Relapsing Polychondritis and Airway Involvement

Respiratory Failure Secondary to Relapsing Polychondritis

“…Currently, the diagnosis is made if there are at least three of the following features: ocular inflammation; audiovestibular damage; non-erosive seronegative polyarthritis; and bilateral auricular, nasal, and laryngotracheal chondritis, along with histological confirmation and response to steroids/dapsone. 2,3 Although commonly seen, airway involvement indicates poor prognosis if not diagnosed early. 4 Involvement of larger airways such as the larynx and tracheobronchial tree is seen in almost half of patients with RP.…”

“…A complete blood profile was significant for anemia, with hemoglobin of 8 g/dL and leukocytosis of 27,000 white blood cells/L. Arterial blood gas results revealed respiratory acidosis with pH 7.29, P aCO 2 58 mm Hg, and P aO 2 Other relevant laboratory data included an elevated erythrocyte sedimentation rate of 120 mm/h and C-reactive protein of 23.7 mg/dL. The anti-neutrophil cytoplasmic antibody panel, anti-nuclear antibody, and rheumatoid factor were unremarkable.…”

A Patient with Relapsing Polychondritis who Had Been Diagnosed as Intractable Bronchial Asthma

“…The present case exhibited saddle nose and flare/swelling of ear auricles, which was not connected with her airway symptoms by her doctor. Several studies reported that the incidence of airway symptoms in RPC patients ranged from 20 to 50%, and that airway symptoms were initially present in 10 to 15% (3,9). Other common sites involved in RPC included the auricles, joints, and nasal cartilage although many patients might not show all symptoms at onset.…”

“…The disappearance of the tracheal cartilage ring by bronchoscopy suggested advanced cartilage destruction, consistent with a flow-volume curve indicating the pattern of intrathoracic airway stenosis. Since common causes of death in RPC patients included respiratory failure and airway infection, insertion of a tracheobronchial stent must be considered (3,14).…”

Tracheobronchomalacia after allogeneic BMT; pulmonary function test findings similar to those of bronchiolitis obliterans

“…8 Relapsing polychondritis, an autoimmune connective tissue disease, has been reported to cause TBM. 9 Although chondritis associated with cGVHD has not been reported, cGVHD could potentially cause chondritis. 10 The first case was misdiagnosed as BO.…”