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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 two siblings with cystic fibrosis and systemic amyloidosis. The major clinical problem in both cases was recurrent respiratory infection with pulmonary fibrosis and bronchiectasis prior to death at ages 20 and 22 years. Findings from postmortem examinations disclosed diffuse amyloidosis. In addition, amyloid infiltration developed in both patients, with enlargement of the thyroid gland, and one required thyroidectomy. An autopsy review of 17 additional cases of cystic fibrosis failed to disclose any other instances of systemic amyloidosis.
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PMID:Systemic amyloidosis in cystic fibrosis. 88 4

A generalized form of primary cutaneous amyloidosis was found in two siblings when microscopic examination was carried out on areas of scattered hypopigmentation. Multiple biopsies from different sites of the skin suggested that the pigmentary disorder was probably a process independent of the amyloidosis. One of the siblings died of interstitial pulmonary fibrosis, congenital pulmonary stenosis and diabetes mellitus. Post mortem examination of the lungs showed the presence of few amyloid deposits in the diffusely fibrotic tissue, suggesting that these were secondary to the chronic pulmonary disease. Because of the absence of amyloid involvement in parenchymatous organs and the absence of cutaneous signs of systemic amyloidosis as macroglosia or petechiae, this case suggests that primary cutaneous amyloidosis is distinctly different from the cutaneous manifestation of primary systemic amyloidosis.
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PMID:Familial generalized dyschromic amyloidosis cutis. 99 96

A 10-year follow-up of a family with X-linked cutaneous amyloidosis confirmed no more than streaks or spots of brown pigmentation of the skin in females but much more varied and severe manifestations in males. These included neonatal colitis, infantile diarrhea, recurrent respiratory infections, corneal dystrophy, photophobia, unruly hair with a frontal upsweep, dry skin, and mottled, muddy-brown pigmentation seen first on the inner thighs and spreading diffusely to the buttocks, trunk, and arms. Amyloid was found in the pigmented skin of adults of both sexes but not in children. An autopsy of a 50-year-old man, subject to recurrent pneumonia, confirmed the presence of amyloid in the skin, but it was not found in other organs. Changes in the lungs were those of late-stage diffuse pulmonary fibrosis. The pattern of inheritance is X-linked, but the pathogenesis remains obscure.
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PMID:X-linked cutaneous amyloidosis: further clinical and pathological observations. 270 73

The association between amyloid and systemic lupus erythematosus is rarely reported. Our patient, a 26-year-old woman with systemic lupus erythematosus, developed steroid-resistant exertional dyspnea and pulmonary fibrosis, without proteinuria or the nephrotic syndrome. Our studies showed that she had pulmonary amyloidosis.
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PMID:Pulmonary amyloidosis associated with systemic lupus erythematosus. 371 59

Rheumatoid arthritis is a multi-system disease. Pulmonary manifestations and complications include pleural disease, pulmonary infections, pneumonitis and interstitial pulmonary fibrosis, bronchogenic carcinoma, arteritis with pulmonary hypertension, obliterative bronchiolitis, bronchiectasis, and amyloidosis. Pulmonary rheumatoid nodules, including rheumatoid pneumoconiosis (Caplan's Syndrome), can result in spontaneous pneumothorax. In this article, the authors present a patient with rheumatoid arthritis and recurrent spontaneous pneumothorax. Through investigation, a bronchopleural fistula caused by a rheumatoid nodule was revealed. The authors also discuss the potential pitfalls caused by a lung nodule in a patient with rheumatoid arthritis, including the overlap with bronchogenic carcinoma and confusion with tuberculosis.
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PMID:Case report: recurrent pneumothorax in a patient with rheumatoid arthritis. 807 34

Either total hip arthroplasty (THA), total knee arthroplasty (TKA) or both were performed in 105 patients from 1981 to 1994. These patients were experiencing severe joint destruction in the lower extremities due to rheumatoid arthritis (RA). These patients were followed for more than 2 years after their last operation. Eighty-six patients were alive and 19 patients had died at the time of follow-up. The 86 living patients were divided into four groups based on the number of replaced joints. Their pre- and postoperative conditions, including such factors as pain, mobility and disability for the quality of life (QOL), were compared. All of the four groups showed some reduction in pain and disability, and an improvement in ambulation after the operations. The 19 deceased patients were classified into two groups, one including those with multiple (three or four) arthroplasties and the other, those with only a small number (one or two). The mean age at death was lower (55.7+/-6.2 years) in patients with multiple arthroplasties than that (69.1+/-7.5 years) in patients with only a small number of arthroplasties. Secondary diseases from RA, such as amyloidosis, spinal injury and pulmonary fibrosis, were found to be the primary cause of death in patients with multiple arthroplasties. The most important finding in this study is that although RA patients with multiple arthroplasties in the lower extremities improved their QOL, they were still afflicted with secondary diseases derived from RA and experienced complications that could shorten their lifespan.
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PMID:Outcomes for patients undergoing one or more total hip and knee arthroplasties. 964 10

A 52-year-old woman was admitted to our department because of fever and dysphasia in November 1994. She had noticed Raynaud's phenomenon, arthralgia, and stiffness in the skin for the past 20 years without receiving specific treatment. A diagnosis of progressive systemic sclerosis (PSS) was made based on the presence of sclerosis of the skin, sclerodactyly, pulmonary fibrosis, and the dilatation of the lower esophagus. It was also confirmed that systemic lupus erythematosus (SLE) overlapped based on the presence of an oral ulcer, polyarthralgia, leukocytopenia, renal dysfunction, positive tests for antinuclear antibodies and anti-DNA antibodies. A gastric biopsy specimen revealed amyloid deposits which showed green birefrigence by Congo red staining under polarized light microscopy. The disappearance of the green birefrigence after potassium permanganate treatment indicated that the deposits consisted of AA amyloid. No amyloid deposits were detected in the rectum or the kidney. The average of the SAA level in active SLE patients is 174 micrograms/ml in our department and the SAA level in the present case was 280 micrograms/ml. Although AA amyloidosis is rarely associated with SLE and PSS, a long-term elevation of SAA level could cause the development of amyloidosis even in the cases of SLE and PSS.
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PMID:[A case of systemic lupus erythematosus overlapping with progressive systemic sclerosis accompanied by deposition of AA amyloid in the stomach]. 1004 19

Interstitial lung disease often starts as a reaction to a causative insult or antigen, such as an inhaled environmental organic or inorganic dust and autoimmune mediated injury. When no underlying cause can be found, the inflammatory change in the gas exchanging parts of the lungs and associated fibrosis is called idiopathic pulmonary fibrosis (cryptogenic fibrosing alveolitis). The common causes of pulmonary fibrosis can be classified as granulomatosus, nongranulomatous, neoplastic, and iatrogenic. There are other multisystem disorders not included in this classification that are associated with interstitial lung disease. Amyloidosis, Behcet disease, Kikuchi disease, and Whipple disease are such multiorgan disorders that involve the lungs.
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PMID:Unusual systemic disorders associated with interstitial lung disease. 1158 78

A patient who presented with dyspnea and suspected interstitial pulmonary fibrosis suffered a fatal pulmonary haemorrhage with no feasible cause for bleeding. Autopsy revealed abundant amyloid deposits in both lungs with a diffuse alveolar septal distribution pattern. Amyloid was also found in the cardiac interstitium and in many vessel walls. Considering the affected organs and the histological characteristics, the deposits were regarded as light chain-type. Amyloidosis, which is generally an uncommon disease, very rarely affects the lung predominantly. Haemorrhagic diathesis is a known complication in amyloidosis patients, although fatal haemorrhage is rare and has not yet been reported solely of pulmonary origin. This report describes an uncommon case of idiopathic systemic amyloidosis mainly manifesting in the lungs. The diagnosis was established after fatal pulmonary haemorrhage caused by vessel impairment due to additional vascular amyloid deposits.
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PMID:Idiopathic systemic amyloidosis primarily affecting the lungs with fatal pulmonary haemorrhage due to vascular involvement. 1855 60

Serum amyloid P component (SAP), also known as pentraxin-2, is a member of the pentraxin protein family with an established relationship to the immune response. In the last century, SAP has been used as a diagnostic marker in amyloidosis diagnosis and patient follow-up. SAP has been thought to have potential for treating and curing amyloidosis and fibrosis diseases. More recently, it has been shown that SAP may serve as both a diagnostic marker and a therapeutic target for many immune-related diseases, such as cardiovascular, pulmonary, nephritic, neurological and autoimmune diseases. In the cardiovascular system, SAP has been defined as the culprit in amyloidosis in the heart. SAP may also exert a protective role during the early stage of atherosclerosis and myocardial fibrosis. In noncardiovascular system diseases, SAP is being developed for the treatment of pulmonary fibrosis. In this review, we summarize SAP history, structure, and its roles in immune-related diseases in different systems with emphasis on the cardiovascular system.
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PMID:SAP: structure, function, and its roles in immune-related diseases. 2582 3


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