Caplan's syndrome


Caplan's syndrome or Caplan disease or Rheumatoid pneumoconiosis1 is a combination of rheumatoid arthritis RA and pneumoconiosis that manifests as intrapulmonary nodules, which appear homogenous and well-defined on chest X-ray2

Contents

  • 1 Signs and symptoms
  • 2 Causes
  • 3 Pathophysiology
  • 4 Diagnosis
  • 5 Management
  • 6 Prognosis
  • 7 Epidemiology
  • 8 History
  • 9 References
  • 10 External links

Signs and symptomsedit

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

Caplan syndrome is a nodular condition of the lung occurring in dust-exposed persons with either a history of rheumatoid arthritis RA or who subsequently develop RA within the following 5–10 years3 The nodules in the lung typically occur bilaterally and peripherally, on a background of simple coal workers' pneumoconiosis There are usually multiple nodules, varying in size from 05 to 50 cm The nodules typically appear rapidly, often in only a few weeks Nodules may grow, remain unchanged in size, resolve, or disappear and then reappear They can cavitate, calcify, or develop air-fluid levels Grossly, they can resemble a giant silicotic nodule Histologically, they usually have a necrotic center surrounded by a zone of plasma cells and lymphocytes, and often with a peripheral inflammatory zone made of macrophages and neutrophils

Causesedit

Caplan 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

Pathophysiologyedit

The presence of rheumatoid arthritis alters how a person's immune system responds to foreign materials, such as dust from a coal mine1 When a person with rheumatoid arthritis is exposed to such offensive materials, they are at an increased risk of developing pneumoconiosis1

Diagnosisedit

  • Chest radiology shows multiple, round, well defined nodules, usually 05-20 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 DLCO
  • 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

Managementedit

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

Prognosisedit

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

Epidemiologyedit

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 cases of complicated silicosis marked by progressive massive pneumoconiosis

Historyedit

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 publication4 He followed this with further articles exploring the disease56 Caplan syndrome was originally described in coal miners with progressive massive fibrosis

Referencesedit

  1. ^ a b c Murray, John F 2010 Murray and Nadel's textbook of respiratory medicine 5th ed Philadelphia, PA: Saunders/Elsevier p 1566 ISBN 978-1-4160-4710-0 
  2. ^ Ondrasík M 1989 "Caplan syndrome" Baillière's Clinical Rheumatology 3 1: 205–10 PMID 2661027 doi:101016/S0950-35798980045-7 
  3. ^ Caplan A March 1953 "Certain unusual radiological appearances in the chest of coal-miners suffering from rheumatoid arthritis" Thorax 8 1: 29–37 PMC 1019224  PMID 13038735 doi:101136/thx8129 
  4. ^ Caplan, Anthony 1953 "Certain Unusual Radiological Appearances in the Chest of Coal-miners Suffering from Rheumatoid Arthritis" Thorax 8 1: 29–37 ISSN 0040-6376 PMC 1019224  PMID 13038735 doi:101136/thx8129 
  5. ^ Miall, W E; Anthony Caplan; A L Cochrane; G S Kilpatrick; P D Oldham 1953-12-05 "Rheumatoid Arthritis Associated with Characteristic Chest X-ray Appearances in Coal-workers" British Medical Journal 2 4848: 1231–1236 ISSN 0007-1447 PMC 2030245  PMID 13106392 doi:101136/bmj248481231 
  6. ^ Caplan, A; R B Payne; J L Withey September 1962 "A Broader Concept of Caplan Syndrome Related to Rheumatoid Factors" Thorax 17 3: 205–212 ISSN 0040-6376 PMC 1018697  PMID 13876317 doi:101136/thx173205 

External linksedit

  • 00057 at CHORUS
  • Chest CTpermanent dead link Caplan Syndrome Radiology


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