Case Presentation: A 76 year old male with past medical history of hypertension, hepatitis C, and unprovoked pulmonary embolism presented to an outside hospital with multiple complaints. He first noticed fatigue over weeks and then developed fevers for one week. He also endorsed several days of pain and swelling of bilateral lower extremities and severe pain, swelling and redness of his ears, left greater than right. At the outside hospital he was found to have leukocytosis of 18,000 cells/m3 and fever to 102 degrees F. He started on broad spectrum antibiotics for presumed sepsis secondary to otitis media versus bilateral lower extremity cellulitis. After five days of antibiotics and negative infectious work-up, the patient had no improvement in symptoms and continued having fevers. During his hospitalization he developed acute inferior hemifield vision loss of his right eye and sharp left sided temporal headaches. He was transferred to our tertiary hospital for ENT and ophthalmology evaluation. Upon transfer, vital signs were notable for temperature 100.5 degrees F and heart rate 105 beats/min. On examination, bilateral pinnae were erythematous, swollen and warm with severe tenderness to palpation. He also had tenderness over the cartilaginous portions of the nasal bridge and left temporal region, along with hoarseness of voice. His lower extremities were slightly erythematous and bilateral ankle joints were painful to touch. On limited visual exam, he had visual field loss in the right inferior medial quadrant. Labs were significant for erythrocyte sedimentation rate > 140 mm/hr, C reactive protein 167 mg/L and white blood count 17,000 cells/m3.We had high clinical suspicion for temporal arteritis given the patient’s left sided temporal pain, visual changes and high inflammatory markers. High dose steroids were empirically started which led to marked improvement in symptoms within 24 hours. Rheumatology was consulted and raised concern for an underlying diagnosis of relapsing polychondritis (RPA), given the constellation of symptoms including swelling of the ears and nasal bridge. However, temporal arteritis still remained on the differential and the patient underwent a temporal artery biopsy, which was negative. About one week after admission to our hospital, the patient’s symptoms, except hoarse voice and stable right visual field loss, resolved on a steroid taper. He was discharged home on prednisone with outpatient rheumatology follow up.
Discussion: In the setting of hospital medicine, many patients appear to meet “sepsis” criteria, but after thorough review of their history and physical examination, a different underlying diagnosis becomes evident. Our patient initially seemed to have cellulitis given fever, leukocytosis and erythema. However, after no improvement on antibiotics and odd presentation of bilateral ear and leg swelling, our suspicion grew for a unifying inflammatory process to explain his symptoms. RPA is a rare autoimmune disease that targets cartilaginous tissue, characteristically the ears, but can involve any organ including nose, eyes, airways and skin. Etiology is unknown and treatment includes steroids with or without disease modifying agents. Prognosis is generally favorable but information is lacking given the rarity of the disease.
Conclusions: We hope to raise awareness regarding the varying clinical manifestations of RPA and the benefit that steroids may provide after ruling out infectious etiology.