Case Presentation: A 71-year-old man presented with 2 months of progressive diffuse weakness and myalgias to the point of not being able to ambulate without a cane. He noticed lumps on his left forearm during that time, which improved spontaneously. He also endorsed photophobia, right eye swelling, and pain worsened by extraocular movement. He had left ear swelling and pain worsened by contact on presentation. Additionally, he had constant aching of the throat with negative strep and flu tests. He denied joint pain, stiffness and swelling, fevers, and cough. He had history of multiple emergency department visits during the 2 months prior to presentation.On presentation, vital signs were within normal limits. He had minimal conjunctival congestion and some eyelid erythema and swelling. His left upper auricle was erythematous, swollen, and tender to palpation. Fundoscopy showed retinal hemorrhages and cotton wool spots in the right eye. Flexible laryngoscopy showed mild erythema throughout the glottis. C-reactive protein was 10.5 mg/dL (normal 0-0.9 mg/dL), erythrocyte sedimentation rate was 114 mm/hr (normal 0-11 mm/hr), and rheumatoid factor was 26 IU/mL (normal <14 IU/mL). Anti Sm, anti RNP, anti SSA, anti SSB, anti-cyclic citrullinated peptide IgG, and anti-neutrophil antibodies were negative. He was diagnosed with relapsing polychondritis based on clinical features and was treated with high dose steroids. He was discharged with rheumatology follow up after near resolution of symptoms
Discussion: Relapsing polychondritis can involve nearly every organ system. The most common presenting feature is ear inflammation, which appears in up to 90% of patients (Kent, et al). Ocular manifestations occur in up to 60% of patients during the course of disease. Greater than 50% of patients develop laryngotracheal disease. Skin findings occur in only 25% of patients. Symptoms may also involve the nose, joints, heart, kidneys, nervous system, and gastrointestinal tract.McAdam or Damiani criteria can be used to establish a diagnosis of relapsing polychondritis. McAdam criteria require the presence of at least three of the following symptoms, plus compatible histology: bilateral auricular chondritis, nonerosive, seronegative inflammatory polyarthritis, nasal chondritis, eye inflammation, chondritis of the respiratory tract, and cochlear and/or vestibular dysfunction. According to Damiani criteria, a diagnosis is confirmed if at least three of McAdam’s criteria are met, two McAdam’s criteria are met with positive histology, or chondritis is present in at least 2 anatomic locations with a response to steroids or dapsone.
Conclusions: The hospitalist should be aware of the symptoms of relapsing polychondritis. Prompt diagnosis and treatment with corticosteroids will provide rapid relief of symptoms and prevent progression to life threatening organ compromise, including irreversible lung, heart, and kidney inflammation.