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A Curious Case of Emphysema and Fibrosis
1Assistant Professor, Department of Pulmonary Medicine, PGIMSR-ESIC Medical College and Hospital, New Delhi, India.
The Journal of the Association of Physicians of India
|August 3, 2026
Summary
This case study details rheumatoid arthritis-associated combined pulmonary fibrosis and emphysema (RA-CPFE) where lung issues preceded joint pain. Early HRCT diagnosis is vital for managing this serious condition.
Area of Science:
- Pulmonology
- Rheumatology
- Radiology
Background:
- Rheumatoid arthritis (RA) is a systemic autoimmune disease with common extra-articular manifestations, notably pulmonary involvement.
- Combined pulmonary fibrosis and emphysema (CPFE) is a syndrome of upper-lobe emphysema and lower-lobe fibrosis, increasingly seen in connective tissue diseases like RA.
- Pulmonary involvement in RA can precede articular symptoms, necessitating a high index of suspicion.
Purpose of the Study:
- To describe an uncommon case of rheumatoid arthritis-associated combined pulmonary fibrosis and emphysema (RA-CPFE).
- To highlight a presentation where pulmonary manifestations preceded the onset of inflammatory polyarthritis.
- To emphasize the importance of early recognition and multidisciplinary management of RA-CPFE.
Main Methods:
- Case report of a 50-year-old nonsmoking woman with progressive dyspnea and cough.
- Clinical evaluation including physical examination, laboratory tests (inflammatory markers, serology), and pulmonary function testing.
- Diagnostic imaging with high-resolution computed tomography (HRCT) of the thorax and echocardiography.
Main Results:
- The patient presented with symptoms of dyspnea and cough for 3 years before developing inflammatory polyarthritis, consistent with seronegative RA.
- HRCT confirmed CPFE with upper lobe emphysema and lower lobe honeycombing.
- Pulmonary function tests revealed preserved airflow, reduced diffusion capacity, and exercise-induced desaturation; echocardiography showed mild pulmonary hypertension.
Conclusions:
- RA-CPFE can present atypically with pulmonary disease preceding joint symptoms.
- Early diagnosis via HRCT and comprehensive pulmonary evaluation is critical for managing RA-CPFE.
- RA-CPFE is associated with significant morbidity, pulmonary hypertension, and poor prognosis, requiring multidisciplinary care including immunosuppressants and antifibrotics for progressive disease.
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