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)

We report a 52-years-old female patient with collagen disease and pulmonary hypertension. Denopamine, beta-adrenergic agonist, decreased her high pulmonary arterial pressure and improved dyspnea on exercise after long term use. She had suffered from Raynaud's phenomenon, pulmonary fibrosis, shortening of lingual frenulum and positive ANA and RA test. Although her pulmonary fibrosis had been well controlled by azathioprine, dyspnea on exercise became worse, so she admitted to our hospital for further examination in Feb 1988. Right heart catheterization revealed her high pulmonary arterial pressure (mean 29 mmHg). Under right heart catheterization, denopamine markedly decreased her pulmonary arterial pressure and increased her cardiac output. After about 6 weeks' use of denopamine, her mean pulmonary arterial pressure decreased to 15 mmHg, PO2 increased from 43 to 62 mmHg and dyspnea improved. Denopamine has been regarded as a selective beta 1-adrenergic agonist. In this case, denopamine might have beta 2-agonist effect to dilate pulmonary vasculature, or have secondary effect to increase PO2 by the improvement of cardiac function. Denopamine might be useful for pulmonary hypertension with collagen diseases.
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PMID:[Denopamine responsive pulmonary hypertension in a patient with collagen disease]. 257 12

Twenty-four cases of polymyositis, 3 (12.5%) of them Dermatomyositis, have been treated and followed up for over 12 years. Majority (75%) were males. Inflammatory lesion was the cause in 18 (75%), collagen disease in 4 (16.7%) and malignancy in 2 (8.35%). Presenting features were fever (100%), proximal muscle weakness (95.8%) and tenderness (54.2%), facial and respiratory muscle weakness (4.2%). Raised CPK and transaminases, electromyogram abnormality and positive muscle biopsy were recorded in all. All were treated with steroids. Complication/associations noted were arthalgia (25%), dysphagia (20.6%), peripheral neuritis (8.35%), diabetes mellitus (4.2%), pulmonary fibrosis (4.2%) and malabsorption (4.2%). Fourteen cases (58%), all of inflammatory aetiology, recovered completely. Seven cases (29.2%) developed permanent atrophy of affected muscles. Cases with collagen disease and malignancy fared worse and deteriorated because of the primary disease.
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PMID:Polymyositis--a review and follow up study of 24 cases. 800 72

Endostatin is an angiogenesis inhibitor that is an endogenously produced proteolytic fragment of type XVIII collagen. Serum levels of endostatin have been studied extensively in patients with malignant diseases. Recently, elevated serum endostatin levels were observed in patients with systemic sclerosis accompanying pulmonary fibrosis. To determine whether elevated serum endostatin can be observed in patients with idiopathic pulmonary fibrosis (IPF), we measured serum levels of endostatin in 69 patients with benign respiratory disease using an ELISA kit. The median of the serum endostatin levels in these patients was 50.8 pg/mL. Seven of 11 patients (63.6%) with collagen disease-associated pulmonary fibrosis (CDPF), and 19 of 24 patients (79.2%) with IPF had higher serum endostatin levels than the median level of the 69 patients. There was no statistical difference in serum endostatin levels between the patients with IPF and those with CDPF (P=0.7898). Serum endostatin levels in 24 patients with IPF were significantly higher than those in 34 patients with respiratory diseases other than IPF and CDPF (P=0.0001). Elevated serum levels of endostatin were observed in patients with IPF. Although the mechanisms are unclear, elevated serum levels of endostatin may be related to the fibrosing process in the lung.
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PMID:Increased serum levels of endostatin in patients with idiopathic pulmonary fibrosis. 1602 79

The possible causal connection between systemic scleroderma and malignant neoplasms is a controversial issue. In the case of dermatomyositis, a malignant tumor is viewed as a possible trigger of the collagen disease; in contrast, scleroderma is suspected of causing tumors because of the long-term tissue fibrosis. A 68-year-old woman presented with acral scleroderma but already had metastatic bronchial carcinoma without evidence for previous pulmonary fibrosis. The rapid spread of the tumor and the sudden appearance of scleroderma suggest that in this case scleroderma could represent a paraneoplastic syndrome. Attention is directed to several growth factors and cytokines. On the one hand they play a part in the neoplastic transformation of the host cells and on the other hand they can change fibroblasts into a profibrotic phenotype and induce fibrosis. Impressive in this case is the fact that acral scleroderma definitely developed after the malignant tumor but before treatment, so that both fibrosis of the lung and side effects of chemotherapy and radiation can be excluded as triggers.
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PMID:[Acral scleroderma presenting simultaneously with small-cell bronchial carcinoma: a paraneoplastic disease?]. 1635 Oct 15

CA19-9 is a specific tumor marker in patients with gastrointestinal cancer; however, some patients with respiratory disease can have elevated serum levels of CA19-9 as well. In this study we evaluated serum CA19-9 levels of patients with nonmalignant respiratory diseases. We also estimated the prognostic significance of elevated serum levels of CA19-9 in patients with interstitial lung diseases. The study included 554 patients who had been diagnosed at our hospital during the period of 1984-2005. Serum CA19-9 levels in these patients were measured with a commercially available kit. Elevated levels (>37 U/mL) of CA19-9 were observed in 30.7% of patients with lung cancer. Furthermore, 38.9% of patients with idiopathic interstitial pneumonia (IIP), collagen disease-associated pulmonary fibrosis (CDPF), diffuse panbronchiolitis (DPB), and bronchiectasis had elevated serum CA19-9 levels. Survival rates were significantly lower in patients with interstitial lung diseases (IIP and CDPF) and elevated serum CA19-9 levels than in those with levels in the normal range (P=0.0065). Serum CA19-9 was elevated in some patients with nonmalignant diffuse lung diseases. Therefore, clinicians should pay attention to the evidence that increased serum CA19-9 levels can be found in nonmalignant respiratory disease patients. In patients with IIP and CDPF, elevated serum CA19-9 levels may be related to poor prognosis.
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PMID:Serum levels of CA19-9 in patients with nonmalignant respiratory diseases. 1738 65

Carbohydrate antigen 19-9 (CA19-9) is a specific tumor marker of the biliary, pancreatic and gastrointestinal tracts. CA19-9 is occasionally elevated in serum in patiens with benign pulmonary diseases such as bronchiectasis, idiopathic interstitial pneumonia or collagen disease-associated pulmonary fibrosis. Intralobar pulmonary sequestration is an uncommon congenital lung anomaly. It is dissociated from the normal tracheobronchial tree and is supplied by an anomalous systemic artery. There have been some reports of elevation of CA19-9 in this lesion. We report a case of intralobar pulmonary sequestration with elevated serum CA19-9 in a 29-year-old man who was diagnosed with bronchiectasia of left lower lung field on general check up. He had no evidence of any malignant disease in pancreatobiliary or gastrointestinal tracts. Elevated serum CA19-9 level might be encountered with benign pulmonary disease such as pulmonary sequestration.
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PMID:Intralobar Pulmonary Sequestration Showing Increased Serum CA19-9. 2310 Oct 18

Pulmonary alveolar proteinosis (PAP) is characterized by the accumulation of periodic acid-schiff stain-positive lipoproteinaceous materials in the alveolar space due to impaired surfactant clearance by alveolar macrophage. Autoimmune PAP is the most common form of PAP, but rarely accompanies collagen disease or sarcoidosis. We report here a rare case of autoimmune PAP preceded by systemic sclerosis and sarcoidosis. A 64-year-old woman was admitted to our hospital for blurred vision, muscle weakness of extremities, Raynaud's phenomenon, and exertional dyspnea. We diagnosed her as having systemic sclerosis complicated with sarcoidosis. Chest computed tomography (CT) and transbronchial lung biopsy showed the findings of pulmonary fibrosis without PAP. We treated her with corticosteroid and intravenous cyclophosphamide therapy, followed by tacrolimus therapy. Thereafter, her symptoms improved except for exertional dyspnea, and she began to complain of productive cough thirteen months after corticosteroid and immunosuppressant therapy. On the second admission, a chest CT scan detected the emergence of crazy-paving pattern in bilateral upper lobes. Bronchoalveolar lavage (BAL) fluid with milky appearance and a lung biopsy specimen revealed acellular periodic acid-schiff stain-positive bodies. The serum titer of anti-granulocyte macrophage colony stimulating factor (GM-CSF) antibodies was elevated on first admission and remained high on second admission. We thus diagnosed her as having autoimmune PAP. Reducing the dose of immunosuppressive agents and repeating the segmental BAL resulted in the improvement of her symptoms and radiological findings. Immunosuppressant therapy may trigger the onset of autoimmune PAP in a subset of patients with systemic sclerosis and/or sarcoidosis.
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PMID:Elevated Serum Anti-GM-CSF Antibodies before the Onset of Autoimmune Pulmonary Alveolar Proteinosis in a Patient with Sarcoidosis and Systemic Sclerosis. 2896 13