Relapsing polychondritis is an autoimmune disorder associated with inflammation in cartilaginous structures and other tissues throughout the body, particularly the ears, nose, eyes, joints and respiratory tract.
77-year-old white female, a nonsmoker presented with a history of intermittent hemoptysis of 3 months duration. Two weeks following an episode of upper respiratory infection, she noted a teaspoonful of hemoptysis. One month later she had similar upper respiratory symptoms followed by hemoptysis. Due to increasing frequency of hemoptysis she was referred to ENT, who performed a direct laryngoscopy which was nondiagnostic. Three days prior to her pulmonary consultation she had swelling and erythema of the left ear. She denied any unintentional weight loss, hematuria, mouth sores or epistaxis. She had noted an erythematous blotchy rash on her legs since her initial hemoptysis. Prior to this she had some migratory, nonpainful, very erythematous lesions that would appear on various parts of her body. She had an episode of arthritis three years ago manifesting as swollen and erythematous small joints of the hands and swelling in her right knee. She denied any prior history for venous thromboembolic disease or valvular heart disease. Her past medical history included otitis externa in the right ear one year ago. Her current medications included ferrous gluconate, guaifenacin/dextromethorphan, cetrizine, amoxicillin/clavulanate and multivitamins. Family history was unremarkable. She did not have any pets or birds at home. Blood pressure was 165/69, pulse of 86 per minute, temperature is 97.6 F and weight was 157 pounds. Physical exam revealed crackles at the left lung base, swelling and erythema of the left external ear and erythematous macular-type lesions on her upper thighs bilaterally and in the neck. Labs showed a white count of 5 × 103 cells/αL with normal differential count, hematocrit of 29 % and platelet count of 356000/cu mm. Coagulation panel, iron panel and comprehensive metabolic profile were normal. Urinalysis showed 3–9 RBCs, 1+ blood and no proteinuria. C-reactive protein was 68.6 (normal range: 0.0–3.2 mg/L).
Further work up including rheumatoid factor, Cyclic Citrullinated Peptide IgG antibody, antinuclear antibody, anti-glomerular basement antibody, cryoglobulins, hepatitis profile and c-ANCA were negative. P-ANCA was positive. Myeloperoxidase antibody was positive at 8 U (Positive >1.0 U). Proteinase 3 antibody was negative. CT scan of chest revealed patchy alveolar infiltrates in lingula and left lower lobe. A fibreoptic bronchoscopy showed multiple bead like lesions in anterior wall of trachea from cartilage rings suggestive of tracheopathia osteo-chondroplastica. She also had fresh blood in the lingula and left lower lobe but bronchoalveolar lavage with serial aliquots of normal saline did not reveal any evidence of persistent alveolar hemorrhage. There were no endobronchial lesions. Results of cytology and cultures were nondiagnostic. Transbronchial biopsies from left lower lobe showed intra-alveolar hemosiderin-laden macrophages consistent with recent hemorrhage. Patient was started on prednisone at 60 mg/day. Patient had symptomatic improvement over next 2 weeks with complete resolution of left ear chondritis and hemoptysis. Renal biopsy was obtained about 3 weeks after starting prednisone and showed few fibrous crescents (3 of 19 glomeruli) with associated mild tubular atrophy and interstitial fibrosis, no significant cellular proliferation or cellular crescents identified. Differential diagnosis of this case includes Wegener’s granulomatosis, Goodpasture’s syndrome, microscopic polyangitis and ANCA associated small vessel vasculitis. Based on history of recurring auricular cartilage involvement, microscopic hematuria, hemoptysis, arthralgia, livedo racemosa over the skin of lower extremities, laboratory evaluation and clinical response to response to oral steroids we believe that this patient has relapsing polychondritis with systemic vasculitis affecting the skin, kidneys and lungs.
A systematic diagnostic approach looking for multiple organ involvement with vasculitis is warranted in a patient with hemoptysis.
Rupesh Dave, No Financial Disclosure Information; No Product/Research Disclosure Information