Progressive narrowing of the central airways due to diffuse inflammation is a potential life-threatening condition. A number of diseases have been described as possible causes. We present two siblings with severe central airway obstruction. Despite considerable efforts we have not been able to match the clinical appearance of our patients with the diagnostic criteria of any of the disease entities known to cause this condition.
Keywords: ACE, angiotensin converting enzyme; ANA, anti-nuclear antibody; ANCA, anti-neutrophil cytoplasmic antibody; CMV, cytomegalovirus; CRP, C-reactive protein; CT, computer tomography; DLCO, diffusing capacity of the lung for carbon monoxide; FEV1, forced expiratory volume in one second; FVC, forced vital capacity; GP, general practitioner; Obstruction; Polychondritis; RF, rheumatoid factor; RPC, relapsing polychondritis; Siblings; TLC, total lung capacity; WG, Wegener's granulomatosis.