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Ssc: Notes and Notes
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Rituximab in the treatment of systemic sclerosis, the results of early studies in progress
CATHOLIC UNIVERSITY STUDY
Scleroderma: A New Strategy for the more aggressive forms
You can avoid the heavy effects of therapy combining low doses of immunosuppressive drugs with a biologic
MILAN - Nine patients are not sufficient to determine the success of a treatment, but the experimentation which is conducting about three years the group OU of Rheumatology at the Catholic University of Rome offers new hope for people, especially younger ones, affected by the more aggressive forms of systemic sclerosis.
The disease presents in two capacities: "The first and most frequent - said Gianfranco Ferraccioli, study coordinator and director of operations in the Roman structure - this is a limited form, has a slower progression and less aggressive.
The second variant, called widespread, affecting a fifth of patients, is more aggressive and affects younger patients, which are usually treated with high doses of cortisone and anticancer drugs. " "Drugs - added the rheumatologist - who, however, important side effects, such as infertility or bladder and lung complications. And unfortunately, as the latest clinical trials, they also have limited effectiveness. "
. STUDY - Just in these patients is focusing the research , whose preliminary results were published in recent weeks in the journal Arthritis Research & Therapy . "The original objective - Ferraccioli explained - was to find ways to avoid these very young patients with infertility problems related to therapy with alkylating immunosuppressants." And the solution has been the identification of a new therapeutic target that, in addition to be effective, has allowed to clarify the characteristics of a very complex disease. The new target are B lymphocytes, cells of the immune system that, in case of disease, produce antibodies against constituents of the organism. "We've eliminated" adds the expert "with a biologic agent, which, combined with a very low dose of immunosuppressant, has produced better results than traditional treatments," continued the rheumatologist. The treatment enables both a reduction dell'ispessimento skin is having to stop damage to internal organs. As to side effects, the suppression of a population of cells critical to the immune system can lead to a slight increase in infections, but it is acceptable contraindications and less serious than those caused by the therapy in use today. The drug (rituximab) is already used in the treatment of lymphoma, and recently also in certain autoimmune diseases such as rheumatoid arthritis and lupus. Now you could also add systemic sclerosis: "The treatment is performed with two infusions 15 days apart - Ferraccioli explained - and the patient is observed for 6 months. The moment reappear B cells therapy can be performed again. "
FIRST STEP TOWARDS THE TURNING POINT? - While admitting that it is a "promising treatment" in an editorial published on the same issue, Jacob M. Van Laar Institute of Cellular Medicine Newcastle University, however, warns of the need for further studies evaluating a larger population and long-term efficacy and safety of the therapy. "Trials like 'being' could be costly and complex, requiring perseverance and ingenuity as well as to develop methodologies that sopperiscano the complexity of a disease that has a low haze and a wide range of presentations and clinical courses." On the other hand, the same Ferraccioli reiterated that this treatment is now indicated only for people with a very aggressive form of the disease. "Moreover, to be effective, conventional immunosuppressive therapy should precede - albeit at low doses - one with the new drug." However, seems to be approaching the time when patients suffering from scleroderma can enjoy an effective and acceptable by the side effects: "Now that we have in hand a potentially effective and relatively safe drug - said van Laar - is approaching the moment when groped to overcome the impasse in the treatment of systemic sclerosis. "
Antonino Michienzi - June 22, 2010
Corriere della Sera.it Health / Rheumatology © REPRODUCTION RESERVED
But what is rituximab? It is a drug that is primarily used to treat people with a form of cancer known as lymphoma. He had the approval of the FDA since 1997 for this purpose. A lymphoma involves cells of the immune system specialists calls B cells and the drug causes the lysis (= disintegration) of the malignant B cells.
A principal function of B lymphocytes in a healthy body is to produce antibodies. Rituximab is also approved for the treatment of severe active rheumatoid arthritis where other treatments have not been successful. On an experimental basis, has been used to treat a number of what are called autoimmune diseases in which the body produces antibodies harmful that attack normal tissues, these are known as autoantibodies. Examples of immune and autoimmune diseases where rituximab has been used include SLE (systemic lupus erythematosus), multiple sclerosis, Sjogren's syndrome and bullous skin diseases (eg, pemphigoid).
At the moment the Rituximab has no indication in the treatment of Systemic Sclerosis and is therefore not normally used in the centers rheumatologic as a treatment. However in many Italian towns protocols are being tested on a large number of patients, we await with great anticipation the results of these studies will be published presumably in the middle of this year.
March 8, 2012
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Italian League Onlus Systemic SclerosisSPINE AND RESPIRATORY REHABILITATION: Rehabilitation in SSc
Systemic sclerosis is a chronic evolution, which features more 'obvious is the hardening and thickening of the skin in more or less extensive areas of the body surface. Affecting multiple devices, and not least the skin, in an important way impairs the quality of life of affected individuals both for what concerns the disease and the significant limitations that entails, both for the psychological component that follows.
The thickening of the skin it causes significant limitations such as the joint leading to a progressive reduction in the number and quality of the assets of the entity that can play every day, also alters the disease so devastating at times the appearance of those affected, to the point of not be p ORE correspondence between the age and the established.
Alongside the purely aesthetic, must be taken into consideration the problems arising from the functional limitations of the temporo-mandibular joint, preventing the normal course of daily activities such as the feeding, oral hygiene, normal conversation.
The skin changes are characterized by thinning of the epidermal layer, distorted by compact collagen bundles which run parallel to the skin itself, or that connect with the underlying tissues. The dermal appendages are atrophic and disappear papillary anchors.
At the level of muscle assists in the early stage to interstitial edema with perivascular infiltration by lymphocytes and plasma cells, while in the full-blown progressive phase are found the usual compact bundles of collagen that lead to a progressive interstitial fibrosis and frequently to regressive alterations of the muscle fibers themselves .
The rehabilitation therapy
Drug therapy for essential, may find benefit from the association of rehabilitation therapy that works on several fronts of involvement of the disease to offer the patient the best possible quality of life.
From the point of view fisiochinesiterapico is possible to improve the situation of the patient's motor and prevent further complications.
At the Institute of Scientific Montescano, Pavia - Maugeri Foundation - IRCCS - has been set, among others, a multidisciplinary rehabilitation intervention targeted sessions consisting of gymnastics and track of the spine associated with respiratory gymnastics.
Kinetic of the spine. 
The decreased elasticity of the connective tissue caused by scleroderma, induces a retraction of all muscles. This retraction is reflected at the level of all the body structures including the vertebral column and the tracks which, given their importance from a functional point of view must be particularly taken into account in rehabilitation training. It 'should therefore seek to maintain and possibly increase the joint range-of-motion exercises with specific and general.
At the medical center Montescano, have identified a series of exercises that, in part, are designed specifically to affect the spine and the tracks and, in part, are associated with respiratory rehabilitation. In this way you can get an intense mobilization of the spine and reducing the duration of the session tracks without straining the patient too.
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The anatomy of the joint, and in particular the corresponding apparatus capsular ligament laxity and elasticity, allows the understanding that the joint is not a drive mechanism whose movement takes place only within predetermined patterns. Next to the movement that the joint can take actively there is, in fact, the ability to play passively movements much broader, or even movements that are not possible voluntarily. E 'especially in this type of movement, called game articular, which are addressed maneuvers manipulative joints. It represents a margin of "tolerance" which allows you to perform movements aimed, which must always be accompanied by accessory movements of adjustment. E 'important to note that, in the cases of limitation, is detectable a sharp decrease of the game articular. Restoring these accessory movements with specific maneuvers (slipping and translations elle articular surfaces), you will act on the apparatus capsular ligament with contrasting effect the movement retrazionale.
March 5, 2012
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Maugeri Foundation - UO Montescano, PaviaNeuromotor Rehabilitation
Head Dr. Guido Felictti
Raynaud's phenomenon, very often you end up in Angiology, many patients receive a correct diagnosis?
From 2009 to Sandro Pertini Rome an interdisciplinary course strongly backed by friendly, attentive and cooperative. The impetus for this action is dall'Angiologia, which too often on specific patients in obvious presence of Raynaud's phenomenon are addressed by their general practitioners.
"At the 'UOSD of Angiology of Sandro Pertini of Rome has been operational since 2009, an interdisciplinary course devoted to patients with SSC supported by a cardiologist, pulmonologist, radiologist, pathologist, angiologist, grief therapist, plastic surgeon and specialist nurse in the care of ulcers in scleroderma.
Coordinator Dr. Antonella Marcoccia that, in the presence of Raynaud's Phenomenon, starting from capillaroscopy positive for "scleroderma pattern", inserts the patient in a process led to completion diagnostic targeted to the early diagnosis of systemic sclerosis.
The route includes the performance of pulmonary function tests with DLCO, echocardiography, immunological tests, and in case of deterioration of pulmonary function tests, high-resolution chest CT. Depending on the outcome of the examinations, the patient is sent in a rheumatology or cardiology or pulmonary expertise.
In the absence of target organ damage, the patient is treated with basic therapy with calcium antagonists, proton pump inhibitors and antiplatelet agents, is trained in the rules of life and is followed with regular follow-up half-yearly or yearly.
At the DH of Angiology, in the presence of severe Raynaud's phenomenon with digital ulcers, the patient is treated with vasoactive infusion therapy, analgesic and possible antibiotic treatment in the presence of infection in addition to accurate local dressings. At the first onset of ulcers is the patient known to be the path followed in other centers but patients with a diagnosis already known, it has a clinical audit privileged access to our service within 24-48 hours. "
We just have to compliment you for the commendable work done in these departments and hope that, in every Italian Angiology, our patients can find competent and prompt and accurate information on diagnostic and therapeutic path to follow.
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Italian League Onlus Systemic Sclerosis
February 17, 2012
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