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Caplan Syndrome

Caplans Syndrome

Caplan syndrome is a rare type of lung disease in which individuals suffering from rheumatoid arthritis develop lung nodules in association with exposure to silica and coal dust. Patient history, imaging studies, and serology are necessary steps in order to confirm this clinical entity.


Presentation

Having in mind the frequent asymptomatic nature of coal worker's pneumoconiosis (CWP) or silicosis, the principal occupational factors in the pathogenesis of Caplan syndrome, patients often present with little or no respiratory symptoms [1] [2]. Mild dyspnea, most notably on exertion, was noted as the principal complaint in recent case reports [1] [2], while a cough and hemoptysis are absent. In fact, the disease is often discovered incidentally, while virtually all patients present with one or more manifestations of rheumatoid arthritis (RA) [2] [3]:

  • Polyarthritis, tendinitis, bursitis - Most commonly involving the wrist and hand joints; profound inflammation seen in RA causes polyarthritis accompanied by morning stiffness, usually in a symmetrical fashion.
  • Nodules - Firm, nontender nodules that develop most frequently in the forearms, calfs, and the sacrum, are one of the main features of RA, and their subcutaneous appearance may be followed by their development in visceral organs, including the lungs, which is the cardinal feature of Caplan syndrome.
  • Constitutional symptoms - Fever, fatigue, malaise and cachexia ensue as a result of extensive inflammatory changes.

Although seen in a very small number of patients, cardiac (ischemic heart disease, cardiomyopathy, pericarditis) and hematologic (anemia, neutropenia, splenomegaly) features may also be encountered in RA patients [3].

Constitutional Symptom
  • Constitutional symptoms - Fever, fatigue, malaise and cachexia ensue as a result of extensive inflammatory changes.[symptoma.com]
Recurrent Infection
  • This condition involves abnormal enlargement of spleen which shows symptoms like Anorexia, loss of Appetite, weight loss, joint deformity, recurrent infections, anaemia, swelling of lymph nodes and low white blood cell count.[findarthritistreatment.com]
Cough
  • Examination of the chest may show diffuse rales that do not disappear on coughing or taking a deep breath.[patient.info]
  • 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.[en.wikipedia.org]
  • Mild dyspnea, most notably on exertion, was noted as the principal complaint in recent case reports, while a cough and hemoptysis are absent.[symptoma.com]
  • Clinical findings Swollen joints, rheumatoid nodules, cough and dyspnoea.[medical-dictionary.thefreedictionary.com]
Dyspnea
  • Mild dyspnea, most notably on exertion, was noted as the principal complaint in recent case reports, while a cough and hemoptysis are absent.[symptoma.com]
  • Review Topic QID: 100904 4 Malignant pulmonary mesothelioma M1 Select Answer to see Preferred Response PREFERRED RESPONSE 5 (M1.PL.118) A 42-year-old male presents to your office complaining of dyspnea on exertion.[medbullets.com]
  • Once the disease progresses, patients can develop dyspnea that is typically progressive. If left untreated, patients can develop signs of right-sided heart failure.[lecturio.com]
Rales
  • Examination of the chest may show diffuse rales that do not disappear on coughing or taking a deep breath.[patient.info]
Hemoptysis
  • Mild dyspnea, most notably on exertion, was noted as the principal complaint in recent case reports, while a cough and hemoptysis are absent.[symptoma.com]
Productive Cough
  • Patients with simple CWP can be asymptomatic or can present with non-specific symptoms such as a productive cough. Once the disease progresses, patients can develop dyspnea that is typically progressive.[lecturio.com]

Workup

Because Caplan syndrome is strongly associated with occupational exposure to coal dust or silica, a detailed patient history with an emphasis on previous occupations may be one of the most important steps during workup. Similarly, assessment of signs and symptoms suggestive of RA (if the diagnosis is not already known) can only be made after a meticulous history taking, but the role of a thorough physical examination is equally important. The diagnosis of Caplan syndrome, however, relies on findings obtained from imaging studies - radiography and computed tomography (CT) of the thorax [4] [5]. Well-defined lung nodules that range from 0.5-5 cm, predominantly located in the periphery, are typical radiographic lesions seen in these patients [1] [4]. Coalescence of nodules and formation of a large confluent nodule, cavitation or calcification may also be observed [4]. On the other hand, confirmation of RA is necessary by performing appropriate laboratory tests, in addition to clinical criteria. Evaluation of C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), rheumatoid factor (RF) and anti-cyclic citrullinated peptide (anti-CCP) antibodies are mandatory if clinical suspicion toward RA exists. The combination of radiographic findings, occupational risk factors, and laboratory results should be sufficient to make the diagnosis of Caplan syndrome.

Kerley A Lines
  • Fleischer (1874-1965)German ophthalmologist Kearns-Sayre カーンズ・セイヤー Kearns-Sayre syndrome カーンズ・セイヤー症候群 Keith-Flack キース・フラック Keith-Flack node キース・フラック小結節 Keith-Wagener キース・ワグナー Keith-Wagener classification キース・ワグナー分類 Kent ケント Kent bundle ケント束 Kerley カーリー Kerley[jams.med.or.jp]
Pulmonary Infiltrate
  • Rheumatoid arthritis extra-articular manifestations : • arm paresthesias and hyperreflexia C1-C2 subluxation (increased risk with intubation) • cough, fever, pulmonary infiltrates bronchiolitis obliterans organizing pneumonia • foot drop or wrist drop[mnemocine.wordpress.com]

Treatment

  • Physical treatment should proceed as for rheumatoid arthritis (RA). Smoking should cease. Drugs After exclusion of TB, steroids are used. Treatment of the RA will include disease-modifying anti-rheumatic drugs (DMARDs) at an early stage.[patient.info]
  • AutoimmuneMom.com is not a medical practice and does not provide medical advice, diagnosis, or treatment.[autoimmunemom.com]
  • Other tests can include: Chest x-ray CT scan of the chest Joint x-rays Pulmonary function tests Rheumatoid factor test Treatment There is no specific treatment for Caplan syndrome, other than treating any lung and joint disease.[coordinatedhealth.com]
  • Treatment of Caplan’s Syndrome or Coal Workers Pneumoconiosis No particular cure is available for the treatment of Caplan’s syndrome.[epainassist.com]

Prognosis

  • Prognosis [ edit ] 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.[en.wikipedia.org]
  • 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.[ipfs.io]
  • Prognosis This is as for RA. Severe respiratory disability is uncommon but massive pulmonary fibrosis can progress at times. Spontaneous remission of the lung disease can occur.[patient.info]

Etiology

  • . • A chronic inflammatory disease of unknown etiology marked by a symmetric, peripheral polyarthritis. • Most common form of chronic inflammatory arthritis 3. • A systemic Disease • Extraarticular manifestations : 1.[slideshare.net]

Epidemiology

  • Epidemiology It was said to affect 1 in 100,000 people but the incidence is falling as the coal mining industry has been in decline. The prevalence is so low that much of the literature is case reports rather than trials.[patient.info]
  • Epidemiology [ edit ] 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).[en.wikipedia.org]
  • This review summarizes history, definition and current knowledge on epidemiology, pathology, pathophysiology, clinical presentation and treatment of Caplan's syndrome. Copyright 2010 European Federation of Internal Medicine.[ncbi.nlm.nih.gov]
  • 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 cases of complicated silicosis (marked by progressive massive pneumoconiosis).[ipfs.io]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology [ edit ] The presence of rheumatoid arthritis alters how a person's immune system responds to foreign materials, such as dust from a coal mine. [1] When a person with rheumatoid arthritis is exposed to such offensive materials, they are[en.wikipedia.org]
  • This review summarizes history, definition and current knowledge on epidemiology, pathology, pathophysiology, clinical presentation and treatment of Caplan's syndrome. Copyright 2010 European Federation of Internal Medicine.[ncbi.nlm.nih.gov]
  • Pathophysiology The presence of rheumatoid arthritis alters how a person's immune system responds to foreign materials, such as dust from a coal mine. [1] When a person with rheumatoid arthritis is exposed to such offensive materials, they are at an increased[ipfs.io]

Prevention

  • Prevention People with rheumatoid arthritis (RA) must not be exposed to additional risk factors for lung disease.[patient.info]
  • Prevention People with rheumatoid arthritis should avoid exposure to hazardous dust.[coordinatedhealth.com]
  • Though associated lung and joint problems can be treated, prevention is the only form of treatment that can be suggested for Caplan’s syndrome.[epainassist.com]
  • This will prevent breathing problems from becoming severe, as well as further damage to your lungs. People with RA should avoid exposure to inorganic dust.[nlm.nih.gov]

References

Article

  1. De Capitani EM, Schweller M, Silva CM, Metze K, Cerqueira EM, Bértolo MB. Rheumatoid pneumoconiosis (Caplan's syndrome) with a classical presentation. J Bras Pneumol. 2009;35(9):942-946.
  2. Rozenberg D, Shapera S. What to do with all of these lung nodules? Can Respir J. 2014;21(3):e52-e54.
  3. Longo DL, Fauci AS, Kasper DL, Hauser SL, Jameson J, Loscalzo J. eds. Harrison's Principles of Internal Medicine, 18e. New York, NY: McGraw-Hill; 2012
  4. Schreiber J, Koschel D, Kekow J, Waldburg N, Goette A, Merget R. Rheumatoid pneumoconiosis (Caplan's syndrome). Eur J Intern Med. 2010;21(3):168-72.
  5. Arakawa H, Honma K, Shida H, Saito Y, Morikubo H. Computed tomography findings of Caplan syndrome. J Comput Assist Tomogr. 2003;27(5):758-760..

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Last updated: 2019-07-11 21:49