Caplan's syndrome

Caplan's syndrome


Caption =
DiseasesDB = 1961
ICD10 = ICD10|J|99|0|j|95 ICD10|M|05|1|m|05
ICD9 = ICD9|714.81
MedlinePlus = 000137
eMedicineSubj =
eMedicineTopic =
MeshID = D002205

Caplan's syndrome (or Caplan's disease) is a combination of rheumatoid arthritis and pneumoconiosis that manifests as intrapulmonary nodules, which appear homogenous and well-defined on chest X-ray. [cite journal |author=Ondrasík M |title=Caplan's syndrome |journal=Baillieres Clin Rheumatol |volume=3 |issue=1 |pages=205–10 |year=1989 |pmid=2661027 |doi=10.1016/S0950-3579(89)80045-7]


igns and symptoms

There is cough and shortness of breath. In addition there are the features of RA with 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.


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.



*Chest radiology shows multiple, round, well defined nodules, usually 0.5-2.0 cm in diameter, which may cavitate and resemble tuberculosis.
*Lung function tests may reveal a mixed restrictive and obstructive ventilatory defect with a loss of lung volume. There may also be irreversible airflow limitation and a reduced gas transfer factor.
*Rheumatoid factor, antinuclear antibodies, and non-organ specific antibodies may be present in the serum.
*Silicosis and asbestosis must be considered in the differential with TB.


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.


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.


Incidence is currently 1 in 100,000 people but is likely to fall as the coal mining industry declines.


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


External links


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