Caplan's syndrome

Caplan's syndrome
Classification and external resources
ICD-10 J99.0 M05.1
ICD-9 714.81
DiseasesDB 1961
MedlinePlus 000137
MeSH D002205

Caplan's syndrome (or Caplan's disease) is a combination of rheumatoid arthritis (RA) and pneumoconiosis that manifests as intrapulmonary nodules, which appear homogenous and well-defined on chest X-ray.[1]. The syndrome is named after Dr. Anthony Caplan, a physician on the Cardiff Pneumoconiosis Panel, who identified the constellation of findings as a distinct entity in a 1953 publication.[2] He followed this with further articles exploring the disease.[3][4]

Contents

Signs and symptoms

Caplan's syndrome presents with cough and shortness of breath in conjunction with features of rheumatoid arthritis such painful joints and morning stiffness. Examination should reveal tender, swollen MCP joints and rheumatoid nodules; auscultation of the chest may reveal diffuse râles that do not disappear on coughing or taking a deep breath.

Causes

Caplan's syndrome occurs only in patients with both RA and pneumoconiosis related to mining dust (coal, asbestos, silica).The condition occurs in miners (especially those working in anthracite coal-mines), asbestosis, silicosis and other pneumoconioses. There is probably also a genetic predisposition, and smoking is thought to be an aggravating factor.

Pathophysiology

Diagnosis

Management

Once tuberculosis has been excluded, treatment is with steroids. All exposure to coal dust must be stopped, and smoking cessation should be attempted. Rheumatoid arthritis should be treated normally with early use of DMARDs.

Prognosis

The nodules may pre-date the appearance of rheumatoid arthritis by several years. Otherwise prognosis is as for RA; lung disease may remit spontaneously, but pulmonary fibrosis may also progress.

Epidemiology

Incidence is currently 1 in 100,000 people but is likely to fall as the coal mining industry declines.It has also been shown to occur in case of complicated silicosis(marked by progressive massive pneumoconiosis).

History

Caplan's syndrome was originally described in coal miners with progressive massive fibrosis.

References

External links