Caplan's syndrome
Encyclopedia
Caplan's syndrome is a combination of rheumatoid arthritis (RA)
and pneumoconiosis
that manifests as intrapulmonary nodules, which appear homogenous and well-defined on chest X-ray
.. 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.
He followed this with further articles exploring the disease.
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.
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.
should be attempted. Rheumatoid arthritis should be treated normally with early use of DMARDs.
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).
.
Rheumatoid arthritis
Rheumatoid arthritis is a chronic, systemic inflammatory disorder that may affect many tissues and organs, but principally attacks synovial joints. The process produces an inflammatory response of the synovium secondary to hyperplasia of synovial cells, excess synovial fluid, and the development...
and pneumoconiosis
Pneumoconiosis
Pneumoconiosis is an occupational lung disease and a restrictive lung disease caused by the inhalation of dust, often in mines.-Types:Depending upon the type of dust, the disease is given different names:...
that manifests as intrapulmonary nodules, which appear homogenous and well-defined on chest X-ray
Chest X-ray
In medicine, a chest radiograph, commonly called a chest X-ray , is a projection radiograph of the chest used to diagnose conditions affecting the chest, its contents, and nearby structures...
.. 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.
He followed this with further articles exploring the disease.
Signs and symptoms
Caplan's syndrome presents with coughCough
A cough is a sudden and often repetitively occurring reflex which helps to clear the large breathing passages from secretions, irritants, foreign particles and microbes...
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
Auscultation
Auscultation is the term for listening to the internal sounds of the body, usually using a stethoscope...
of the chest may reveal diffuse râles
Rales
Crackles, crepitations, or rales are the clicking, rattling, or crackling noises that may be made by one or both lungs of a human with a respiratory disease during inhalation. They are often heard only with a stethoscope...
that do not disappear on coughing or taking a deep breath.
Causes
Caplan's syndrome occurs only in patients with both RA and pneumoconiosisPneumoconiosis
Pneumoconiosis is an occupational lung disease and a restrictive lung disease caused by the inhalation of dust, often in mines.-Types:Depending upon the type of dust, the disease is given different names:...
related to mining dust (coal, asbestos, silica).The condition occurs in miners (especially those working in anthracite coal-mines), asbestosis
Asbestosis
Asbestosis is a chronic inflammatory and fibrotic medical condition affecting the parenchymal tissue of the lungs caused by the inhalation and retention of asbestos fibers...
, silicosis
Silicosis
Silicosis, also known as Potter's rot, is a form of occupational lung disease caused by inhalation of crystalline silica dust, and is marked by inflammation and scarring in forms of nodular lesions in the upper lobes of the lungs...
and other pneumoconioses. There is probably also a genetic predisposition, and smoking
Smoking
Smoking is a practice in which a substance, most commonly tobacco or cannabis, is burned and the smoke is tasted or inhaled. This is primarily practised as a route of administration for recreational drug use, as combustion releases the active substances in drugs such as nicotine and makes them...
is thought to be an aggravating factor.
Diagnosis
- Chest radiologyChest X-rayIn medicine, a chest radiograph, commonly called a chest X-ray , is a projection radiograph of the chest used to diagnose conditions affecting the chest, its contents, and nearby structures...
shows multiple, round, well defined nodulesNodule (medicine)For use of the term nodule in dermatology, see Nodule In medicine, a nodule refers to a relatively hard, roughly spherical abnormal structure....
, usually 0.5-2.0 cm in diameter, which may cavitate and resemble tuberculosisTuberculosisTuberculosis, MTB, or TB is a common, and in many cases lethal, infectious disease caused by various strains of mycobacteria, usually Mycobacterium tuberculosis. Tuberculosis usually attacks the lungs but can also affect other parts of the body...
. - 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 DLCODLCODLCO is the extent to which oxygen passes from the air sacs of the lungs into the blood. Commonly, it refers to the test used to determine this parameter. It was introduced in 1909....
. - Rheumatoid factorRheumatoid factorRheumatoid factor is an autoantibody most relevant in rheumatoid arthritis. It is defined as an antibody against the Fc portion of IgG. RF and IgG join to form immune complexes that contribute to the disease process...
, antinuclear antibodies, and non-organ specific antibodies may be present in the serum. - SilicosisSilicosisSilicosis, also known as Potter's rot, is a form of occupational lung disease caused by inhalation of crystalline silica dust, and is marked by inflammation and scarring in forms of nodular lesions in the upper lobes of the lungs...
and asbestosisAsbestosisAsbestosis is a chronic inflammatory and fibrotic medical condition affecting the parenchymal tissue of the lungs caused by the inhalation and retention of asbestos fibers...
must be considered in the differential with TB.
Management
Once tuberculosis has been excluded, treatment is with steroids. All exposure to coal dust must be stopped, and smoking cessationSmoking cessation
Smoking cessation is the process of discontinuing the practice of inhaling a smoked substance. This article focuses exclusively on cessation of tobacco smoking; however, the methods described may apply to cessation of smoking other substances that can be difficult to stop using due to the...
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
IncidenceIncidence (epidemiology)
Incidence is a measure of the risk of developing some new condition within a specified period of time. Although sometimes loosely expressed simply as the number of new cases during some time period, it is better expressed as a proportion or a rate with a denominator.Incidence proportion is the...
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 fibrosisProgressive massive fibrosis
Progressive Massive Fibrosis , characterized by the development of large conglomerate masses of dense fibrosis , can complicate Silicosis and Coal worker's pneumoconiosis...
.
External links
- Chest CT Caplan Syndrome Radiology