A 61-year-old Caucasian man, with a remote eight pack-year smoking history, seasonal allergies, gastroesophageal reflux disease, obstructive sleep apnea, and dyslipidemia presented with pleuritic chest pain and dry cough of five months duration. There was no improvement with two courses of oral antibiotics. There was no evidence of sinusitis, arthralgias, inflammatory arthritis, rash or other symptoms of connective tissue disease. Physical examination revealed a healthy, obese man with a normal set of vital signs and resting room air pulse oximetry of 97%. Chest auscultation was notable for inspiratory crackles at the right lung base. His musculoskeletal and skin examinations were normal. Laboratory studies demonstrated high-positive anti-CCP and anti-Ro (SSA) antibodies, and a weakly positive rheumatoid factor (Table 1). Latent tuberculosis assessment was negative. Thoracic high-resolution computed tomography (HRCT) imaging revealed several nodules and thick-walled cavities predominantly in the right lung (Fig. 1). The patient had a normal nuclear cardiac stress test, a negative ventilation/perfusion scan and normal pulmonary physiology (Table 1). Surgical lung biopsy revealed necrotizing granulomatous inflammation with geographic necrosis, vasculitis and lymphocytic pleuritis (Fig. 2) most compatible with an autoimmune-mediated vasculitis.
After the lung biopsy, treatment with oral corticosteroids (0.5 mg/kg/day prednisone) was initiated and the patient reported rapid clinical improvement in respiratory symptoms. Two months after lung biopsy, as corticosteroids were tapered; the patient developed symmetric inflammatory polyarthritis involving the small joints of the hands, wrists, and feet. Synovitis was identified on physical examination. Plain radiographs of the hands and feet were normal. Injectable methotrexate was initiated and rapidly titrated to 25 mg weekly. Corticosteroids were gradually tapered off over the subsequent three months, and synovitis symptoms, pleurisy, and cough remained quiescent. No new symptoms or signs of systemic vasculitis have developed. A follow-up HRCT, reflecting three months of therapy with corticosteroids and methotrexate demonstrates that the pulmonary cavitary nodularity is gradually improving (Fig. 3). Follow-up autoantibody testing three and six months after initiation of methotrexate was notable for persistent high-positive anti-CCP and low-titer positive RF, and negativity to anti-proteinase-3, anti-myeloperoxidase and C- and P-anti-neutrophil cytoplasmic antibody (ANCA).
Initial high-resolution computed tomographic image demonstrating right lower lobe nodular, cavitary lung disease.