Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0034069 (pulmonary fibrosis)
 7,050 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The clinical and serological findings on 24 patients with pulmonary hypertension (PHT) seen at the Lupus Clinic of St. Thomas' Hospital, London are presented. Twenty-two patients had systemic lupus erythematosus (SLE), one other a primary antiphospholipid syndrome and another an SLE/progressive systemic sclerosis (PSS) overlap syndrome. In 21 of the 24 patients, the disease resembled the primary idiopathic variety with clear lung fields and no clinical evidence of pulmonary thromboembolism, although angiography and nuclear perfusion scans were not performed. Two patients clearly suffered from thromboembolic PHT, one with SLE and one with an antiphospholipid syndrome. One patient with SLE/PSS overlap syndrome developed pulmonary fibrosis. The frequency of antiphospholipid antibodies (lupus anticoagulant and antibodies to cardiolipin was 68% which appears to be higher than generally found in patients with SLE, and the clinical significance of this finding is unknown. Other associated features of the antiphospholipid syndrome in this group were uncommon. Death occurred in 13 of the 24 patients, 4 were lost to followup and 7 are known to be alive. The cause of death was circulatory failure in the majority; sudden death once this complication occurred was particularly common. One patient died from adult respiratory distress syndrome and one from hemorrhagic shock while undergoing heart/lung transplantation. Two patients underwent successful heart/lung transplantation. One, however, died of a mesenteric occlusion and bowel infarction following a second lung transplantation because of rejection of the first heart/lung transplantation after one year. The other patient is alive and well 2 years later.
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PMID:Pulmonary hypertension in a lupus clinic: experience with twenty-four patients. 212 32

We report a case of secondary antiphospholipid syndrome (APS) occurring in a progressive systemic sclerosis (PSS) patient who took herbal medication. Clinical findings compatible with APS included positive IgM anticardiolipin antibody (ACL), thrombocytopenia, and obstruction of the left radial artery on digital subtraction angiography (DSA). Clinical findings compatible with PSS included sclerodactyly and digital ulcers, Raynaud's phenomenon, pulmonary fibrosis and pulmonary hypertension, proteinuria and renal mesangial reaction, and myocarditis.
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PMID:Antiphospholipid syndrome associated with progressive systemic sclerosis. 867 27

We report the case of coexistence of five autoimmune diseases in a 36-year-old woman, who initially developed psoriasis. Several years later, the patient was diagnosed with a mixed connective tissue disease and primary biliary cirrhosis (PBC). On admission to the Department of Rheumatology and Connective Tissue Diseases, the patient fulfilled classification criteria of an overlap syndrome systemic lupus erythematosus (SLE) with secondary antiphospholipid syndrome/systemic sclerosis (SSc)/Sjogren's syndrome (SS) with coexisting PBC and psoriasis. The SLE symptoms included discoid lupus erythematosus, arthritis, pancytopenia, antinuclear antibodies and anticardiolipin antibodies. Moreover, the patient met the criteria of antiphospholipid syndrome diagnosed based on preterm delivery before week 34, and high values of anticardiolipin antibodies were found at repeated determinations. The SSc symptoms included sclerodactyly, pulmonary fibrosis with pulmonary hypertension and esophageal dysfunction. The SS syndrome involved xerostomia, xerophthalmia, the positive Schirmer's test and presence of anti-SS antibodies. The literature reports overlap syndromes in various combinations; however, the coexistence of five autoimmune diseases is extremely rare.
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PMID:Coexistence of five autoimmune diseases: diagnostic and therapeutic difficulties. 1832 Jan 93

Pulmonary manifestations of systemic lupus erythematosus (SLE) typically include pleuritis, alveolar hemorrhage, and infectious pneumonia due to immunosuppression with less common entities including bronchiolitis, interstitial pneumonia, and pulmonary fibrosis. More rare manifestations include organizing pneumonia (OP) and diffuse alveolar damage (DAD). A similar but distinct entity of acute fibrinous and organizing pneumonia (AFOP), characterized by intra-alveolar fibrin deposition and associated organizing pneumonia, has been reported in association with connective tissue disorders, but has not been described in association with SLE. Reported herein is a patient with SLE and accompanying antiphospholipid syndrome with recent pulmonary embolism, persistent respiratory symptomology, and persistent radiographic abnormalities who underwent lung biopsy displaying features of AFOP. This case in conjunction with previous literature indicates that AFOP can be a manifestation of connective tissue disease including SLE and may be an underreported variant of medical lung disease due to overlap in histological characteristics with OP and DAD.
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PMID:Acute fibrinous and organizing pneumonia in systemic lupus erythematosus: a case report and review of the literature. 2094 26

Antiphospholipid syndrome (APS) is an acquired prothrombotic condition characterized by vascular thrombosis and/or obstetric complications, in the persistent positivity of circulating antiphospholipid antibodies (aPLs). The clinical spectrum of manifestations associated with aPL positivity is progressively expanding, including the description of several lung manifestations. The most common pulmonary involvement related to aPL positivity is pulmonary embolism (PE), which has been reported to occur in 14.1% of APS patients during disease course. PE acknowledges a purely thrombotic etiology and thus might be accounted as a criterion for APS, making imperative to test aPL in young subjects with unprovoked PE. Pulmonary hypertension (PH) can manifest in APS patients, being the second most common lung complication of the syndrome, with a prevalence ranging between 1.8 and 3.5%. aPL-positive patients might present PH following a PE, might develop pulmonary arterial hypertension associated with connective tissue disease, or might present pulmonary venous hypertension due to Libman-Sacks endocarditis. Additional lung manifestations, such as diffuse alveolar hemorrhage, acute respiratory distress syndrome, and pulmonary fibrosis, are rarely described in APS patients; it is still not clear whether in these settings aPLs exert a pathogenic role or is a mere epiphenomenon. Hereby we discuss impact, clinical presentation, histopathologic findings, etiology, and treatment of each aPL-associated lung manifestation.
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PMID:Lung Disease in Antiphospholipid Syndrome. 3113 66