Rheumatologic Manifestations of CF

Abstract

While the pulmonary manifestations of cystic fibrosis (CF) most obviously contribute to the morbidity and mortality of the disease, many patients with CF suffer from bone and joint manifestations as well. A subset of patients with CF experience severe intermittent joint pain from an episodic arthropathy, progressive joint pain from hypertrophic pulmonary osteoarthropathy, or joint pain due to extensive use of fluoroquinolone antibiotics. Others may experience an idiopathic vasculitic rash. The majority of patients with CF will experience decreased bone mineral density as they age, in some cases leading to pathologic fractures. Understanding and treating these conditions is important to providing optimal care for children and adults with CF.