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Query: UMLS:C0019079 (hemoptysis)
 6,129 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two brothers, aged 3 and 6 years, respectively, had their pulmonary conditions diagnosed as idiopathic pulmonary hemosiderosis (IPH). Both boys had severe iron-deficiency anemia, chronic cough, hemoptysis, and exertional dyspnea, and one had recurrent epistaxis. The results of light microscopic lung histopathologic studies in both patients showed numerous hemosiderinladen macrophages and chronic interstitial pneumonitis. No specific patterns of immunofluorescence of the alveolar capillary basement membranes were found. The results of electron microscopic examinations showed intact alveolar and capillary basement membranes and no evidence of electron-dense deposits. The lack of clinical or biochemical evidence for renal disease as well as the absence of serum antinuclear and antibasement membrane antibodies excluded associated autoimmune disorders. Evaluation for milk-protein allergy was negative and neither child demonstrated a clinical response to a milk-free diet. Sequential pulmonary function studies performed over four years showed episodes of acute obstructive airway disease that correlated with pulmonary hemorrhage and mild persistent restrictive lung disease. The results of this family study suggested that some cases of IPH may have a genetic basis.
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PMID:Familial idiopathic pulmonary hemosiderosis. 37 18

Pulmonary hemorrhage is generally due to neoplasm, tuberculosis, necrotizing pneumonia, or bronchiectasis. If these are not found, kidney diseases, including anti-glomerular basement membrane antibody-induced bleeding (Goodpasture's syndrome), should be considered. Hemoptysis in renal disease is more often due to azotemic hypervolemia than immune reaction. Typically linear immunofluorescent patterns along the glomerular and pulmonary alveolar basement membranes must be demonstrated to confirm the diagnosis of Goodpasture's syndrome.
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PMID:Pulmonary hemorrhage in renal disease: Goodpasture's syndrome and other causes. 83 Mar 52

Fourteen cases of anti-GBM antibody-induced RPGN were evaluated retrospectively in terms of renal function improvement and therapeutic risks. Nine men and 5 women (mean age: 55.3 years) were observed over a 9 year period; in three patients, hemoptysis was associated with renal disease (Goodpasture's syndrome). Most of these patients had received combinations of steroid therapy (ST), immunosuppressive drugs (IS) and plasma exchanges (PE). Age, duration of symptoms prior to diagnosis, initial renal function, therapeutic modalities and complications were assessed according to renal outcome: 9 patients (group A, "non-responders") remained on dialysis irrespective of the treatment administered; 5 patients (group B, "responders") recovered renal function. Complications, especially infections, were twice as frequent in group A. Two of the 4 recorded deaths were related to the disease or the treatment. Analysis of clinical and pathological values at the time of entry into the study for both groups indicated that oliguria/anuria, serum creatinine greater than 500 mumol/l and greater than 50% crescents, when associated, were factors predictive of poor renal outcome; in these patients, dialysis may be required except in cases of pulmonary hemorrhage. In all other patients, treatment with ST, IS and PE is recommended. Active hemoptysis necessitates pulse steroids or PE; if absent, further tests (carbon monoxide uptake, bronchoalveolar lavage, lung biopsy) are indicated before use of aggressive therapy.
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PMID:[Anti-basement-membrane antibody mediated, rapidly progressive, glomerulonephritis. Diagnostic and therapeutic strategy based on a retrospective study of 14 cases]. 225 83

Diffuse pulmonary hemorrhage is an uncommon condition that is difficult to differentiate radiographically from diffuse pneumonia or pulmonary edema. The diagnosis should be suspected when a patient has even mild hemoptysis or has one of the diseases known to be associated with diffuse pulmonary hemorrhage. This paper reviews the clinical and radiographic features of diffuse pulmonary hemorrhage and presents a classification scheme depicted as a Venn diagram formed by four overlapping circles representing pulmonary hemorrhage, renal disease, immune complex disease, and antiglomerular basement membrane (anti-GBM) disease. This scheme results in six categories of pulmonary hemorrhage: associated with glomerulonephritis and anti-GBM antibody; associated with renal disease without demonstrable immunologic abnormalities; associated with glomerulonephritis and immune complex disease; associated with immune complex disease without renal disease; associated with anti-GBM antibodies without renal disease; without associated immunologic or renal abnormality. Examples of these disorders are illustrated. Improved clinical-radiographic correlation may lead to earlier diagnosis and treatment of diffuse pulmonary hemorrhage and its causes.
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PMID:Diffuse pulmonary hemorrhage: a review and classification. 315 69

We have reviewed the alveolar hemorrhage (AH) syndromes, defined as immune or idiopathic disorders associated with diffuse microvascular hemorrhage into the acinar portion of the lung. The disorders that are most often associated with AH include antibasement membrane antibodies (ABMA) disease, idiopathic pulmonary hemosiderosis, systemic lupus erythematosus, systemic vasculitides, and idiopathic rapidly progressive glomerulonephritis. An approach to the recognition, diagnosis, and treatment of the AH syndromes has been outlined and several illustrative case studies have been presented. Recognition of AH is not usually difficult, but does require a high index of suspicion, since many disease processes may give rise to hemoptysis with infiltrates on chest roentgenogram. Recognition of AH is aided by careful clinical and laboratory assessment for evidence of extrapulmonary disease; simple hematologic studies such as sequential hemoglobins and iron studies; and measurement of carbon monoxide uptake by the lungs. Early recognition of AH may decrease the likelihood of respiratory failure and end-stage renal disease. The specific etiology of AH is usually determined by clinical examination, serologic assay for ABMA, and percutaneous renal biopsy by immunofluorescence. Open-lung biopsy is required in a minority of cases. High-dose pulse methylprednisolone appears to effectively control AH of diverse etiology. Combined plasma exchange and immunosuppression controls AH in ABMA disease and is the treatment of choice in this disorder. Cyclophosphamide is used for Wegener's granulomatosis, and sometimes in systemic necrotizing vasculitis, in an attempt to prevent irreversible damage to the kidneys.
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PMID:Alveolar hemorrhage syndromes: diffuse microvascular lung hemorrhage in immune and idiopathic disorders. 639 80

We have evaluated long-term pulmonary function in 14 patients who were treated for anti-glomerular basement membrane disease at our institution during the last 17 years. Eight of these patients had evidence of pulmonary involvement, as manifested by hemoptysis, pulmonary infiltrates on chest x-ray film, or anemia. These patients were compared with a control group of 15 patients who had renal disease and who were matched for degree and duration of renal disease, age, smoking history, and method of renal replacement. The following variables were measured in each patient: forced vital capacity, forced expiratory volume in 1 minute, vital capacity, total lung capacity, residual volume, functional residual capacity, single-breath carbon monoxide transfer factor, and single-breath carbon monoxide transfer factor corrected for alveolar volume (KCO). These patients also participated in a graded exercise test and measurements of oxygen uptake, carbon dioxide production, minute ventilation, and oxygen saturation were taken. Patients with anti-glomerular basement membrane disease and a prior history of pulmonary hemorrhage had a significantly reduced KCO (46% +/- 10% v 68.7% +/- 14.7%) compared with the control group. There was no difference in any of the other measured parameters.
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PMID:Antiglomerular basement membrane disease: the long-term pulmonary outcome. 820 60

From a series of 95 patients biopsied for rapidly progressive glomerulonephritis, twelve patients were identified with anti-glomerular basement membrane-mediated renal disease who were also tested for antineutrophil cytoplasmic antibody (ANCA). Six patients had both anti-glomerular basement membrane and ANCA antibodies. Three of the latter six patients had significant extrarenal disease, including severe hemoptysis, while the remaining three patients had only renal disease. The three patients with extrarenal disease had either a myeloperoxidase-positive perinuclear-ANCA (two patients) or a proteinase-3-positive cytoplasmic-ANCA (one patient). Two patients with renal disease alone had a myeloperoxidase-negative and proteinase-3-negative perinuclear-ANCA, and one patient had a proteinase-positive cytoplasmic-ANCA. Renal biopsy in all six patients showed a severe necrotizing and crescentic glomerulonephritis involving 94 to 100% of glomeruli. Renal arteritis was also noted in one perinuclear-ANCA patient. Despite aggressive therapy with steroids, cyclophosphamide, and plasma exchange, two of the six double-antibody patients died and four are on dialysis. We conclude that ANCA is commonly present in anti-glomerular basement membrane-associated disease and believe that this observation may have implications in the serologic evaluation of ANCA- and anti-glomerular basement membrane-positive patients.
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PMID:Coexistent anti-neutrophil cytoplasmic antibody and antiglomerular basement membrane antibody associated disease = report of six cases. 824 8

Among 66 patients with Wegener's granulomatosis, 9 had an intra-alveolar haemorrhage which revealed the disease. The diagnosis was based on dyspnoea (n = 9), haemoptysis (n = 9) and anaemia (n = 9) with a mean haemoglobin level of 8 +/- 1 g/dl. Radiology showed bilateral alveolar infiltrates (n = 9), and numerous siderophages were found either in the alveolar lavage fluid (n = 7/7) or in sputum (n = 2). In every case, the alveolar haemorrhage was accompanied by a rapidly progressive extracapillary glomerulonephritis and by lesions of the upper airways which preceded it by several months or years. All patients received corticosteroids combined, in 8 cases, with cyclophosphamide. The respiratory disease improved rapidly, in contrast with the renal disease which became worse (n = 5). Two patients died in the acute phase of the vasculitis: one of acute renal failure, the other of infectious shock.
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PMID:[Intra-alveolar hemorrhage in Wegener's granulomatosis. Retrospective study of 9 cases]. 851 Nov 24

Five distinct clinical syndromes of pulmonary angiitis and granulomatosis are currently recognized: Wegener granulomatosis, lymphomatoid granulomatosis, necrotizing sarcoid granulomatosis, bronchocentric granulomatosis, and allergic angiitis and granulomatosis (Churg-Strauss syndrome). Patients typically present in middle age with fever, cough, hemoptysis, dyspnea, or chest discomfort. Upper airway involvement such as sinusitis suggests Wegener granulomatosis. Medical renal disease is associated with Wegener granulomatosis and Churg-Strauss syndrome. Asthma may be present in bronchocentric granulomatosis and Churg-Strauss syndrome. Pathologic examination of these entities demonstrates vasculitis, granulomatous inflammation, and parenchymal necrosis. The radiologic manifestations of pulmonary disease are varied, but the most typical appearance is that of multiple nodules or masses that may demonstrate cavitation. Diffuse multifocal air-space opacities with or without cavitation may also be seen. Pulmonary hemorrhage is a well-known presenting manifestation of Wegener granulomatosis and, less commonly, of Churg-Strauss syndrome. Because of the multifocal lung involvement in these diseases, pulmonary metastases and infectious causes are often considered in the differential diagnosis. Affected patients are treated with cytotoxic agents and corticosteroids. The prognosis is variable, depending on the specific syndrome, but may be favorable in the absence of significant complications.
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PMID:Pulmonary angiitis and granulomatosis: radiologic-pathologic correlation. 959 92

The clinical course of 15 patients with Wegener's granulomatosis (WG) and eight patients with microscopic polyangiitis (MPA) from one nephrological clinical center is presented for the period from 1984 to 1993, when testing for antineutrophil cytoplasmic antibodies (ANCA) was gradually introduced into routine clinical practice. We found a high degree of prolonged time periods with symptoms attributable to WG or MPA until the specific diagnosis was made. Nine patients with WG and one patient with MPA had symptomatic prediagnostic periods of more than three years, which extended in one case up to twenty years. In these prediagnostic periods, often even severe flares of vasculitic activity resulted in spontaneous remission without immunosuppressive therapy. One patient on chronic dialysis for four months because of rapidly progressive glomerulonephritis, experienced sufficient spontaneous regain of residual renal function to stay off dialysis for 6 years. Despite a high amount of spontaneous recovery, recurrent flares of disease eventually led to death in those cases without sufficient immunosuppressive therapy. Contrary to long courses of disease, one patient with WG had a fulminate exacerbation of disease with lethal hemoptysis after a prediagnostic period of only three months. Renal disease, respiratory and other symptoms did not occur sequentially, but each could precede the other. We conclude in agreement with published former experience, that WG and MPA show a highly variable spontaneous disease course, that requires extended observational periods for evaluating maintenance therapies.
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PMID:Clinical course and symptomatic prediagnostic period of patients with Wegener's granulomatosis and microscopic polyangiitis. 960 40


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