Musculoskeletal complications of cystic fibrosis

Musculoskeletal complications of cystic fibrosis

Cystic fibrosis is an autosomal recessive disease characterized by decreased mucous production leading to obstructive lung disease and malabsorption. Other organs, including sinuses, pancreas, liver, sweat glands, and reproductive tract can be affected. It is attributable to a defect in the CFTR gene on chromosome 7, which encodes for a membrane glycoprotein (CFTR) that is a chloride ion channel. In cystic fibrosis, one of the chloride ion channels present on the apical membrane of the epithelial cell is either absent or defective. This leads to increased sodium absorption and decreased chloride secretion, resulting in decreased extracellular water content. Patients have obstruction with infections in the lung and malabsorption from the gut. Consequently, patients are susceptible to osteoporosis (up to 75% of adults) attributable to poor calcium and vitamin D absorption. Additionally, 2% to 9% of patients have an episodic nondestructive oligoarthritis most commonly involving the fingers and lower extremity large joints. The arthritis is felt to be a result of immune complexes attributable to chronic lung infection. Attacks last for a few days (median 7 days) and may be associated with fever and painful nodular skin lesions and purpura. Rarely, hypertrophic osteoarthropathy (5%) and small vessel vasculitis can occur. Musculoskeletal symptoms are more common the longer the disease duration (adults > adolescence > children), the more severe the disease, and in patients infected with Pseudomonas aeruginosa

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