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)

A case of mucoepidermoid carcinoma in thymus in a 59-year-old Japanese female is presented. She died of cardiac tamponade due to tumor invasion ater a 5 years' clinical course. At autopsy the main tumor was found in the thymic region with metastases to the sternum, regional lymph nodes, pericardial, and left pleural cavity. The mucoepidermoid carcinoma might be probably originated from a hen's egg-sized cyst which was located in the upper posterior aspect of the tumor-involved thymus. No teratomatous components were present. The cyst was most likely to be of thymic or bronchogenic cyst origin, though it was not determined, in view of the lining with pseudostratified ciliated columnar epithelium of the cystic wall and the surrounding with the thymic tissue outside. Moreover, there was thymic hyperplasia with germinal center that was compatible with SLE-like symptoms in her past history and autoimmune nature of the autopsy findings of pulmonary fibrosis.
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PMID:Mucoepidermoid carcinoma of the thymic region. 711 3

Medical records of five patients with scleroderma (SSc), each of whom had pericardial effusion with an estimated volume of more than 200 ml, were reviewed to study the clinical and immunological significance of massive pericardial effusion in SSc. Diffuse SSc (4/5), with a wide area of pigmentation (4/5), flexion contracture (4/5), oesophageal hypomotility (5/5), pulmonary fibrosis (4/5) and autoantibodies to topoisomerase I (3/5) were the common features in this group. High protein, lactate dehydrogenase and low white blood cell count were the characteristics of pericardial fluid. None of the patients had signs of acute pericarditis. Four of the five cases died within 9 months of the diagnosis of pericarditis; two with renal failure, one with cardiac tamponade and another with sudden death. The pericarditis in diffuse SSc, especially in cases with anti-topoisomerase I, may be characterized by a chronic form of pericarditis with poor prognosis, often complicated by renal failure.
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PMID:Massive pericardial effusion in scleroderma: a review of five cases. 876 Dec 3

Erdheim Chester disease is a very rare histiocytic disorder characterised by tissue infiltration by lipid laden histiocytes. The most common presentation is bone pains typically involving the long bones. Over time almost 50% of the patients develop extraosseous involvement. The prognosis depends on the extent and distribution of the extraskeletal manifestations. Cardiovascular involvement is seen in up to 40% of the patients and the most common manifestations are periaortic fibrosis and pericardial involvement. Respiratory distress, extensive pulmonary fibrosis, and cardiac failure are the most common causes of death in these patients. Cardiac tamponade has also been documented to cause death in these patients. We describe a patient of Erdheim Chester disease who presented with recurrent and very rapidly occurring cardiac tamponade in a short duration of time and benefited from timely recognition and management.
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PMID:Recurrent and rapidly occurring pericardial tamponade in Erdheim Chester disease. 2257 39

Spontaneous Pneumomediastinum (SPM) is a benign, self-limiting but potentially fatal condition specially in underlying pulmonary disease. Spontaneous pneumomediastinum is rarely associated with connective tissue related interstitial lung disease. We report a patient of interstitial pneumonia with autoimmune features who presented with acute onset breathlessness and was diagnosed to have spontaneous pneumomediastinum, pneumopericardium, and subcutaneous emphysema. This condition are easily diagnosed by clinical findings like Hamman's crunch and plain chest radiographs in cases with normal underlying lungs. In cases of extensive pulmonary fibrosis like Interstitial Lung Diseases both clinical findings and Plain chest radiographs can be inconclusive. CT of thorax is the gold standard diagnostic modality. Pneumopericardium rarely leads to cardiac tamponade, which requires immediate surgical intervention. We conclude that every case of acute onset breathlessness in a known case of interstitial lung disease may not necessarily be an exacerbation of the underlying lung condition. Physicians need to have a high index of suspicion while treating patients of interstitial lung disease. CT thorax is gold standard diagnostic modality especially in presence of fibrotic lung diseases.
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PMID:Spontaneous pneumomediastinum, pneumopericardium and subcutaneous emphysema in a case of interstitial pneumonia with autoimmune features. 3307 60