Pleuro-pulmonary complications of rheumatoid arthritis.

Rheumatoid arthritis (RA) is a chronic systemic inflammatory disease whose distinctive feature is the development of symmetrical polyarthritis. It has a prevalence of 1% in the general population, predominating in females and peaking between the ages of 30 and 50 years; it is found worldwide, although some ethnic groups show an increased propensity for the disease. The burden of illness in these patients is manifest in a mortality rate that is more than twice that of age-matched individuals in the general population, with poor prognostic factors including rheumatoid factor seropositivity, anemia of chronic disease, presence of anti-cyclic citrullinated peptide antibodies, and extra-articular manifestations. Ellman and Ball1 first described lung disease in association with RA, and since that time, various pulmonary manifestations, including pleural effusions, lung nodules, obstructive lung disease, and vasculitis, have been described in the literature. In addition to problems caused by the disease, immunosuppressant agents and other medications used to treat RA can be directly pneumotoxic, predispose the patient to opportunistic infection, and impair wound repair or healing. Clinical features associated with RA-associated lung disease include male gender, rheumatoid factor seropositivity, severe articular disease, subcutaneous nodules, and other extra-articular manifestations. Progressive nodular and pleural lung disease associated with RA represents a particular clinical challenge. The nodules are often multiple and involve both lungs, varying in size from a few millimeters to several centimeters. A propensity for the lung peripheries and fissures with an ability to become necrotic and rupture results in complications such as recurrent secondary pneumothorax, bronchopleural fistula formation with sizeable air leak, hemorrhage, pulmonary abscess formation, and empyema. The inflammation, fibrous visceral peel formation (with trapped lung) and noncompliant parenchyma associated with rheumatoid lung mean achieving key clinical objectives such as closure of the air leak, obliteration of the pleural space, eradication of infection, and effective pleurodesis may prove impossible. An additional tendency for the pleuropulmonary complications of RA to recur can result in disease that requires repeated intervention and is resistant to standard therapy. This case series illustrates the challenges that may occur in severe pleuro-pulmonary disease associated with RA, alongside a review of the literature relating to this area of practice.