Síndrome de Caplan: la paradoja del pulmón como origen de la artritis reumatoide / Caplan syndrome: the paradox of the lung as the origin of rheumatoid arthritis

Resumen Históricamente, los estudios de campo observacionales han sido el punto de partida para el desarrollo de grandes avances en el entendimiento de las enfermedades autoinmunes. En el caso de la artritis reumatoide (AR), se han descrito varias asociaciones clínicas hasta la actualidad que tienen en común modelos inmunológicos transversales a la historia natural de la misma, lo que ha permitido avanzar en el desarrollo de nuevos objetivos terapéuticos. Se pretende hacer una breve descripción del compromiso nodular en AR, partiendo de las observaciones clínicas del Dr. Anthony Caplan en pacientes con riesgo de neumoconiosis, con el fin de reconocer el valor de este tipo de asociaciones en el ejercicio médico profesional.

Historically, observational field studies have been the starting point for the development of great advances in the understanding of autoimmune diseases. In the case of rheumatoid arthritis (RA), several clinical associations have been described to date which have in common immunological models transverse to its natural history, which has allowed progress in the development of new therapeutic objectives. Our aim is to make a brief description of nodular involvement in RA, based on the clinical observations of Dr. Anthony Caplan in patients at risk of pneumoconiosis, in order to recognize the value of this type of association in professional medical practice.

A Case of Rheumatoid Pneumoconiosis Presenting with Pleuritis and Pericarditis / 대한내과학회지

Caplan's syndrome is characterized by multiple small distinct nodules with progressive massive fibrosis and rheumatic arthritis in pneumoconiosis. Although pleural effusions occur infrequently as an extra-articular manifestation, pleuritis can develop without joint involvement in patients with rheumatoid arthritis. We treated an 81-year-old man who had been diagnosed with silicosis with progressive massive fibrosis. He suffered from progressive dyspnea, and chest computed tomography (CT) and echocardiography revealed pleural and pericardial effusions. We speculated that the multiple serositis was related to a rheumatic disorder because the rheumatic factor was elevated in both the pleural and pericardial effusions. After corticosteroid treatment, the serositis improved. We suggest that this case is an atypical pattern of Caplan's syndrome presenting as serositis without arthritis. Rheumatoid serositis should be considered as the cause of pleural or pericardial effusions in patients with pneumoconiosis.

A Case of Caplan's Syndrome Presenting as Acute Onset Polyarthritis

Caplan originally described distinctive pulmonary nodules in miners who had suffered from rheumatoid arthritis. Later, the pulmonary nodules, together with a history of rheumatoid arthritis and exposure to inorganic dust were called Caplan's syndrome. This syndrome has been described by case reports in many countries but only two cases have been reported in Korea up to now. The patient in this case report was a 70-years-old man who had worked in the construction field for 20 years mainly demolishing buildings. He was diagnosed with silicosis by a lung biopsy 1 year prior to admission. He suddenly developed arthralgia and morning stiffness in multiple joints 2 weeks prior to admission. Chest imaging revealed aggravation of the bilateral pulmonary nodules. He was diagnosed with seropositive rheumatoid arthritis. The lung nodules, arthralgia, and morning stiffness improved clinically after treatment with the corticosteroid and disease modifying anti-rheumatic drugs.

Caplan's Syndrome Presenting as Multiple Pulmonary Nodules / 결핵및호흡기질환

We report a case of Caplan's Syndrome, which presented as multiple pulmonary nodules. A 58-year-old male was admitted to hospital due to multiple pulmonary nodules. In addition, the patient presented with multiple arthritis, and dyspnea on exertion. Rheumatoid arthritis had been diagnosed 35 years ago. The patient had worked as a stonemason for 20 years. Computed Tomography (CT) revealed numerous well-defined tiny nodules scattered in both lungs, which was suspicious of miliary tuberculosis or malignancy. The patient was started on antituberculous medications and referred to our hospital. First, a transbronchial lung biopsy was performed, which showed no evidence of granuloma. It was our opinion that the biopsy was insufficient, and a follow-up video-associated thoracoscopy was performed. The pathological report determined necrotizing granulomatous inflammation and silicosis on background. According to imaging studies, pathologic reports, and clinical symptoms, we concluded that the patient had Caplan's syndrome. We controlled his rheumatic medications, and instructed him to avoid exposure to hazardous dust.

Pneumoconiose reumatoide (síndrome de Caplan) com apresentação clássica / Rheumatoid pneumoconiosis (Caplan's syndrome) with a classical presentation

Apesar de rara, a pneumoconiose reumatoide, também chamada de síndrome de Caplan, pode ser diagnosticada entre trabalhadores expostos à sílica e entre pacientes com silicose, pneumoconiose dos mineiros de carvão e asbestose. A maior prevalência ocorre entre os silicóticos, apesar de ter sido descrita inicialmente em mineiros de carvão com pneumoconiose. O achado que define o tipo clássico da síndrome é a presença de nódulos reumatoides nos pulmões, independente da presença ou não de pequenas opacidades pneumoconióticas, ou de grandes opacidades de fibrose pulmonar maciça, associada ou não a um quadro de artrite reumatoide em atividade. Relatamos o caso de uma mulher com quadro de artrite reumatoide, diagnosticada 34 anos após exposição à sílica livre em uma indústria de porcelana por 7 anos, apresentando radiograma de tórax com opacidades arredondadas de 1 a 5 cm de diâmetro, distribuídas na periferia de ambos os pulmões. A biópsia transtorácica guiada por TC de um dos nódulos revelou tratar-se de nódulo reumatoide com macrófagos em paliçada, típico da síndrome de Caplan. São discutidos aspectos de diagnóstico da síndrome, classificação e ocorrência, ressaltando a importância da anamnese ocupacional em casos de artrite reumatoide com opacidades radiológicas pulmonares.

Although rare, rheumatoid pneumoconiosis, also known as Caplan's syndrome, can occur in workers exposed to silica, as well as in patients with silicosis, coal workers' pneumoconiosis or asbestosis. Prevalence is higher among patients with silicosis, despite the fact that it was originally described in coal workers with pneumoconiosis. The classical finding that defines this syndrome is that of rheumatoid nodules in the lungs, regardless of whether there are small rounded opacities suggestive of pneumoconiosis or large opacities consistent with massive pulmonary fibrosis, with or without clinical rheumatoid arthritis. We describe the case of a female patient with rheumatoid arthritis, diagnosed 34 years after 7 years of occupational exposure to silica at a porcelain plant. A chest X-ray showed circular opacities of 1-5 cm in diameter, bilaterally distributed at the periphery of the lungs. A CT-guided thoracic punch biopsy of one of those nodules revealed that it was rheumatoid nodule surrounded by a palisade of macrophages, which is typical of Caplan's syndrome. Aspects of diagnosis, classification and occurrence of this syndrome are discussed, emphasizing the importance of the occupational anamnesis of patients with rheumatoid arthritis and lung opacities on chest X-rays.

A Case of Caplan's Syndrome with Silicosis / 대한산업의학회지

BACKGROUND: Caplan originally described a condition, now reffered to as Caplan's syndrome or Caplan's disease, where progressive massive fibrosis was exhibited by coal miners with multiple pulmonary nodules, and rheumatoid arthritis. This syndrome is very rare in Korea. CASE REPORT: The patient was a 49-year old male, with occupational history as a stonemason for 25 years. Silicosis was diagnosed by chest X-ray, while rheumatoid arthritis was diagnosed by immunological examination. Caplan's syndrome was confirmed based on these findings, occupational history, and clinical symptoms. DISCUSSION: The authors report on the patient's Caplan's syndrome, with a particular focus on his silicosis.

Artrite reumatóide e fibrose pulmonar: relato de caso / Rheumatoid arthritis and pulmonary fibrosis: report of a case

Os autores descrevem o caso de uma paciente de 42 anos, portadora de artrite reumatóide (AR) fortemente soropositiva de dez anos de evoluçäo, com antecedente de exposiçäo à sílica por quatro anos posterior ao desenvolvimento da AR. Apresentou-se com quadro de dispnéia progressiva, com evidênias radiológicas de fibrose pulmonar. Rapidamente evoluiu para insuficência respiratória, complicada por infecçäo, indo a óbito em cinco dias. Achados necroscópicos revelaram intensa fibrose pulmonar intersticial, bronquiectasia infectada com metaplasia escamosa, espessamento de paredes arteriais e cor pulmonale. Pesquisa de sílica resultou negativa

Síndrome de Caplan: relato de caso / Caplan's syndrome: report of a case

Trata-se do relato de um caso de síndrome de Caplan em paciente masculino, com 59 anos, com história de ter trabalhado em tunelizaçäo dos 17 aos 20 anos. Esteve assintomático até 53 anos quando apresentou escarros hemópticos. Biopsia pulmonar demonstrou sílico-antracose sem granuloma tuberculoso. Tratamento com drogas antituberculose por seis meses näo alterou o aspecto radiológico. Seis meses antes já vinha apresentando dor com flogose das articulaçöes, acompanhada de febre matinal. Houve aparecimento de nódulos subcutâneos na superfície extensora de cotovelos. Exame radiológico do tórax mostrava vários gânglios na cadeia paratraqueal e carena, com calcificaçöes na periferia. Biopsia do nódulo subcutâneo revelou tratar-se de nódulo reumatóide. Prova de funçäo pulmonar mostrou padräo obstrutivo leve com hipoxemia. Houve melhora dos sintomas com aspirina

Sindrome de Caplan en un minero. / Caplan's syndrome in a miner

Se describe el primer caso de sindrome de Caplan en un minero de este pais, que padecia silicosis por haber trabajado expuesto durante muchos anos a polvos de silice y silicatos. Se reviso la literatura relacionada al caso y se hacen comparaciones con los casos informados en ella

Sindrome de Caplan e po de pedra. / Caplan's syndrome and silica dust

Os autores apresentam um caso de artrite reumatoide e fibrose intestinal multinodular, em operador de moinho de pedra, configurando a sindrome de Caplan. A doenca revelou-se mutilante, levando ao grau II/III da artrite reumatoide, principalmente das pequenas articulacoes das maos e dos pes