INTRODUCTION:Multiple lung complications may be seen in patients with rheumatoid arthritis caused either by infections in the setting of immunosuppressive therapy, side effects of the medications used or the effect of the main disease process itself. We are describing here a less common lung involvement seen in patients with rheumatoid arthritis.
CASE PRESENTATION:A 42 year-old-male previously diagnosed with rheumatoid arthritis, presented to the hospital with complaints of fevers, chills, night sweats, productive cough, shortness of breath and a ten pounds weight loss. He has been treated for the last two years with methotrexate 75mg weekly and prednisone 5mg daily. The physical examination revealed an acutely ill looking male. His temperature was 102F, oxygen saturation 88% on room air, respiratory rate 28/min, heart rate 110/min and blood pressure 90/50 mmHg. There was no evidence of septal perforation or polychondritis. The lung examination revealed bilateral coarse rhonchi. The cardiovascular and abdominal examination was normal. He had purpuric lesions on both lower extremities. The neurological exam did not show any focal deficits. The complete blood count showed white count of 14,000 (normal; 4,000-11,000) with 86% neutrophils. His hemoglobin and platelets were normal. The erythrocyte sedimentation rate was 111 mm/hr (normal; 0-15 mm/hr) and the rheumatoid factor was 500 IU (normal; 0-20 IU). His chest x-ray and CT scan (Fig 1) showed bilateral nodular infiltrates with cavitations. There was no significant hilar or mediastinal lymphadenopathy. Sputum cultures showed normal respiratory flora. Bronchoscopy with bronchoalveolar lavage and transbronchial lung biopsy was done. All the cultures obtained during bronchoscopy including acid fast cultures and fungal cultures were negative. The pathology of the transbronchial biopsy specimens showed (Fig 2) prominent suppurative necrosis, surrounded by granulomatous reaction with vasculitis. These findings were consistent with Wegener’s granulomatosis. His C-ANCA was ordered and was found to be positive. Patient was started on intravenous cyclophosphamide with significant improvement of his symptoms.
DISCUSSIONS:The association of rheumatoid arthritis and Wegener’s disease is extremely rare. The review of medical literature since 1966 showed only six cases. All the patients described were female; the median age was 43y. In all the cases the rheumatoid arthritis preceded the onset of Wegener’s by seven to nine years. Five patients had upper respiratory involvement and four of them had lower respiratory involvement. Three of the patients had a positive ANCA. No significant difference in survival was seen in these patients compared with the patients with Wegener’s disease alone.
CONCLUSION:Rheumatoid arthritis and Wegner’s granulomatosis have traditionally been considered as separate autoimmune disease entities. However, our case emphasizes the possibility of overlapping syndromes.
DISCLOSURE:Ioana Amzuta, No Financial Disclosure Information; No Product/Research Disclosure Information