Successful therapy with tocilizumab in a patient with therapy-refractory relapsing polychondritis

The 44-year-old female patient presented with acute progressive swelling, redness, and pain of the nose without any previous illnesses and continuous medication. Oral antibiotic therapy under the suspected diagnosis of erysipelas was unsuccessful. In the course of time, the patient developed a recurrent conjunctivitis and a saddle nose. Laboratory chemistry revealed elevated CRP and ANA, and unremarkable ANCA and renal function. Coronary sinus CT, CT thorax and spirometry were unremarkable. Histopathologic examination of a tissue sample of the nasal mucosa, including review by a consultation and reference center for vasculitis diagnostics, resulted in a diagnosis of uncharacteristic rhinitis. Histopathologic workup of a biopsy of the auricle in the presence of unilateral perichondritis that occurred during the course revealed evidence of relapsing polychondritis. After an interdisciplinary workup of the findings in the Center of Excellence for Inflammation Medicine of the UKSH, the patient was treated with methotrexate (15 mg 1x per week), folic acid (10 mg) and cortisone (the starting dose was 100 mg). In the absence of improvement of the symptoms, off label therapy with a humanized monoclonal Il-6 receptor antibody (Tocilizumab 163 mg 1x per week) was successfully initiated. Relapsing polychondritis is a rare disease. Diagnosis is usually difficult and various differential diagnoses must be excluded. In an untreated state, the course is destructive and possibly life-threatening (tracheal manifestation). No uniform standards have been described for immunomodulatory therapy. Agents specifically affecting the inflammatory metabolic pathways represent promising new therapeutic options.

Publication History

Article published online:
12 May 2023