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      A Rare Cause of Pulmonary-Renal Syndrome

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          Diseases affecting both the lung and the kidney have grave prognosis and serious diagnostic and therapeutic consequences. Here, 3 cases of pulmonary-renal syndrome caused by antiphospholipid syndrome are reported. The patients presented with dyspnea, renal insufficiency, pulmonary infiltrates on chest X-ray and areas of ground glass attenuation on computed tomography of the lungs. There were no signs of infectious disease, vasculitis or myocardial insufficiency. Clinical findings, antiphospholipid levels and histological findings in transbronchial and/or renal biopsy proved the diagnosis of antiphospholipid syndrome. Antiphospholipid syndrome is a comparatively rare disorder which is relevant in the differential diagnosis of diseases affecting both lung and kidney and requires specific therapeutic measures.

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          Most cited references 3

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          Catastrophic antiphospholipid syndrome. Clinical and laboratory features of 50 patients.

          We analyzed the clinical and laboratory characteristics of 50 patients with catastrophic antiphospholipid syndrome (APS) (5 from our clinics and 45 from a MEDLINE computer-assisted review of the literature from 1992 through 1996). Thirty-three (66%) patients were female and 17 (34%) were male. Twenty-eight (56%) patients had primary APS, 15 (30%) had defined systemic lupus erythematosus (SLE), 6 (12%) had "lupus-like" syndrome, and 1 (2%) had rheumatoid arthritis. Mean age of patients in this series was 38 +/- 14 years (range, 11-74 yr). Three (6%) patients developed the clinical picture of catastrophic APS under the age of 15 years, and 11 (22%) were 50 years old or more. In 11 (22%) patients, precipitating factors contributed to the development of catastrophic APS (infections in 3, drugs in 3, minor surgical procedures in 3, anticoagulation withdrawal in 2, and hysterectomy in 1). The presentation of the acute multi-organ failure was usually complex, involving multiple organs simultaneously or in a very short period of time. The majority of patients manifested microangiopathy--that is, occlusive vascular disease affecting predominantly small vessels of organs, particularly kidney, lungs, brain, heart, and liver--with a minority of patients experiencing only large vessel occlusions. Thrombocytopenia was reported in 34 (68%) patients, hemolytic anemia in 13 (26%), disseminated intravascular coagulation in 14 (28%), and schistocytes in 7 (14%). The following antibodies were detected: lupus anticoagulant (94%), anticardiolipin antibodies (94%), anti-dsDNA (87% of patients with SLE), antinuclear antibodies (58%), anti-Ro/SS-A (8%), anti-RNP (8%), and anti-La/SS-B (2%). Anticoagulation was used in 70% of the patients, steroids in 70%, plasmapheresis in 40%, cyclophosphamide in 34%, intravenous gammaglobulins in 16%, and splenectomy in 4%. Most patients, however, received a combination of nonsurgical therapies. Death occurred in 25 of the 50 (50%) patients. In most, cardiac problems seemed to be the major cause of death. In several of these, respiratory failure was also present, usually due to acute respiratory distress syndrome and diffuse alveolar hemorrhage. Among the 20 patients who received the combination of anticoagulation, steroids, and plasmapheresis or intravenous gammaglobulins, recovery occurred in 14 (70%) patients. The use of ancrod and defibrotide appeared to be effective in the 2 respective patients in whom they were used.
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            Antiphospholipid antibodies and thrombosis.

            Antiphospholipid antibodies are associated with arterial and venous thrombosis, recurrent pregnancy loss, and thrombocytopenia. Although the antibodies have not been conclusively shown to be causal in thrombosis and miscarriage, they are useful laboratory markers for the antiphospholipid syndrome. The identification of the syndrome is clinically important because of the risk of recurrent thrombosis and the need for antithrombotic therapy in many cases. Diagnosis and treatment of antiphospholipid syndrome is difficult, however, because of the protean clinical manifestations and associations, limitations of existing laboratory tests for antiphospholipid antibodies, and the absence of evidence-based guidance on best management.
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              Thrombotic thrombocytopenic purpura in pulmonary-renal syndromes.

              Thrombotic thrombocytopenic purpura pathologically consists of a thrombotic microangiopathy that classically spares lung tissues. We describe a case of TTP that presented as a pulmonary-renal syndrome. In reviewing the international literature, pulmonary involvement is not as rare as once was thought, and the diagnosis of TTP should be considered in the differential diagnosis of pulmonary-renal syndromes. Copyright 2000 Wiley-Liss, Inc.

                Author and article information

                S. Karger AG
                July 2002
                01 July 2002
                : 91
                : 3
                : 516-520
                aMedizinische Klinik I, und bKlinik für Röntgendiagnostik und interventionelle Radiologie, Universitätsklinik Marienhospital, Ruhr-Universität Bochum; cAbteilung Zelluläre und Molekulare Pathologie am Deutschen Krebsforschungszentrum, Heidelberg; dFreie Universität Berlin, Medizinische Klinik und Poliklinik, Universitätsklinik Benjamin Franklin, Berlin, Deutschland
                64300 Nephron 2002;91:516–520
                © 2002 S. Karger AG, Basel

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                Page count
                Figures: 1, References: 23, Pages: 5
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                Case Report

                Cardiovascular Medicine, Nephrology

                Pulmonary-renal syndrome, Antiphospholipid antibodies


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