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Caplan syndrome

The Caplan syndrome (also known as rheumatoid pneumoconiosis) is the combination of seropositive rheumatoid arthritis and a characteristic pattern of fibrosis.

Although first described in coal miners (coal workers' pneumoconiosis), it has subsequently been found in patients with a variety of pneumoconioses 2

Epidemiology

Affects 2 - 6% of patients with pneumoconiosis (in Wales) 4 and is caused by disintegrating macrophages leaving a necrotic core with a surrounding pigmented (black) dust ring and fibroblasts 4-5.

Radiographic features

  • 5 - 50 mm well-defined nodules in the upper lung lobes / lung periphery.
  • nodules may remain unchanged, multiply, calcify, or become thick walled cavities.
  • background changes of pneumoconiosis
  • may have an accompanying pleural effusion
  • features often grow in short bursts

Etymology

First described by A Caplan in 1953 1

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