Marble pneumoconiosis associated with mixed connective tissue disease

Alexander P Rozin,Kohava Toledano

doi:10.7243/2052-6954-1-6

Abstract

Caplan's syndrome: combination of pneumoconiosis and lung nodes due to seropositive rheumatoid arthritis (RA) is well known entity. We present 45 years old marble worker with fever, cough, and exertion dyspnoe with multiple lung nodes, infiltrates and fibrosis by chest CT and Raynaud syndrome with vascular finger tip lesions. Our patient had 7 years history of mixed connective tissue disease. Skin vascular lesions rapidly regressed after nifedipine therapy and methylprednisolone pulses. Substantial response of the lung disease to steroid and antibiotic therapy was not observed. Due to further respiratory failure the patient was underwent lung transplantation. Histology from removed lung showed dust laden macrophages with weak polarization positivity, advanced silicotic nodules with focal calcifications surrounded by chronic inflammation and massive fibrosis. No acid fast bacteria were found. Above mentioned findings extend a field of Caplan's syndrome. Different dusts and other rheumatic diseases, a part from RA, might be implicated.

Highlights

We are all acquainted with well known entity known as Caplan’s syndrome, a combination of pneumoconiosis and lung nodes due to seropositive rheumatoid arthritis (RA)
We present a 45 years old marble worker (15 years exposure) with fever, cough, and exertional dyspnea with multiple lung nodes, infiltrates and fibrosis on chest CT (Figure 1)
Exposure to marble dust has recently been reported as a risk factor for lung silicosis [3]

Summary

Introduction

We are all acquainted with well known entity known as Caplan’s syndrome, a combination of pneumoconiosis and lung nodes due to seropositive rheumatoid arthritis (RA). Our patient had a 7 year history of polyarthritis as part of a mixed connective tissue disease (RA, SLE, Sjogrene) with positive serology: RF, ANA, RNP, SSA, SSB, polyclonal hypergammaglobulinemia, ANCA negative and with good response of the arthritis, polycytopenia and Raynaud’s phenomenon to hydroxychloroquine and prednisone therapy. His lung disease was not associated with constitutional symptoms and respiratory disability for an extended period of time. Physical examination showed Raynaud’s phenomenon, multiple finger tip ischemic and necrotic lesions (Figure 2) and bilateral end-inspiratory crackles.

Discussion

Walker WC and Wright V

Findings

Gong H Jr and Tashkin DP