Cases reported "Raynaud Disease"

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1/16. Acute congestive heart failure associated with a limited form of systemic sclerosis and primary biliary cirrhosis.

    This is the first case of a limited form of systemic sclerosis (ISSc) associated with acute congestive heart failure (CHF) and primary biliary cirrhosis (PBC). A 58-year-old woman with ISSc was admitted because of a sudden onset of CHF. The intravenous administration of nitroglycerine and furosemide ameliorated the symptoms of CHF within 24 hours. She had both anticentromere antibodies and anti-p25 doublet/triplet antibodies to intrahepatic microsomes. thallium scintigraphy at rest demonstrated significant perfusion defects in both the anteroseptal and inferior myocardium. A coronary angiogram revealed normal coronary arteries and no vasospasm was provoked by the intracoronary administration of acetylcholine. The present case indicates that minute care should thus be taken for the prevention of acute CHF even in patients with a limited form of SSc when thallium perfusion defects are identified.
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2/16. Primary pulmonary hypertension with severe systemic hypertension, Raynaud's phenomenon and relative polycythemia.

    A 32-year-old Japanese man was hospitalized for evaluation of unconsciousness. He was diagnosed as having primary pulmonary hypertension (PPH) with severe systemic hypertension, Raynaud's phenomenon and relative polycythemia. Hemostatic studies revealed increased coagulation and decreased fibrinolysis, similar to findings of chronic disseminated intravascular coagulation (DIC). Although activation of coagulation and systemic hypertension were improved after treatment with phlebotomy and administration of nifedipine, enerapril and warfarin, pulmonary hypertension was unchanged, suggesting that irreversible change had already occurred in the pulmonary arteries. Those complications could modify the process of pulmonary hypertension by inducing a hyperviscosity state.
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3/16. Vascular events associated with alpha interferon therapy.

    Alpha Interferon (IFN) is a biological agent used for the therapy of an increasing number of diseases, either as an established effective therapeutic tool or in the context of clinical trials. The use of IFN may be complicated by serious adverse reactions. We describe here the clinical course of a variety of vasculopathic complications in association with IFN-therapy in 12 patients with the diagnosis of chronic myeloid leukemia and 1 patient with malignant melanoma treated at our institute. Vascular manifestations in these patients include Raynaud's phenomena, digital ulcerations and gangrene, pulmonary vasculitis, pulmonary hypertension and thrombotic thrombocytopenic purpura/hemolytic uremic syndrome (TTP/HUS). These reactions occurred after 3 months to 3 years of 3-10 million units (MU) daily IFN therapy. Concomitant administration of hydroxyurea (HU) was noted in 5 patients. Discontinuation of IFN and initiation of immunosuppressive therapy brought about a complete resolution or arrested progression of these reactions. IFN-therapy may be complicated by severe vasculopathic/vasospastic complications that usually improve after its discontinuation. Possible underlying mechanisms for these complications are discussed. The early diagnosis of these complications may be vital and IFN should be immediately discontinued when early signs of these complications become evident.
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4/16. warfarin-associated multiple digital necrosis complicating heparin-induced thrombocytopenia and Raynaud's phenomenon after aortic valve replacement for adenocarcinoma-associated thrombotic endocarditis.

    necrosis of the digits is a rare complication of warfarin therapy of obscure pathogenesis. We report a 61-year-old woman with a 12-month history of Raynaud's phenomenon who developed multiple digital necrosis following aortic valve replacement with mechanical prosthesis for aortic insufficiency caused by nonbacterial thrombotic endocarditis. Exacerbation of Raynaud's phenomenon occurred during the postoperative period, with daily episodes of ischemia of the fingers and toes that improved with local warming. However, coincident with the occurrence of immune heparin-induced thrombocytopenia, and while undergoing routine warfarin anticoagulation because of the mechanical valve prosthesis, the patient abruptly developed progression of digital ischemia to multiple digital necrosis on postoperative day 8, at the time the international normalized ratio reached its peak value of 4.3. All limb pulses were readily palpable, and vascular imaging studies showed thrombosis only in the superficial femoral and popliteal veins of the right leg. Coagulation studies showed greatly elevated levels of thrombin-antithrombin complexes and prothrombin fragment F1.2 levels, consistent with uncontrolled thrombin generation. After vitamin k administration, no abnormalities of the protein c anticoagulant pathway were identified, consistent with previous studies of other patients with warfarin-induced necrosis complicating heparin-induced thrombocytopenia. Subsequently, the patient was shown to have metastatic breast adenocarcinoma, which explained the patient's initial presentation with nonbacterial thrombotic endocarditis. This patient case suggests that multiple digital gangrene can result from the interaction of various localizing and systemic factors, including compromised microvascular blood flow (Raynaud's phenomenon), increased thrombin generation (heparin-induced thrombocytopenia, adenocarcinoma), and warfarin-induced failure of the protein c natural anticoagulant pathway.
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5/16. Low-titer cold agglutinin disease with systemic sclerosis.

    A 60-year-old woman with systemic sclerosis (SSc) was admitted because of severe anemia and Raynaud's phenomenon. Her anemia was associated with a low serum haptoglobin level and positive results of direct Coomb's tests, which indicated the presence of autoimmune hemolysis. Other laboratory investigations revealed positive anti-nuclear antibodies, anti-topoisomerase antibody, cold agglutinins, as well as low serum levels of IgM, C3, C4 and CH50. bone marrow aspiration showed discrete hyperplasia of the erythropoietic system. She was diagnosed as low-titer cold agglutinin disease rousing secondarily to SSc. The anemia was alleviated with the oral administration of prednisolone. This case emphasized, in terms of pathogenesis, the close association between systemic rheumatic diseases and hematological abnormalities evoked by autoimmunity.
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6/16. Acral necrosis after inadequate excessive administration of bleomycin in a testicular cancer patient.

    BACKGROUND: Testicular cancer has a favorable prognosis in the majority of patients due to the excellent susceptibility to chemotherapy with cisplatin, etoposide and bleomycin (BEP), which is commonly administered over 3-4 cycles of treatment. CASE REPORT: A 22-year-old male failed to achieve complete response after unconventional treatment with 6 courses of BEP for intermediate-risk metastasized testicular cancer. The patient developed chemotherapy-induced digital necrosis and substantial loss of digital function after this excessive treatment. This condition resolved with infusional alprostadil combined with oral clonidin and pentoxifyllin. RESULT: Infusional alprostadil adds substantial clinical benefit to combined vasoactive therapy in chemotherapy-induced vascular toxicity, even after the onset of digital necrosis.
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7/16. Severe hypertriglyceridaemia in idiopathic (non-HCV-related) mixed cryoglobulinaemia.

    We describe a 28-year-old male patient in whom the development of idiopathic mixed cryoglobulinaemia (MC) with typical clinical manifestations such as polyarthralgia, weakness, purpura, and Raynaud's phenomenon was associated with a remarkable increase in serum triglycerides (TG). prednisone administration was effective in classical cryoglobulinaemia symptoms and also in dyslipidaemia. Infective, autoimmune, and haematological disorders are often related to cryoglobulinaemia and it has been analysed that some of them also show an increase in TG.
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8/16. Effects of prostaglandin E1 on collagen diseases with high levels of circulating immune complexes.

    Prostaglandin E1 (PGE1) was administered to 4 patients with collagen diseases presenting with high levels of circulating immune complexes (CIC) in sera. Our study patients had progressive systemic sclerosis, systemic lupus erythematosus, polyarteritis nodosa, and rheumatoid arthritis. In all 4 patients, CIC levels significantly decreased after administration of PGE1 by continuous infusion at 10/ng/kg/min via central venous catheter for 72 h. In addition, the skin ulcer in a patient with PSS healed completely, and the finger necrosis in a patient with RA improved. These results suggest that PGE1 given by continuous venous infusion is effective in reducing CIC, in addition to improving peripheral vascular disorders.
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9/16. The protective effect of auto-immune buccal urine therapy (AIBUT) against the Raynaud phenomenon.

    The efficacy of Auto-Immune Buccal urine Therapy (AIBUT) against allergic symptoms depends upon sublingual administration of the correct dose of urine as determined by bio-assay in individual patients. Succeeding effective turn-off doses occur at the troughs of a sinusoidal dose-response curve. Efficacy of the administered dose is confirmed by reduction in the severity and duration of Cold-water-induced Raynaud symptoms after administration of effective doses of unboiled urine in AIBUT. Boiled urine does not affect the Raynaud phenomenon.
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10/16. Collapse during operation following i.v. ergometrine. A case report.

    Peripheral pulses became impalpable following the administration of ergometrine during Caesarean section. The subsequent clinical course suggested sensitivity to ergometrine in a patient with Raynaud's disease.
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