1/26. Atypical esophageal diverticula associated with progressive systemic sclerosis.Five cases of unusual esophageal diverticula associated with progressive systemic sclerosis (scleroderma) or collagen vascular disease are presented. These wide-mouthed saccular diverticula were infrequently seen in a group of PSS patients with the typical motility disturbance of esophageal involvement and are reminiscent of the diverticula of the colon involved by PSS.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
2/26. Anti-rna polymerase iii antibodies in the diagnosis of scleroderma renal crisis sine scleroderma.We describe the use of antibodies to rna polymerase iii in the diagnosis of scleroderma in 2 patients who presented with renal crisis without other clinical features of the condition. Both presented with accelerated hypertension, rapidly progressive acute renal failure, microangiopathic hemolytic anemia, and thrombocytopenia. One patient developed digital infarcts in the course of his initial illness. Neither showed evidence of skin thickening at presentation. Nailfold capillaroscopy was normal in one patient and showed capillary dropout in the other. Renal biopsy showed findings consistent with thrombotic microangiopathy and both had anti-rna polymerase iii antibodies.- - - - - - - - - - ranking = 0.86157029414511keywords = microangiopathy (Clic here for more details about this article) |
3/26. Systemic sclerosis and antineutrophil cytoplasmic autoantibody-associated renal failure.We report three patients who developed antineutrophil cytoplasmic autoantibody (ANCA)-associated crescentic glomerulonephritis, two of whom showed clinical features of limited scleroderma and one whose results of serological tests were suggestive of limited scleroderma without cutaneous features. All had anticentromere antibodies and antimyeloperoxidase antibodies. No patient showed the features of typical scleroderma renal crisis such as accelerated hypertension or microangiopathy. Our patients were normotensive at the time of onset of renal failure, and the clinical picture was characterised by only modest features of limited scleroderma. All three patients had crescentic glomerulonephritis at various stages of chronicity. One patient responded to immunosuppressive therapy with improvement in renal function; the other two patients rapidly developed end-stage renal failure. These patients and others recently described may represent a newly described form of scleroderma renal disease.- - - - - - - - - - ranking = 0.86157029414511keywords = microangiopathy (Clic here for more details about this article) |
4/26. A case of progressive systemic sclerosis complicated by massive pleural effusion with elevated CA125.A tumor marker, CA125, is known to increase in the serum or other body fluids in various malignancies such as ovarian cancer. Here we present a case of progressive systemic sclerosis (PSS) with massive pleural effusion, in which CA125 in the serum and pleural fluid were elevated. The serum level of CA125 decreased in accordance with the change of the pleural effusion. CA125 level may be an indicator for the activity of serositis in some cases with collagen vascular diseases.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
5/26. Progressive appearance of overlap syndrome together with autoantibodies in a patient with fatal thrombotic microangiopathy.We describe an extraordinary patient with overlap syndrome (systemic lupus erythematosus, systemic sclerosis, and rheumatoid arthritis) having positive autoantibodies against Sm, double stranded dna, dna topoisomerase I, and centromere, together with rheumatoid factor. The patient had multiple organ involvement resulting from thrombotic microangiopathy that mimicked so-called normotensive scleroderma renal crisis, and died mainly of massive pulmonary hemorrhage caused by thrombotic thrombocytopenic purpura. The clinical presentations of the case support the concept of strong associations between disease-specific autoantibodies and clinical features.- - - - - - - - - - ranking = 4.3078514707255keywords = microangiopathy (Clic here for more details about this article) |
6/26. Palliation of systemic sclerosis-associated pulmonary hypertension by atrial septostomy.The onset of pulmonary hypertension in patients with systemic sclerosis carries a poor prognosis. Atrial septostomy has been used successfully to palliate endstage primary pulmonary hypertension but has not been attempted in other forms of pulmonary vascular disease. We report substantial clinical improvement following atrial septostomy in a patient with systemic sclerosis complicated by severe, isolated pulmonary hypertension. After the procedure, exercise capacity was improved and exertional syncope abolished. We suggest that this procedure should be considered for other patients with this diagnosis.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
7/26. Renal failure due to scleroderma with thrombotic microangiopathy developing in a woman treated with carboplatin for ovarian cancer.Acute renal failure in association with microangiopathic hemolytic anemia and the pathological finding of thrombotic microangiopathy may occur in a number of conditions including hemolytic uremic syndrome, thrombotic thrombocytopenic purpura, and systemic sclerosis. Distinguishing between these conditions on clinical grounds may be difficult, and further investigations, including serological tests, are normally helpful. We present a patient who was treated with 5 doses of monthly carboplatin chemotherapy for stage IIb ovarian carcinoma and who subsequently developed acute renal failure and microangiopathic hemolysis together with some cutaneous features of systemic sclerosis. Initial serological tests, including anti-nuclear antibody titers measured using rat hepatocytes, were normal, and renal biopsy showed features of microangiopathic hemolysis, fibrinoid change, patchy tubular atrophy, and concentric intimal proliferation. A clinical diagnosis of diarrhea-negative hemolytic uremic syndrome was made and she was treated with plasma exchange and fresh frozen plasma infusion. However, she remained dialysis-dependent. Several weeks later she died following a cardiac arrest. Post-mortem examination revealed medial hypertrophy, concentric intimal proliferation, and thrombi within the small arteries of the kidneys and lungs. Subsequent results from tests taken at the time of her presentation with acute renal failure revealed a normal von willebrand factor qualitative distribution, and a positive anti-nuclear antibody titer (using a human cell line) in association with positive autoantibodies to RNA polymerase types I, II, and III. Taken together, the clinical, laboratory, and post-mortem findings were suggestive of a diagnosis of systemic sclerosis. We discuss the differential diagnoses, and the associations between these and malignancy and chemotherapy. Finally, we consider the serological tests used for the diagnosis of systemic sclerosis that were, in this case, initially misleading.- - - - - - - - - - ranking = 4.3078514707255keywords = microangiopathy (Clic here for more details about this article) |
8/26. Digital necrosis related to carboplatin and gemcitabine therapy in systemic sclerosis.We present a woman with scleroderma who developed multiple ischemic digits after chemotherapy for lung cancer. The ischemia started during treatment with carboplatin and gemcitabine and required amputation of the affected digits. A review of the literature shows that thrombotic episodes coinciding with chemotherapy are not uncommon, though venous thrombosis occurs more frequently than arterial. Scleroderma patients are at particular risk for digital infarction because of their underlying vascular disease and associated Raynaud's phenomenon. This case illustrates the risk of severe digital ischemia and digital loss in patients with scleroderma during chemotherapy with carboplatin and gemcitabine.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
9/26. Severe refractory fingertip ulcerations in a patient with scleroderma: successful treatment with sildenafil.Systemic sclerosis (scleroderma) is a multisystem fibrotic disease that commonly manifests with severe Raynaud's phenomenon and slow-healing cutaneous ulcerations. Reduced nitric oxide levels have been proposed to play a role in the pathogenesis of vascular disease in scleroderma, and therefore sildenafil (which increases nitric oxide levels) is an attractive therapeutic prospect. We describe a patient with limited cutaneous systemic sclerosis who presented with severe nonhealing finger ulcerations despite conventional management, who showed marked improvement with oral sildenafil.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
10/26. Secondary pulmonary arterial hypertension: treated with endothelin receptor blockade.Secondary pulmonary arterial hypertension (SPAH) is an adverse outcome of a variety of systemic disorders. These include collagen vascular diseases, chronic thromboembolism, human immunodeficiency virus, portopulmonary hypertension, and other diseases. Progression of SPAH may persist despite stabilization of the causative disease, thereby contributing to the poor quality of life and unfavorable survival in these patients. Treatment of the underlying cause and oxygen supplementation may alleviate symptoms, but no specific therapy to treat SPAH currently exists. Endothelin receptor blockade with bosentan has been shown to be beneficial in the treatment of primary pulmonary hypertension, but efficacy of this therapy in SPAH has not been established. We describe a case series of 6 patients with disparate causes of SPAH, who benefited from endothelin receptor blockade therapy. The causes of SPAH included collagen vascular disease (scleroderma) (1); systemic lupus erythematosus (2); chronic thromboembolic disease (2); and granulomatous vasculitis from sarcoidosis (1). Therapy with bosentan led to improvements in symptoms, new york heart association functional class, and walking distance in all patients. Distance walked in 6 minutes increased from a mean of 151.67 /- 69.30 m at baseline to 314.83 /- 89.09 m after an average of 14 months of bosentan treatment. Pulmonary arterial pressure decreased in most but not all 6 patients on follow-up echocardiography. This case series suggests a role for endothelin receptor blockade therapy in SPAH and should generate further interest in pharmacologic management of SPAH. A prospective controlled clinical trial of bosentan in SPAH is urgently needed.- - - - - - - - - - ranking = 2keywords = vascular disease (Clic here for more details about this article) |
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