CRA66 EXPLORING THE ENIGMA: CAPLAN SYNDROME UNVEILED

Norsyarifah Hashim, Chen Yong Tan, Noorul Afidza Muhammad
Respiratory Department, Hospital Sultan Idris Shah

Introduction

Caplan syndrome, a rare disease referring to pneumoconiosis associated with rheumatoid arthritis. Its prevalence is between 0.4% to 1.5% usually affecting patients with exposure to silica. 

Objectives

To identify clinical presentations, radiological and pathological features of caplan syndrome, its management and complications.

Methodology

We reviewed case notes, investigation results, imaging studies, and discussed treatment options based on literature review.

Results

65 year old Indian gentleman, a non smoker was working in the quarry for 37 years. He presented with shortness of breath 2 years prior quitting his job. He also complaint of multiple joint pains involving wrists, right shoulder, knees and ankles. Bloods investigations showed rheumatoid factor of 64 IU/ml and anti-CCP more than 340 U/ml with bony erosions at left wrist. Patient was started on oral sulfasalazine. Chest x-ray showed bilateral patchy consolidations with diffuse reticulonodular and restrictive picture on spirometry with FVC 2.16L (55% of predicted). Tuberculosis work up was negative. HRCT thorax showed classical silicosis with multiple centrilobular nodules at both lungs, with no contrast enhancement on MRI, thus a diagnosis of Caplan syndrome was made. Subsequent spirometry showed worsening FVC 1.66L (43% of predicted) with reduced TLC and DLCO. Serial HRCT Thorax showed progression to extensive mediastinal nodes with conglomerate nodules at the upper lobes, evidence of progressive massive fibrosis.

Discussions

Patient presented with respiratory symptoms and seropositive arthritis, with history of silica exposure should prompt the suspicion of Caplan syndrome. Radiological and pathological features should be familiarised. Treatment should be focused on smoking cessation, limiting occupational exposure and controlling the extra pulmonary manifestations of rheumatoid arthritis. There is no data to support the use of antifibrotic. Complications including progressive massive fibrosis, silicotuberculosis and malignancy should be monitored.