Pulmonary Fibrosis as an Unusual Clinical Manifestation of a Pulmonary-Renal Vasculitis in Elderly Patients

doi:10.1016/S0025-6196(12)62575-0

Mayo Clinic Proceedings

Volume 65, Issue 6, June 1990, Pages 847-856

https://doi.org/10.1016/S0025-6196(12)62575-0 Get rights and content

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.

Section snippets

PATIENTS AND METHODS

Between 1978 and 1988, three patients examined at the Mayo Clinic had an initial diagnosis of idiopathic pulmonary fibrosis and subsequently were correctly diagnosed as having a pulmonary-renal vasculitis. Their medical records were reviewed, and their histories were abstracted. Laboratory determinations were performed by routine techniques in the Mayo Medical Laboratories. Determinations of antineutrophil cytoplasmic antibodies (ANCA) were performed on stored serum samples by indirect

Case 1.

A 71-year-old man with a past medical history of chronic obstructive pulmonary disease sought medical attention because of a 1-month history of progressive dyspnea, fatigue, myalgias, and an 11.5-kg weight loss. His blood pressure was 145/80 mm Hg, and bilateral dry crackling rales were present. No clubbing of the fingers was noted. A chest roentgenogram disclosed bilateral basilar interstitial infiltrates and pleural effusions. Thoracentesis yielded a fluid that was consistent with an

RESULTS

All three cases described herein involved elderly patients who had predominantly respiratory complaints of progressive dyspnea and nonproductive cough; fatigue and weight loss were other common findings (Table 1). Fever, serositis, arthralgias, and myalgias occurred infrequently even though the duration of the illness was generally long. Dermatologic, gastrointestinal, and neurologic manifestations were absent. On physical examination, all three patients had bilateral pulmonary dry crackling

DISCUSSION

The clinical, radiologic, and functional findings in the three patients described in this report are consistent with an interstitial pulmonary process. A presumptive diagnosis of idiopathic pulmonary fibrosis was made. Idiopathic pulmonary fibrosis is characterized by progressive dyspnea on exertion and nonproductive cough, dry basilar rales, bilateral interstitial infiltrates, and a restrictive pattern in conjunction with reduced lung volumes and diffusing capacity for carbon monoxide,13, 14,

ACKNOWLEDGMENT

We are indebted to Dr. J. Charles Jennette and Dr. Ronald J. Falk, Chapel Hill, North Carolina, for the determinations of anti-neutrophil cytoplasmic antibodies.

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Pulmonary Fibrosis as an Unusual Clinical Manifestation of a Pulmonary-Renal Vasculitis in Elderly Patients

Section snippets

PATIENTS AND METHODS

Case 1.

RESULTS

DISCUSSION

ACKNOWLEDGMENT

Mayo Clin Proc

Kidney Int

Am J Kidney Dis

Kidney Int

Clin Chest Med

Clin Geriatr Med

Chest

Am J Med

Am J Med

Clin Chest Med

Am J Kidney Dis

Hum Pathol

Lancet

The spectrum of vasculitis: clinical, pathologic, immunologic, and therapeutic considerations

Ann Intern Med

Pulmonary vasculitis

Am Rev Respir Dis