SESSION TITLE: Miscellaneous Student/Resident Case Report Posters III
SESSION TYPE: Medical Student/Resident Case Report
PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM
INTRODUCTION: Pulmonary involvement in relapsing polychondritis (RP) is usually manifested by episodic tracheo-bronchial chondritis in almost 60% of the patients as the disease progresses, resulting in life-threatening upper airway obstruction. About 30% of the patients have additional autoimmune disorders, most commonly connective tissue diseases and vasculitides. So far, only one case of coexisting relapsing polychondritis and sarcoidosis has been reported in an immunocompetent host. We report an unusual observation of coexisting RP and sarcoidosis. Physicians should be aware of this possible association.
CASE PRESENTATION: A 46 year-old african-american female, a non-smoker with relapsing polychondritis (RP) was admitted with progressive dyspnea and dry cough for 2 months. She had been taking her prednisone and cyclophosphamide for her RP, but has been off her medications for 3 months. She was afebrile with normal vital signs and had a normal pulmonary exam. Evidence of recurrant nasal and auricular cartilage inflammation and damage was noted on exam. Initial laboratory tests revealed mild leukocytosis and normal liver and kidney function. CT scan of the chest revealed multiple new lung nodules. Work-up revealed no evidence of infections or vasculitis (Table 1). PFT was suggestive of fixed airway obstruction and bronchoscopy revealed tracheal stenosis and dynamic airway collapse. Trans-bronchial biopsy of the nodules showed non-necrotizing granuloma without any evidence of infection or malignancy. Based on her CT findings, laboratory work up and biopsy results, a diagnosis of nodular sarcoidosis was made and the patient was treated with steroids with remarkable improvement. At 3 month follow up she remains completely asymptomatic with immense clinical and radiological improvement (Fig 1) and is currently off steroids.
DISCUSSION: Pulmonary involvement in RP is usually limited to airway involvement owing to recurrent laryngo-tracheal chondritis. Parenchymal nodules have not been described as a manifestation of RP. Nodular sarcoidosis in association with RP is very rare and this is the second case in an immunocompetent host to be reported in literature. It is important to be aware of such an association, as early diagnosis with timely treatment can make a huge difference.
CONCLUSIONS: It is unusual to have pulmonary nodular involvement as a manifestation of RP and we should initiate a thorough work-up in such cases. Once, infections and malignancy have been ruled out, in the correct clinical setting, it is important to consider nodular sarcoidosis as part of the differential diagnosis.
Reference #1: Relapsing polychondritis in the Department of Defense population and review of the literature. Seminars in Arthritis & Rheumatism. 2012; 42(1):70-83
Reference #2: Coexisting relapsing polychondritis and sarcoidosis: an unusual association. Rheumatology International. 2010; 30(11):1507-9
Reference #3: Pulmonary manifestations of relapsing polychondritis. Clinics in Chest Medicine. 2010; 31(3):513-8
DISCLOSURE: The following authors have nothing to disclose: Amratash Malodiya, Michael Vest, Gerald O'Brien
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