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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].

Respiratoric

  • Cough

    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]

    Examination of the chest may show diffuse rales that do not disappear on coughing or taking a deep breath. [patient.info]

    Clinical findings Swollen joints, rheumatoid nodules, cough and dyspnoea. [medical-dictionary.thefreedictionary.com]

  • Dyspnea

    Case presentation: A 58-year-old man, had worked for 21 years as a truck driver with exposure to silica, until the age of 52, when he developed dyspnea with polyarthritis involving wrists and ankles. He had dyspnea in effort. [oatext.com]

    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]

    The most typical symptoms are dry cough, limited exercise tolerance and progressive dyspnea, although there is not usually a good clinical correlation between the dyspnea and radiological findings. [archbronconeumol.org]

    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]

    Three years following his biopsy, the patient presented with lower extremity edema and dyspnea on exertion. [ncbi.nlm.nih.gov]

  • 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]

    However, patients should be monitored for rare complications such as hemoptysis or pneumothorax from the cavitated nodules as well as end stage pulmonary fibrosis. [ncbi.nlm.nih.gov]

    They are generally asymptomatic and rarely cause coughing or hemoptysis. [archbronconeumol.org]

    Case report A 55-year-old female patient was referred to our facility suspected of having metastatic neoplasm in the lung, without weight loss, cough, or hemoptysis. She complained of dyspnea on exertion for four months. [scielo.br]

  • Productive Cough

    She did have a non-productive cough, but it was much of a problem for her. She was certainly saturating less than 90% on room air. Her DLCO was right at 71% predicted, and her white count was 8,000. [hopkinsarthritis.org]

    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]

    The clinical manifestations are non-specific and the most common symptom is dyspnea on exertion, followed by non-productive cough. Chest pain is rare. Fever is a very uncommon symptom. [archbronconeumol.org]

  • Rales

    Examination of the chest may show diffuse rales that do not disappear on coughing or taking a deep breath. [patient.info]

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.

X-Ray

  • 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 [mnemocine.wordpress.com]

  • Kerley A Lines

    カーリー Kerley B line カーリーのB線 Kernig ケルニッヒ Kernig's sign ケルニッヒ徴候 Kienboeck キーンベック Kienboeck's disease キーンベック病 RobertKienböck (1871-1953) Austrian radiologist Kiesselbach キーゼルバッハ Kiesselbach area キーゼルバッハ部位 Killian キリアン Killian operation キリアン手術 Kimmelstiel-Wilson [jams.med.or.jp]

Pulmonary Function Test

  • Restrictive Pulmonary Function

    Pulmonary function tests show a restrictive pattern with a reduced DLCO and BAO and usually show a lymphocytic infiltrate. [hopkinsarthritis.org]

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]

Epidemiology and Demographics Risk Factors Natural History, Complications and Prognosis Diagnosis Diagnostic Study of Choice History and Symptoms Physical Examination Laboratory Findings Chest X Ray CT MRI Other Imaging Findings Other Diagnostic Studies Treatment [wikidoc.org]

[…] in the treatment of these conditions. [archbronconeumol.org]

AutoimmuneMom.com is not a medical practice and does not provide medical advice, diagnosis, or treatment. [autoimmunemom.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]

[…] syndrome Microchapters Home Patient Information Overview Historical Perspective Classification Pathophysiology Causes Differentiating Caplans syndrome from other Diseases Epidemiology and Demographics Risk Factors Natural History, Complications and Prognosis [wikidoc.org]

Etiology

Though Caplan's initial idea was of infectious etiology, two years later several cases of ' rheumatoid pneumoconiosis' with tuberculosis were reported. But the theory of rheumatoid pneumoconiosis and tuberculous pneumoconiosis got rejected by time. [wikidoc.org]

Etiologies of Ischemic Posterior Circulation Stroke Beyond the minority of cases associated with vascular anomalies of the PC previously discussed, several pathological and demographic differences must be considered when determining stroke etiology. [frontiersin.org]

There is no definitive evidence, however, as to what would be the etiology of ischemic events regarding this vessel. [karger.com]

Etiology is unknown, but autoimmune mechanisms have been implicated. [termsciences.fr]

Epidemiology

An epidemiological study was conducted by Miall and associates in 1955 to determine the validity of Caplan syndrome. In 1958, J. [wikidoc.org]

In addition, epidemiological data has shown some association with silica and autoimmunity [3]. [ncbi.nlm.nih.gov]

Anthony Newman Taylor, Paul Cullinan, Paul Blanc, Anthony Pickering CRC Press, ٢٥‏/١١‏/٢٠١٦ - 547 من الصفحات This authoritative text on occupational lung disorders builds upon the fundamentals, including clinical, epidemiological, and predictive approaches [books.google.com]

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]

Pathophysiology

Nishimura Medicine, Biology Cellular & molecular immunology 2007 Immunological analyses may lead to the development of new clinical tools for the modification of the pathophysiological aspects of diseases such as the regulation of autoimmunity or tumor [semanticscholar.org]

Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sharmi Biswas, M.B.B.S Caplans syndrome Microchapters Home Patient Information Overview Historical Perspective Classification Pathophysiology Causes Differentiating Caplans syndrome from other [wikidoc.org]

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]

Prevention

Secondary Prevention Cost-Effectiveness of Therapy Future or Investigational Therapies Case Studies Case #1 Caplans syndrome historical perspective On the Web Most recent articles Most cited articles Review articles CME Programs Powerpoint slides Images [wikidoc.org]

The most important task of occupational health care is prevention of occupational and occupation-related diseases. Preventive medical examinations are important for detecting diseases at early stages. [ujoh.org]

Prevention remains to be the most important measure. The article describes a case of rheumatoid pneumoconiosis in a patient in whom specific radiological changes were revealed during routine screening. [eestiarst.ee]

Prevention People with rheumatoid arthritis (RA) must not be exposed to additional risk factors for lung disease. [patient.info]

The antibiotics were given to prevent an infection of the damaged heart. However, antibiotics are now used much less often. You may need to limit activity that requires more work from your heart. Talk to your provider. [mclaren.org]

References

  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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