11/16. Giant spontaneous hematoma of the thoracic wall in a patient with REST syndrome.We report on a 56-year-old patient with systemic sclerosis of one and a half year's duration who was admitted with an acute soft tissue swelling over the left scapula and a marked reduction of joint mobility of the left shoulder. Over the following days, the swelling extended distally and a hemorrhagic discoloration developed, reaching down to the scrotum. Nuclear magnetic resonance imaging revealed a giant intermuscular hematoma between the serratus anterior and the costal muscles. This is the first report of an extensive spontaneous hematoma of the thoracic wall in a patient with progressive systemic sclerosis. Its development and unusual localization may be explained by both the underlying systemic sclerosis and the patient's previous profession as a trapeze artist. Hemorrhagic complications of systemic sclerosis are rare but should be watched for in these patients.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
12/16. A swinging heart in limited systemic sclerosis.We describe a 3rd patient with limited cutaneous systemic sclerosis (ISSc) complicated by a pericardial effusion sufficient to cause hemodynamic compromise. Although more commonly appreciated as a complication of diffuse systemic sclerosis, the recognition of hemodynamically compromising pericardial effusion presenting in the limited form of ISSc can be lifesaving.- - - - - - - - - - ranking = 1.5keywords = sclerosis (Clic here for more details about this article) |
13/16. Gastric telangiectasis: a rare cause of severe blood loss in crest syndrome.A 46 year old woman presented with the CREST variety of systemic sclerosis and occult gastrointestinal bleeding due to vascular malformations of her stomach. Partial gastrectomy cured her anaemia. In systemic sclerosis, visceral angiography should be performed early when initial investigations have been negative.- - - - - - - - - - ranking = 0.5keywords = sclerosis (Clic here for more details about this article) |
14/16. A case of crest syndrome associated with sick sinus syndrome.Cardiac manifestations of the crest syndrome or limited systemic sclerosis (ISSc) are very rare. We report a case of crest syndrome associated with sick sinus syndrome. Histopathology of cardiac muscle revealed fibrotic changes, suggesting that such changes may be pathogenetically related to crest syndrome.- - - - - - - - - - ranking = 0.25keywords = sclerosis (Clic here for more details about this article) |
15/16. Chronic myelogenous leukemia and porphyria cutanea tarda in a patient with limited systemic sclerosis.Systemic sclerosis is uncommonly associated with hematologic malignancies. We report the case of a patient who had chronic myelogenous leukemia 3 years after the CREST variant of systemic sclerosis was diagnosed. She also later had porphyria cutanea tarda. The majority of patients who had hematologic malignancies after the diagnosis of systemic sclerosis proved to have either multiple myeloma or chronic lymphocytic leukemia. Hematologic malignancies may be found in patients with systemic sclerosis (either limited or diffuse).- - - - - - - - - - ranking = 2keywords = sclerosis (Clic here for more details about this article) |
16/16. The concurrence of rheumatoid arthritis and limited systemic sclerosis: clinical and serologic characteristics of an overlap syndrome.OBJECTIVE: The characteristics of 3 patients with longstanding rheumatoid arthritis (RA) and consecutive evolution of limited cutaneous systemic sclerosis (IcSSc) were evaluated and compared with those of patients with IcSSc alone (n = 20) or with RA alone (n = 120). methods: Clinical features of the different patient populations were compared. Serologic analyses included tests for antinuclear antibodies (ANA) and ANA subsets, in particular anticentromere antibodies (ACA) and anti-heterogeneous nuclear RNP (hnRNP)-A2/RA33 (anti-A2/RA33). RESULTS: The 3 patients with RA developed IcSSc 11, 29, or 50 years after the onset of RA. Features of IcSSc were Raynaud's phenomenon, sclerodactyly, and telangiactasias in all 3 patients, and esophageal dysmotility in 1 patient. rheumatoid factor (RF) and anti-A2/ RA33 were each found in 2 patients, and 1 of these patients was seropositive for both RF and anti-A2/RA33. ACA titers were positive in all cases. However, similar to the development of RA prior to IcSSc, the occurrence of autoantibodies typical of RA preceded the occurrence of ACA, at least in 2 of the patients. Using affinity-purified antibodies, cross-reactivities between anti-centromere protein A (CENP-A) and anti-CENP-B antibodies with anti-A2/RA33 antigens were seen in the 2 anti-A2/RA33-positive patients. Such cross-reactivities were not found in IcSSc patients without concomitant RA. epitope mapping revealed that both autoantibody specificities recognized the known major epitopes: anti-CENP-B reacted with the C-terminal region and anti-A2/RA33 with the second rna binding domain in the N-terminal region of hnRNP-A2. CONCLUSION: The RA-lcSSc overlap syndrome in these 3 patients with longstanding RA was characterized by an incomplete CREST (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias) syndrome. The study demonstrated the presence of autoantibodies typical of both diseases and cross-reactivity of ACA with hnRNP-A2/RA33 in the sera of these patients.- - - - - - - - - - ranking = 1.25keywords = sclerosis (Clic here for more details about this article) |
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