1/25. 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 = 1keywords = hemolytic (Clic here for more details about this article) |
2/25. Limited cutaneous systemic sclerosis associated with MPO-ANCA positive renal small vessel vasculitis of the microscopic polyangiitis type.Renal disease in systemic sclerosis may present in various patterns. A 66-year-old woman with a history of longstanding limited cutaneous systemic sclerosis of the crest syndrome variant presented with a sudden left foot drop and rapidly progressive renal insufficiency associated with mild proteinuria, a nephritic urine sediment, and a urinary output of 900 mL/d. There was no history of intake of D-penicillamine, and there were no signs of malignant arterial hypertension or microangiopathic hemolytic anemia. Renal histology showed a small vessel vasculitis of the microscopic polyangiitis type. serologic tests showed a marked increase of antineutrophil cytoplasmic antibodies with a perinuclear pattern and an elevated titer of antimyeloperoxidase antibodies. No clinical or laboratory signs of sjogren's syndrome were present. This clinical report adds new information to the spectrum of renal disease in systemic sclerosis. It discusses the association between systemic sclerosis and small vessel vasculitis of the microscopic polyangiitis type as well as the possible meaning of serologic markers.- - - - - - - - - - ranking = 1keywords = hemolytic (Clic here for more details about this article) |
3/25. Scleroderma associated with anemia and thrombocytopenia that responded well to cyclosporin.A 55-year-old woman came to our hospital because of cutaneous sclerosis of the limbs in September 1996, and was diagnosed with scleroderma based on a skin biopsy. In August 1997, the cutaneous sclerosis became progressive (hemoglobin level, 4.3 g/dl; platelet count, 7 x 10(9)/l). The laboratory results were positive for the direct coombs test, bone marrow aspiration showed a dry tap, and the bone marrow biopsy showed marked fatty marrow. indium-111 bone marrow scintigraphy showed a markedly decreasing uptake. These findings indicated bone marrow hypoplasia associated with hemolytic anemia. After prednisolone therapy (60 mg) was initiated, the direct coombs test became negative but the blood cell count did not increase. Then, 300 mg of cyclosporin was initiated and anemia and thrombocytopenia improved. The cyclosporin dosage was gradually decreased and the patient's hematological condition was good, although the cutaneous sclerosis changed only a little. This is a rare and interesting case of a patient with scleroderma associated with bone marrow insufficiency and hemolysis who responded well to cyclosporin.- - - - - - - - - - ranking = 1keywords = hemolytic (Clic here for more details about this article) |
4/25. gastric antral vascular ectasia in systemic sclerosis: complete resolution with methylprednisolone and cyclophosphamide.A case of severe, transfusion dependent anaemia in a 72 year old woman, which on endoscopy was found to be due to gastric antral vascular ectasia (GAVE), is reported. Repeated endoscopic sclerotherapy was ineffective. She subsequently developed Raynaud's phenomenon and on further investigation was found to have classical systemic sclerosis with lung involvement. Treatment with pulses of intravenous methylprednisolone and cyclophosphamide resulted in significant improvement in her pulmonary function tests and skin score. Coincidentally, her haemoglobin stabilised and further endoscopic examinations were normal. This is the first report of cyclophosphamide and methylprednisolone leading to complete and sustained resolution of GAVE in association with systemic sclerosis.- - - - - - - - - - ranking = 0.13665797416173keywords = anaemia (Clic here for more details about this article) |
5/25. gastric antral vascular ectasia--a cause of refractory anaemia in systemic sclerosis.Recurrent gastrointestinal haemorrhage is an uncommon manifestation of systemic sclerosis. We report a case of gastrointestinal bleeding due to gastric antral vascular ectasia (GAVE) in a patient with systemic sclerosis. Failure to recognise the condition as a cause of gastrointestinal bleeding may delay the instigation of appropriate treatment. GAVE should be considered in the differential diagnosis of anaemia in patients with autoimmune conditions such as systemic sclerosis and primary biliary cirrhosis.- - - - - - - - - - ranking = 0.68328987080866keywords = anaemia (Clic here for more details about this article) |
6/25. 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 = 3keywords = hemolytic (Clic here for more details about this article) |
7/25. Hemolytic uremic syndrome in a patient with systemic sclerosis treated with cyclosporin A.The case is presented of a 48-year-old female suffering from diffuse cutaneous systemic sclerosis (diffuse scleroderma) since 8 years, who went into renal failure as part of hemolytic uremic syndrome following 3 weeks' treatment with 3.8 mg/kg cyclosporin A. Hemolytic uremic syndrome has previously been described in transplant patients receiving cyclosporin A. There are also four cases reported in the literature of renal failure developing in middle aged females with diffuse cutaneous systemic sclerosis after short-term use of low dosage cyclosporin A treatment. It is suggested, that it may be wise not to use cyclosporin A to this category of patients, in which it can not be ruled out, that even a low dose therapy may trigger the rapid onset of scleroderma renal crisis or as in our case provoke hemolytic uremic syndrome.- - - - - - - - - - ranking = 2keywords = hemolytic (Clic here for more details about this article) |
8/25. Small intestinal complications in progressive systemic sclerosis.A case report of a 33-year-old woman with progressive systemic sclerosis and polymyositis is presented. Admitted with a beta-hemolytic streptococcus infection of the right knee joint, she had progressive systemic sclerosis with small intestinal involvement, which is rare. This led to more serious complications, malabsorption, pneumoperitoneum, and pneumatosis cystoides intestinalis, which forced a decision to treat her with home total parenteral nutrition. The diagnostic as well as treatment problems encountered in this patient illustrate the importance of nursing care in the overall management of patients with this disease.- - - - - - - - - - ranking = 1keywords = hemolytic (Clic here for more details about this article) |
9/25. Thrombotic thrombocytopenic purpura in the setting of systemic sclerosis.OBJECTIVES: To describe the association between thrombotic thrombocytopenic purpura (TTP) and systemic sclerosis (SSc) and the methods to distinguish TTP from scleroderma renal crisis (SRC). methods: A case of TTP that developed in a patient with preexisting SSc is described. medline/pubmed was searched for literature pertaining to an association between TTP and SSc, with special attention given to distinguishing TTP from SRC. In addition, the role of von Willebrand cleaving protease in the pathogenesis of TTP is reviewed. RESULTS: Including the present case, there have been 9 reports of TTP in association with SSc in the literature. In the majority of these cases TTP presented with features compatible with SRC such as renal dysfunction, thrombocytopenia, hypertension, and microangiopathic hemolytic anemia. Von Willebrand cleaving protease activity is depressed in patients with acute TTP. CONCLUSIONS: TTP in association with SSc has been reported rarely. The diagnosis of TTP, and the distinction from SRC, may be challenging, as these cases may resemble SRC. However, the correct diagnosis is critical because treatment differs substantially for each of these life-threatening conditions. Currently, the assessment of von willebrand factor cleaving protease activity may assist in making this distinction.- - - - - - - - - - ranking = 1keywords = hemolytic (Clic here for more details about this article) |
10/25. A case of overlap syndrome with rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis and secondary sjogren's syndrome.We report an unusual case of overlap syndrome that had the coexistence of five autoimmune diseases. A 45-year-old woman initially developed seropositive erosive rheumatoid arthritis (RA) 11 years ago. She then developed progressive systemic sclerosis (PSS) (including pulmonary hypertension, esophageal dysfunction, cardiac involvement and sclerodactilitis), systemic lupus erythematosus (SLE) (including photosensitivity, nephritis, leukopenia, lymphopenia, thrombocytopenia and Coombs positive hemolytic anemia and positive anti-dsDNA), and secondary sjogren's syndrome (SSS) in the last 7 years before she was admitted to our clinic. The patient fulfilled classification criteria for RA, SLE, PSS and SSS, as determined by American College of rheumatology. hypothyroidism with positive autoantibodies due to Hashimoto's thyroiditis, the beginning of which could not be defined, was coexistent with this overlap syndrome. In the literature, although overlap syndromes in different combinations were reported, we very rarely observed a complex case like this patient. In our opinion, this is the first well-documented case of RA, PSS, SLE, SSS and Hashimoto's thyroiditis existing together in the same patient. Although immunosuppressive therapy was administered, the disease rapidly deteriorated and the patient died.- - - - - - - - - - ranking = 1keywords = hemolytic (Clic here for more details about this article) |
| | Next -> |