Acute respiratory failure and pulmonary arteritis without parenchymal involvement: demonstration in a patient with...

A 28-year-old woman with a eight-year history of rheumatoid arthritis presented with a three-day history of dyspnea. Physical and electrocardiographic findings were consistent with pulmonary hypertension. Arterial blood gases revealed a ventilation-perfusion mismatch. Chest roentgenogram was normal. After transient improvement, she suddenly deteriorated and died. At autopsy, a necrotizing pulmonary panarteritis was found without parenchymal involvement by rheumatoid disease. The pulmonary arteries were the only vessels affected. Immunofluorescent staining revealed immunoproteins scattered throughout the vessel walls without localization to the basement membrane. The unique features of the case are discussed in relation to pulmonary hypertension and rheumatoid lung disease in which vascular lesions are usually associated with honeycomb lung. The association between the rheumatoid arthritis and pulmonary vasculitis was probably coincidental.