Caplans syndrome: Difference between revisions
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Revision as of 16:58, 25 September 2012
| Caplans syndrome | |
| ICD-10 | J99.0 M05.1 |
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| ICD-9 | 714.81 |
| DiseasesDB | 1961 |
| MedlinePlus | 000137 |
| MeSH | D002205 |
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Caplans syndrome Microchapters |
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Diagnosis |
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Treatment |
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Case Studies |
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Caplans syndrome On the Web |
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American Roentgen Ray Society Images of Caplans syndrome |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Synonyms and keywords: Caplan's disease; rheumatoid pneumoconiosis; rheumatoid arthritis-pneumoconiosis syndrome; rheumatoid lung
Overview
Caplans syndrome is an inflammation and scarring of the lungs in people with rheumatoid arthritis who have been exposed to coal dust. This combination of rheumatoid arthritis and pneumoconiosis manifests itself as intrapulmonary nodules, which appear homogenous and well-defined on chest X-ray.[1]
Historical Perspective
Caplan's syndrome was originally described in coal miners with progressive massive fibrosis.
Pathophysiology
Some people who have been exposed to the dust have severe lung scarring that makes it difficult for their lungs to carry oxygen to the bloodstream (called progressive massive fibrosis). People with rheumatoid arthritis do not seem more likely to have this complication of scarring.
Persons with rheumatoid arthritis are more likely to develop larger areas of inflammation and scarring in response to coal dust.
Causes
Caplan syndrome is caused by breathing in coal mining dust. This causes inflammation and can lead to the development of many small lung lumps (nodules) and mild asthma-like airway disease.
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.
Epidemiology and Demographics
Caplan syndrome is very rare in the United States. Incidence is currently 1 in 100,000 people but is likely to fall as the coal mining industry declines.
Diagnosis
History and Symptoms
Symptoms include: a cough, shortness of breath, and wheezing. In addition there are the features of RA with joint pain, rheumatoid nodules and morning stiffness.
Physical Examination
Lungs
- Auscultation of the chest may reveal diffuse râles that do not disappear on coughing or taking a deep breath.
Extremities
- Tender, swollen MCP joints and rheumatoid nodules
Diagnosis
- 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.
Persons with Caplan syndrome may be at increased risk for tuberculosis (TB), and should be screened for exposure to TB.
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. Coal worker's pneumoconiosis uncommonly causes significant breathing difficulty or disability.