THEthe name “Lupus” arises from the most common manifestation of the disease with lesions on the sides of the nose that resemble the wolf’s bite between the eyes of prey. Lupus is one systemic autoimmune disease where our immune defenses confuse targets. Instead of activating themselves against infectious agents or tumor structures, they attack their own tissues, causing a state of general inflammation. The latter, when not controlled by drugs, causes severe disturbances. Lupus in particular can also cause the inflammatory nephritis, as a result of which patients may lose the functioning of the kidneys. The disease is defined as systemic because it can damage any organ.
When does it appear?
The illness manifests above all among young womenwhich represent the 90% of the patient audience. They are aged between i 20 and 40 years, very few senile forms. “The female hormones and factors related to chromosome x” – he claims Pierluigi Meroni, Professor of Rheumatology at the University of Milan – «facilitate this gender susceptibility“. Among the autoimmune diseases, only in lupus and Sjogren’s syndrome is there such a high female prevalence.
Getty Images
What is striking?
Statistically the skin is one of the most affected organswith inflammatory manifestations e erythematous-type lesions purplish following exposure to UV rays (on the face, arms and décolleté) or nodules which can ulcerate. The disease also appears in the form of inflammatory arthritis similar to rheumatoid arthritiswith the difference that joint disorders do not cause ankylosis and alterations in the shape of the limbs in the majority of cases. Less than a third of patients reports problems i kidneys. “There acute nephritis unfortunately it is completely asymptomatic “- he says -” and it comes detected initially only through urinalysis and / or in the presence of arterial hypertension“. Ultimately lupus can spawn an inflammation of the central nervous system: «With life threatening»- he continues -« for less than 20% of subjects“.
What are the symptoms?
It’s a devious disease. The only specific symptom is the malar rushsometimes confused with seborrheic dermatitis or a banal rosacea: «I symptoms I’m vague“- he explains -” mostly photosensitivity and joint pain“. Moreover, inflammation of the pericardium and pleura, which determine pericarditis and pleurisy: «Also in this case, the patients do not suffer from particular heart or breathing problems» – he declares – «they only feel pain in the chest. By performing the echocardium, the possible payment is identified “. These “generic” symptoms often slow the diagnosiswhich can be defined even after 6-8 months from the beginning of the disease.
What exams to take?
Some have to be done to ascertain lupus specific exams: «In particular, they are sought markers for various autoantibodies»- he explains -« for example antibodies antinuclear and anti dna, which is a marker of the disease ‘. In addition, i values of complement consumptionthe changes in red blood cells and platelets: «Patients can in fact manifest leukopenia» – he underlines – «thrombocytopenia and haemolytic anemia».
Is it a genetic question?
Lupus it has a genetic component. Exist family clusters of autoimmune diseases: “This does not mean that a gene transmits the disease:” It is not a somatic trait like the color of the hair or eyes “- he specifies -” but the combination of genes that together favor its appearance“. However, genetics are not enough to cause it: “An external stimulus is needed“- he explains -” for example an infection even trivial, like the flu ». The alteration of the immune system, dormant up to that moment, emerges and openly declares: “The good news is that if lupus is not inherited directly»- he comments -«women with this condition can have children without fear of passing it on to the progeny ».
What Happens During Pregnancy?
The 9 months of waiting, with theincrease in circulating estrogen, facilitate lupus disease and they can exacerbate it in some cases. “Until the 1960s and 1970s, doctors advised against pregnancy to women with this pathology “- he declares -“today instead we only ask to program it“. If the patient goes through a phase of active disease she must avoid fertilization: «Yesonly when the disease is in remission at least for 6-8 months “- he remarks -“you can plan the pregnancy”. Not all drugs immunosuppressants for lupus are compatible with gestation And breastfeeding. Also contraceptive methods are not equivalent for these patients. They can use spirals and rings but the estrogen-progestogen pill is not recommended in some patients with particular types of autoantibodies (anti-phospholipid antibodies) which can significantly increase the risk of thrombosis partly associated with the contraceptive pill itself.
What do babies risk?
The eventuality of the neonatal lupuscaused by the passage of autoantibodies from the mother to the fetus through the placenta, is less than 2% for the first pregnancy. «A fetal echocardium»- he explains -« from which yes recognize the symptoms of anti-Ro antibodies“. This last can cause myocardiopathy or alterations of the cardiac conduction tissue to the child: «For newborns with these alterations» – he explains – «a peacemaker to regulate heart rhythm ». They can also occur skin reactions similar to those presented by the mother e reduction in white blood cells or platelets: «However, maternal antibodies are depleted in 5-6 months“- he says -” and these problems are solved completely without further consequences for the baby. Unlike any heart damage “.
What drugs?
A complete clinical remission it is rare. Lupus always requires a regular specialist check-up from the clinical immunologist or rheumatologist: «La survival with the new therapies has improved dramatically»- he explains -« in the 1950s lupus was an often fatal disease, today no more ». Medicine has learned to recognize aggressive forms and “mild” forms, improving their prognosis. Even today the cortisone represents the underlying therapythough we try to reduce its use as much as possible to avoid its long-term side effects (e.g. arterial hypertension, diabetes, osteoporosis etc). The combined use of other drugs like the antimalarials and immunosuppressants it offers excellent results. Next to the classical immunosuppressants (e.g. cyclophosphamide, azathioptin, mycophenolate, methotrexate), much promising are the new generation biological drugs.
What biological drugs?
Exist two types of biologicals. There first destroys cells (B lymphocytes) which produce antibodies, including the “autoantibodies” responsible for tissue damage. There second neutralizes a growth factor of B lymphocytes: «That is, it removes the fuel from the engine» – he explains – «in this way they are unable to mature, multiply and sustain the production of autoantibodies». The use of one or the other it is calibrated according to the intensity of the disease and the involvement of the organs: “The first is used when autoantibodies kill platelets, for example, cause lupus nephritis” – he says – “or are associated with major arthritis”. There administration in the first case it happens for intravenous twice a few weeks apart, in the second subcutis once a week.
How are therapies chosen?
Of course affects the aggressiveness of the diseasebut no less important are the age factor and life expectancy: «A high dosage of some immunosuppressants can induce early the menopause»- he explains -« for this reason we avoid prescribing them to young women. We use them only with special precautions ». There ideal choice is a combination of drugs which in different ways block the progression of the disease: “Without accumulating toxicity»- he affirms -« as in current oncological therapies ».
What does lupus mean today?
There lupus diagnosis is not a sentence. Thanks to science and drugs (although some not officially approved by AIFA but borrowed from the therapy of other pathologies), patients have a good life expectancy. “The data indicate that after 5 years the patients are all alive, they do not risk dying sooner »- he says -« there is only one ‘if’ and it concerns the intake of large quantities of cortisone ». There disease Unfortunately in itself promotes accelerated atherosclerosis which is aggravated by cortisone therapy in the long run: «Le women who take a lot of corticosteroids in the presence of active disease “- he explains -“they more easily develop ischemic heart attacks or strokes, even young premenopausal women ». In any case, the chances of feeling good are many: «I patients should feel encouraged by medical advances»- he concludes -« the message I want to leave is one of total trust in the future and in opportunity to live a normal life“.
iO Donna © REPRODUCTION RESERVED