Pulmonary Fibrosis as an Unusual Clinical Manifestation of a Pulmonary-Renal Vasculitis in Elderly Patients
| Autor: | J.T. Lie, Vicente E. Torres, Jay H. Ryu, Keith E. Holley, Aaron K. Nada |
|---|---|
| Rok vydání: | 1990 |
| Předmět: |
Male
Vasculitis Pathology medicine.medical_specialty Pulmonary Fibrosis Fluorescent Antibody Technique Lung biopsy Pulmonary function testing Diagnosis Differential Idiopathic pulmonary fibrosis Glomerulonephritis Pulmonary fibrosis medicine Humans Microhematuria Lung Aged Hematuria business.industry Syndrome General Medicine medicine.disease medicine.icd_9_cm_classification Capillaries medicine.anatomical_structure Female business Systemic vasculitis |
| Zdroj: | Mayo Clinic Proceedings. 65:847-856 |
| ISSN: | 0025-6196 |
| DOI: | 10.1016/s0025-6196(12)62575-0 |
| Popis: | Between 1978 and 1988, three patients at our institution had an initial diagnosis of idiopathic pulmonary fibrosis but later were correctly diagnosed as having pulmonary-renal syndrome and microscopic polyarteritis. These cases involved elderly patients with progressive dyspnea and nonproductive cough, bilateral dry crackling rales, bilateral interstitial infiltrates evident on a chest roentgenogram, and restrictive findings on pulmonary function testing. In two patients, lung biopsy specimens were obtained, and an initial diagnosis of nonspecific pulmonary fibrosis was made. All three patients eventually had microhematuria and renal insufficiency. A revised diagnosis of small-vessel pulmonary-renal vasculitis was based on the demonstration of segmental necrotizing glomerulonephritis in renal biopsy specimens in two patients, thrombotic microangiopathy consistent with healed vasculitis on postmortem examination of the kidney in one patient, and subsequent detection of small-vessel vasculitis on review of the two lung biopsy specimens. Anti-neutrophil cytoplasmic antibodies with perinuclear staining on indirect immunofluorescence microscopy were positive in the two patients in whom determinations were performed. The clinical manifestations of vasculitis were notably scarce--no involvement of the skin, nervous system, or gastrointestinal tract; no episodes of fever; and minimal or absent musculoskeletal symptoms. These cases illustrate the importance of a high index of suspicion for the diagnosis of systemic vasculitis in elderly patients and the need to consider a vasculitis in the differential diagnosis of idiopathic pulmonary fibrosis, especially if an active urinary sediment is present. |
| Databáze: | OpenAIRE |
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