbreak of the disease.

6 Which contraception to use?

The oestroprogestative pill (most often prescribed) is most often contraindicated and can only be considered after a specialized evaluation. Progestin-only pills are generally preferred, such as chlormadinone acetate (Luteran®), cyproterone acetate (Androcur®), nomegestrol acetate (Lutenyl®) and desogestrel (Cerazette®).

7 Are there any special precautions to consider before surgery, dental care?

Any surgery and dental care can be done, but it should be noted all treatments in progress. Precautions are necessary in case:

1 / treatment with cortisone and / or immunosuppressants: antibiotic treatment before and after the operation may be proposed in some cases because there is an increased risk of infection

2 / prolonged treatment with cortisone: it should never be interrupted 3 / treatment with antivitamin K in cases of anti-phospholipid syndrome: treatment with oral anticoagulant should be stopped just before the operation (including tooth extraction) and resumed immediately after and replaced by subcutaneous anticoagulation according to medical prescriptions. In some cases, antibiotics will be prescribed for dental care.

8 Are there any special precautions to consider before traveling abroad?

There are no contraindications to travel (make sure you have enough medicines for long trips). If anti-phospholipid antibodies are present in the blood, it may be advisable in some cases to give a subcutaneous injection of low molecular weight heparin one hour before departure if traveling by plane to avoid the occurrence of thrombosis. The list of specialized centers abroad is available on the PRINTO website (ref 1).

9 Is it possible to have normal schooling? A sports activity ?

Normal schooling is possible in the vast majority of cases. In case of significant absenteeism, home schooling or other facilities may be offered through the MDPH (House of Disability).

Regular physical activity should be encouraged in children during periods of remission. However, it is necessary to take precautions under certain conditions: 1° violent sports are to be avoided in case of drop of platelets or anticoagulant treatment, which promotes bleeding 2 ° sports activities are to be avoided in case of painful joint damage and the more often during Lupus flares.

10 What financial aid is possible?

Systemic Lupus is recognized as a long-term condition (ALD) with 100% coverage based on the social security tariff for Lupus care, treatment and transport costs. Depending on the impact of your child's illness on daily life, additional help may be requested from the MDPH.

Most of them are female, sometimes with a sex ratio of 9 women for 1 men, as in Lupus and Sjogren's Syndrome. Why this feminine predominance? For a long time, the only incirminated factor was female hormones (estrogens) but this explanation was insufficient because estrogens may even have, under certain circumstances, a protective anti-inflammatory role. In recent years, other interesting original explanations have been made. It is likely that the female X chromosome plays a major role because it carries many genes of immunity. The expression and regulation of these genes could be disrupted in major autoimmune diseases such as lupus.

Other phenomena such as fetal-maternal microchimerism, which is the exchange of cells (especially lymphocytes) during pregnancy through the placenta, may also play a role. So, systemic autoimmune diseases, such as lupus or localized forms of an organ (such as thyroiditis), preferentially affect women. For example, lupus or Sjögren's syndrome are female disorders in 90% of cases. This feminine predominance is an old mystery that is gradually elucidating.

Estrogen... the only the hormonal part of the mystery!

Estrogens are hormones with multiple immune effects that vary according to their type, concentration and target "tissues". They generally have a pro-inflammatory effect but can exert beneficial effects as in the bone where they oppose the osteoclastic resorption. There is an autoimmune disease, lupus, in which there is obviously an estrogen dependence as evidenced by aggravating pregnancy and sometimes the deleterious effect of the estroprogestative pill. In this disease, there may be tissue hyperoetrogenism that may be related to an increase in aromatase activity that transforms male hormones into estrogen. Estrogens are able to act directly on all cells of the immunity by activating inter alia autoreactive T and B lymphocytes in lupus while this phenomenon is not observed in healthy subjects.

Recently, it has also been shown that catabolites of estrogens called catecholoestrogens could have a toxic effect on the DNA, which is likely to make it immunogenic and to favor the appearance of anti-native anti-DNA antibodies characteristic of lupus. In other autoimmune diseases, estrogens probably also have immune effects but this has been less studied. which is likely to render it immunogenic and to promote the appearance of native anti-DNA autoantibodies characteristic of lupus. In other autoimmune diseases, estrogens probably also have immune effects but this has been less studied. which is likely to render it immunogenic and to promote the appearance of native anti-DNA autoantibodies characteristic of lupus. In other autoimmune diseases, estrogens probably also have immune effects but this has been less studied.

The X chromosome is very immune. An attractive genetic explanation (epi) genetic!

The X chromosome carries many genes (several hundred) that encode immunity proteins, some of which have a very important role in autoimmune diseases (TLR7, IRAK1, CD40L). Thus, it has recently been observed that the only model of mouse lupus (mice) occurring in males is due to a duplication of the innate immunity genes called TLR7 involved in interferon synthesis. It is also interesting to observe that some of the rare genetic syndromes like Klinefelter Syndrome, which are "double X" (XXY) men, make as much lupus as women.

Women (XX) have 2 times more X chromosomes than men (XY)! The very ecumenical nature, created a phenomenon of equilibrium between the two sexes which is linked to the fact that the feminine cells will systematically inactivate one of the two X chromosomes randomly. This phenomenon of inactivation of the 2nd X chromosome is linked to methylation of chromosomal DNA. Recently, it has been shown that in lupus women, there is a defect of this inactivation by methylation. Thus, lupus women could therefore express the genes of immunity carried by their 2 X chromosomes, putting them in a state of "hyperimmunity". This very original phenomenon illustrates well the major role of epigenetics (that is to say the control of the

The role of microchimerism or the history of a transplacental fetal-maternal cell exchange!

It has been shown that during pregnancy, there was a "physiological" exchange of cells (especially lymphocytes) across the placenta. Surprisingly, it has been shown that these fetal cells could survive, after childbirth, for many years in women. thus, it was detected, in the circulation of a woman, the cells of her son born 29 years earlier. This phenomenon, called microchimerism, is physiological but may be important in autoimmune diseases. The role of microchimerism has been considered by analogy with a condition called graft-versus-host disease that occurs after a bone marrow or organ transplant, that is, in a chimerism (host / graft) situation. In case of transplant,

The role of microchimerism has been studied in scleroderma women with the detection of an excess of fetal cells in the blood and scleroderma skin lesions. However, it is unclear whether this microchemical phenomenon is the cause or consequence of autoimmune disease. Indeed, it can be speculated that fetal cells could exert a "repair" effect because they retained redifferentiation capabilities useful in tissue repair.

What is the role of microchimerism? Beneficial or adverse?

This remains to be determined but it is possible that this role is not the same in all autoimmune diseases because this phenomenon has not been observed in some of them such as Lupus.

The female predominance of most autoimmune diseases is a reality but the mechanisms that explain it are probably more complicated than previously thought. The discovered discoveries will certainly allow a better understanding of these diseases and perhaps to the identification of new therapeutic strategies and case to follow!