Relapsing polychondritis presenting in a patient with cryptogenic organizing pneumonia

Abstract

Relapsing Polychondritis (RP) is an autoimmune disease that affects cartilaginous tissues in the body, including lower respiratory tract. Involvement of the pulmonary interstitium is very rare. We present a 67-year-old Caucasian man presented to the clinic with one-year of recurrent dyspnea and dry cough along with worsening anemia and thrombocytopenia. CT of the chest demonstrated diffuse, bilateral (left > right) multifocal peribronchovascular ground glass opacities with mediastinal lymphadenopathy. All rheumatologic serology workup was negative as well as infectious workup. Lung biopsy of the left lower lobe consolidation showed plugs of fibroblastic tissue incorporated into alveolar walls and airspaces consistent with COP. No features of malignancy or granulomatous process were seen. Oral prednisone was started and tapered over the next 3 months with improvement of his respiratory symptoms. Two weeks later, he developed bilateral ear pain/redness and conjunctivitis. CT of the sinuses revealed mild prominence in the left nasopharyngeal soft tissue. Biopsy of this lesion showed inflammatory infiltrative changes and RP was diagnosed. Adalimumab was started. However, the patient developed worsening dyspnea that required supplemental oxygen and hospitalization. High dose steroids were again added, along with oral cyclophosphamide. The patient had significant clinical and radiological improvement over the next 6 months. Interstitial lung disease has been rarely associated with RP. Most of the lower respiratory track involvement is in the trachea. We present a case of cryptogenic organizing pneumonia in association with relapsing polychondritis.