A Curious Case of Emphysema and Fibrosis

Rheumatoid arthritis (RA) is a systemic autoimmune disease with frequent extra-articular manifestations, of which pulmonary involvement is the most common and clinically significant. Combined pulmonary fibrosis and emphysema (CPFE) is a distinct clinic-radiological syndrome characterized by the coexistence of upper-lobe emphysema and lower-lobe fibrosis and is increasingly recognized in patients with connective tissue diseases, including RA. We describe the case of a 50-year-old nonsmoking woman who presented with a 3-year history of progressive exertional dyspnea and chronic dry cough, preceding the onset of inflammatory polyarthritis by nearly 2 years. She later developed symmetrical involvement of small and large joints with prolonged morning stiffness, along with constitutional symptoms including low-grade fever and weight loss. Physical examination revealed grade IV digital clubbing and bibasilar inspiratory crackles. Laboratory evaluation showed elevated inflammatory markers with negative rheumatoid factor and anticyclic citrullinated peptide antibodies, consistent with seronegative RA. High-resolution computed tomography of the thorax showed upper lobe emphysema and lower lobe honeycombing, confirming the diagnosis of CPFE. Pulmonary function testing showed relatively preserved airflow with mildly reduced diffusion capacity, along with severe exercise-induced desaturation. Echocardiography revealed mild pulmonary hypertension. This case highlights an uncommon presentation of rheumatoid arthritis-associated combined pulmonary fibrosis and emphysema (RA-CPFE), where pulmonary manifestations preceded articular disease, emphasizing the need for a high index of suspicion. Early recognition through high-resolution computed tomography (HRCT) and comprehensive pulmonary evaluation is crucial, as RA-CPFE is associated with significant morbidity, pulmonary hypertension, and poor prognosis. Multidisciplinary management incorporating immunosuppressive therapy, consideration of antifibrotics in progressive disease, and supportive care remains essential. Further studies are required to better define optimal treatment strategies and prognostic markers in RA-CPFE.