Juvenile dermatomyositis (JDM) is an autoimmune disease characterized by systemic vasculopathy. Its main manifestations include symmetrical proximal muscle weakness, elevated serum muscle enzymes and cutaneous lesions, among which the heliotrope and Gottron's papules are pathognomonic. Early recognition and prompt therapy allow better prognosis and prevent the development of calcinosis. Although the treatment is based on glucocorticoids, the more commonly associated immunosuppressors include methotrexate, azathioprine, cyclosporine, and cyclophosphamide, depending on the severity of disease. The use of immunobiologicals for refractory cases remains under investigation, but the results are controversial or inexpressive. In this review, we highlight recent updates on the pathogenesis and treatment of JDM.
Objectives:To determine the more frequently seen clinical and immunologic manifestations in the antiphospholipid syndrome (APS) in patients of 2 referral centers. Methods: We studied 50 patients with the diagnosis of primary or secondary APS, 49 females and 1 male, with ages ranging between 15 and 65-year-old, using a predetermined protocol in which we measured the immunologic and clinical spectrum, including the determination of anticardiolipin antibodies (ACA) and lupus anticoagulant (LA); in some cases we also measured anti- 2-glycoprotein I. Results: The most frequent clinical manifestations were thromboses in several sites detected in 88% of cases; the most common location was the lower extremities (40%) and as pulmonary emboli was detected in 20%. Arthritis was found in 70% of the cases, livedo reticularis in 52%, Raynaud phenomena in 50%, migraine in 40%, hemolytic anemia in 30%, major depressive disorder in 22% and recurrent miscarriage in 18%. Immunologic studies showed antinuclear antibodies (ANA) in 80%, ACA of IgM and IgG isotypes were found in 56% and 44% respectively, LA in 34% and anti-DNA antibodies in 40%. Conclusions:The main clinical manifestations in patients with APS are: lower extremities thromboses, pulmonary emboli, cutaneous, articular and neurologic involvements, with the presence of ACA (isotype IgM). Introduction:The catastrophic antiphospholipid syndrome (CAPS) is characterized by a rapid-progressive thrombosis of small vessels, multiple organ failure in the presence of antiphospholipid antibodies, might be fatal in more than 50% of cases. The risk-factors identified in the develop of CAPS are, bacterial and/or viral infections, surgery, inadequate treatment of systemic lupus erythematosus (SLE), inadequate treatment of antiphospholipid syndrome (APS), or use of estrogens. Objectives: To identify methods to prevent the progression to CAPS in patients with primary and secondary antiphospholipid syndrome (APS). Patients: Patient 1: 28-year-old female with SLE without treatment, with a previous history of 2 fetal losses and 2 normal vaginal deliveries. During the 3rd trimester of her 5th pregnancy the patient presented with fever, hypertension, renal involvement, deep vein thrombosis, cerebral organic syndrome, convulsions and coma. Patient 2: 22-year-old female with SLE, lupus nephropathy classified as WHO class IV, secondary APS and cutaneous vasculitis. Exacerbation of the SLE was secondary to sun exposure and insufficient corticosteroids doses. Methods:The risk-factors were identified and the patients were treated with: 1. High corticosteroid doses. 2. Full anticoagulation with low molecular weight heparin. 3. Aggressive broad-spectrum antibiotic treatment. 4. Intravenous immunoglobulins infusions at the doses of 1g/kg/d for 2 subsequent days. 5. Control of the hematological disorder in the intensive care unit (ICU). Results: The outcomes of both patients were successful as demonstrated by controlling the events and not developing CAPS. Conclusions: Our experience suggests that ever...
Axial gout can affect all segments of the spine. It is manifested as back pain, as pain associated with neurological symptoms, and as neurological impairment without pain in 17.9%, 75.8% and 4.2% of cases, respectively. These manifestations were the first presentation of gout in many patients. Although x-rays as well as computed tomography and especially magnetic resonance scans can be very suggestive, histopathological, cytological and crystal analyses are the diagnostic gold standard. In most cases involving neurological manifestations, the patient underwent surgery, leading to satisfactory results. There are, however, some reports of full recovery following the usual clinical treatment for gout, suggesting that such treatment may be the initial option for those subjects with a history of gout and radiological findings of axial involvement.
Background Psoriatic Arthritis (PsA) may affect up to 40% of patients with psoriasis (PsO). Usually PsO precedes PsA for many years, though PsA is frequently not identified in dermatology clinics. Diagnosis of PsA is based on clinical recognition of arthritis, enthesitis and spondylitis. Objectives To determine the prevalence of PsA in a large cohort of Brazilian patients with PsO attending different dermatology reference centers. Methods A multicenter study was conducted in four universitary dermatology clinics, from January to March 2011. In each center, consecutive patients with a confirmed diagnosis of PsO were evaluated by a rheumatologist. Following detailed musculoskeletal anamnesis and physical examination, subjects were classified as having: isolated PsO, osteoarthritis (OA) and/or chronic myofascial pain (CMP) syndrome, PsA (CASPAR criteria1). Laboratory and x-Ray tests were performed, as needed, according to the rheumatologist clinical judgment. Results A total of 524 PsO patients were evaluated. Mean age was 48.5 ± 14.5yrs, 50% were females and PsO mean duration was 15.4±11.7yrs. The vast majority (79%) had plaque psoriasis and 57.8% required systemic treatment. Isolated PsO was the diagnosis in 45% of patients, whereas 22% manifested OA and/or CMP. A definitive diagnosis of PsA was documented in 175 patients (33%), of which 49% were newly identified by the rheumatologist. Lab and/or x-Ray tests were necessary for the diagnosis of PsA in 42/175 individuals. In 38/175 subjects, PsA was associated with OA and/or CMP. Most PsA patients (72%) had peripheral, 11% axial and 17% both peripheral and axial involvement. Dactilitis occurred in 20% and clinical enthesitis in 30%. Remarkably, PsA patients were older (51 vs 47yrs, p 0.015), had more nail involvement (59 vs 47%, p 0.008), were less likely to be on any systemic treatment (42 vs 56%, p 0.01) and were more frequently using biologic drugs (21 vs 5%, p>0.001) compared with those without PsA. Conclusions We have demonstrated a similar worldwide prevalence of 1/3 of PsA among Brazilian patients with PsO. The identification of new PsA diagnosis by a rheumatologist in half of patients, previously unrecognized at the dermatology setting, points out to the need for shared care between dermatologists and rheumatologists in order to establish earlier PsA diagnosis and adequate multidisciplinary management. References Taylor W, Gladman D, Helliwell P et al. Classification criteria for psoriatic arthritis: development of new criteria from a large international study. Arthritis Rheum 2006; 54:2665–73 Disclosure of Interest None Declared
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