Treatment with Rituximab in Rheumatoid Pneumoconiosis: A Case Report.

BACKGROUND: Caplan's syndrome, also known as rheumatoid pneumoconiosis (RP), is a rare disease associating pneumoconiosis with rheumatoid arthritis (RA). This is one of the rare cases evaluating the effect of Rituximab, which was used initially for the treatment of RA, on pneumoconiosis Case Presentation: In this case report, we described a 21-year long-standing history of pneumoconiosis and its association with RA. A 67-year-old man diagnosed with pneumoconiosis presented with morning stiffness and symmetrical polyarthritis. Laboratory investigations showed high titers of rheumatoid factor (RF) and anti-citrullinated protein antibodies. The diagnosis of RA was established and the patient was put on leflunomide. Then, he was treated with Rituximab, as he did not respond to leflunomide. The patient showed marked improvement as pain and swelling decreased. More importantly, Caplan's nodules stabilized on chest-computed tomography. CONCLUSION: The use of rituximab in pneumoconiosis does not alter the evolution of the pulmonary nodules. More trials are needed to establish a treatment consensus for RP.

Colinet-Caplan Syndrome: History of an Outbreak of Autoimmune Disease in Scouring Powder Workers.

The first modern description linking rheumatoid arthritis to occupational dust exposure is generally attributed to the British physician Anthony Caplan. In 1953, Caplan reported on a "peculiar" nodular pattern on chest radiographs of Welsh coal miners with rheumatoid arthritis that differed from the typical coal workers' pneumoconiosis. However, as early as 1950, the Belgian rheumatologist Émile Colinet described a similar case of rheumatoid arthritis and concomitant pulmonary opacities in a 30-year-old woman with silica exposure. Soon after, he published a second case. Although this condition initially was called Colinet-Caplan syndrome in the Francophone biomedical literature, Colinet's name was later dropped from the eponym. Because Colinet never clearly described the specific occupational context of his cases, Caplan syndrome has been misconstrued as uniquely a disease of coal miners.We attempted to reconstruct the working conditions of Colinet's patients and found that they were packing Vim, a silica-based scouring powder, at the Savonneries Lever Frères factory in Brussels, Belgium. Colinet's cases were only the first 2 in a series of reports of rheumatoid arthritis and other autoimmune diseases, mainly among young women, in those who worked in the production of silica-based scouring powder between the 1930s and 1980s across Europe. The largest outbreak involved 32 cases of autoimmune disease among 50 former workers of a Spanish scouring powder manufacturing facility. After silica in scouring powders was replaced with less hazardous materials later in the 20th century, no further cases have been reported.Although scouring powder disease is a historical phenomenon, autoimmune disorders linked to occupational exposure to silica and coal dust have not disappeared but instead are reemerging among those who work with silica-based artificial stone and in other dusty trades.

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.

Amyloid A amyloidosis in a patient with Caplan's syndrome, with special reference to genetic predisposition.

Secondary amyloid A (AA) amyloidosis, which is a disorder of protein conformation and metabolism, is an important serious complication of inflammatory diseases, especially rheumatoid arthritis (RA). AA amyloidosis develops when AA fibrils, which are derived from the acute-phase reactant, serum amyloid AA (SAA) protein, in the circulation, are deposited in organs and cause systemic organ dysfunction. Caplan's syndrome, or rheumatoid pneumoconiosis, is a rare type of lung disease in which individuals suffering from RA develop lung nodules that are associated with occupational exposure to silica and coal dust. Confirmation of diagnosing as Caplan's syndrome requires the patient's occupational history, imaging studies, and serology. A 72-year-old male, working as a tunnel construction worker for 38 years, with RA who had both chronic cardiac and renal dysfunction was referred to our hospital. He received a diagnosis of pneumoconiosis about 20 years ago, after which he was also diagnosed with RA. So far we performed medical English literature searches on the combination of Caplan's syndrome with AA amyloidosis; there were no articles in relation to such association. Although RA is one of the most common underlying diseases that occur with AA amyloidosis, our report here is the first description of a case of Caplan's syndrome associated with AA amyloidosis. In this report, we provide details about this rare disease occurring with AA amyloidosis and discuss on the possible pathogenesis of AA amyloidosis from a genetic point of aetiological view.

Atypical presentation of rheumatoid arthritis: a case of massive bilateral pleural effusions in a patient with Caplan syndrome.

Rheumatoid arthritis (RA) is a common connective tissue disorder affecting the synovial joints. In patients with RA, involvement of the lungs occurs in 30%-40% of cases while pleural effusions occur in only 3%-5%. However, the majority of RA-associated pleural effusions are small, unilateral and asymptomatic. We present a case of massive bilateral pleural effusions in a patient with established rheumatoid pneumoconiosis (Caplan syndrome). Interestingly, the pleural effusion occurred following recent treatment for minimal change disease and atrial fibrillation.

What to do with all of these lung nodules?

Caplan syndrome is a rare entity that is specific to rheumatoid arthritis and presents with multiple, well-defined necrotic nodules in patients with occupational dust exposure. The present report describes a case of Caplan syndrome involving a 71-year-old man with a known diagnosis of seropositive rheumatoid arthritis who presented to the authors' centre with a five-year history of multiple, bilateral cavitary lung nodules with mild dyspnea on exertion. He was an ex-smoker (30 pack-years) and had previously worked with silica. The case highlights the clinical, radiological and pathological features of this syndrome and outlines the importance of considering a broad differential in the management of pulmonary nodules, especially in patients with rheumatoid arthritis.

[Caplan's syndrome: rarely presenting as <> syndrome]. / Caplan-Syndrom: eine untypische Differenzialdiagnose des «pulmorenalen¼ Syndroms.

HISTORY AND ADMISSION FINDINGS: A 59-year-old man complained about having dry cough for months and a recent sudden onset of minor hemoptoe, asymmetric arthritis, myalgia as well as lack of appetite. He presented an occupational history of 12-year exposure to an organic dust as uranium miner in German Democratic Republic followed by 21 years as heavy construction worker in Germany and in Switzerland. Laboratory work-up tested positive for microhematuria and anti-neutrophilic cytoplasmic antibodies (ANCA). Chest X-rays and CT scan showed bilaterally scattered nodules. Thoracoscopic wedge resection was performed, histopathological analysis revealed granuloma with central necrotic area containing black coal dust and silica depositions surrounded by histiocytes. The pulmonary opacities on X-ray and the typical histology in the light of significant dust exposure allow the diagnosis of a Caplan's syndrome. TREATMENT AND COURSE: The symptoms improved rapidly under steroid therapy. Further investigations revealed a clear renal cell carcinoma as a cause for the persistent microhematuria. CONCLUSION: Rheumatoid arthritis, pulmonary nodules and history of prolonged dust exposure are classical findings that define Caplan's syndrome. These patients present with different immunological phenomena - in our case ANCA-positivity without vasculitis. Interestingly, the renal cell carcinoma which led to the "pulmorenal" syndrome in our patient is another health problem overrepresented in uranium mine workers.

Pulmonary manifestations of collagen diseases.

Collagen diseases are a large group of systemic inflammatory diseases of autoimmune etiology. The etiopathogenesis of collagen diseases is multifactorial. There is genetic susceptibility, as many connective tissue disorders show family history, and environmental factors may trigger the disease. Collagen diseases can affect almost all the organs of the body. The respiratory system is one of the most frequently affected, although the prevalence of pulmonary disease is not precisely known for the different collagen disorders. Any structure of the respiratory tract can be affected, but perhaps the most frequent is pulmonary parenchymal disease in the form of pneumonitis, which can be produced in any of the idiopathic interstitial pneumonitis patterns. The pleura, pulmonary vessels, airways and respiratory muscles may also be affected. The frequency of lung disease associated with collagen diseases is on the rise. This due in part to the better diagnostic methods that are available to us today (such as high-resolution computed tomography) and also to the appearance of new forms of pneumonitis associated with the new treatments that are currently used. The objective of this article is to offer a global vision of how collagen diseases can affect the lungs according to the latest scientific evidence.

[Rheumatoid arthritis and interstitial lung alterations - a clear case, isn't it?]. / Rheumatoide Arthritis und interstitielle Lungenver-änderungen - Ein klarer Fall! Oder eben doch nicht?

In a patient with rheumatoid arthritis (RA) and asymptomatic, diffuse reticulo-nodular lung parenchymal alterations with upper lobe predominance, a Caplan syndrome (CS) was diagnosed. According to the size of the pulmonary nodules, classification into two subtypes of the CS has been proposed: the classic (Caplan) type and the silicotic type. Patients with CS often present with considerable x-ray or computertomographic changes but relatively few symptoms. However, in case of respiratory symptoms, infectious complications or pneumotoxic side effects of the immunsuppressive/immune-modulating pharmacotherapy for RA must be encountered in the differential diagnosis.

Rheumatoid pneumoconiosis (Caplan's syndrome).

In 1953, Caplan described a characteristic radiographic pattern in coal miners with rheumatoid arthritis (RA) that was distinct from the typical progressive massive fibrosis pattern of coalworkers' pneumoconiosis. It consists of multiple well-defined rounded nodules on chest X-ray, from about 0.5 to about several centimetres in diameter, distributed throughout the lungs but predominantly at the lung periphery. Lesions appear often in crops, may coalesce and form a larger confluent nodule. Nodules often cavitate or calcify. They typically occur in the setting of pre-existing mild pneumoconiosis, but pneumoconiosis is not a prerequisite. The onset of the nodules is typically sudden, and their course varies thereafter, ranging from regression to progression. Histologically, the nodules have a characteristic appearance and are distinguishable from silicotic nodules or progressive massive fibrosis. Individual susceptibility is considered to play a role in the development of the disease. However, the pathogenetic link between exposure to silica, pneumoconiosis and RA has not been clarified conclusively. This review summarizes history, definition and current knowledge on epidemiology, pathology, pathophysiology, clinical presentation and treatment of Caplan's syndrome.

[Caplan's syndrome in marble workers as occupational disease].

Rheumatoid pneumoconiosis is an uncommon combination of occupational lung disease caused by exposure to harmful silica dust with rheumatoid inflammation of the joints, rheumatoid arthritis, with an autoimmune background. Until now, the disease was observed mostly among coal and gold miners and granite workers. Written documents on the theme are summarized. This case study outlines the syndrome pathology with typical features presented by the worker, employed for many years in the marble industry. Although in general marble is free of silica, the collection of occupational anamnesis and familiarity with the patient's work conditions and demands gave the authors an opportunity to uncover the exposure source and to determine the most probable diagnosis.

Rheumatoid pneumoconiosis (Caplan's syndrome) with a classical presentation.

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.

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.

Parkinsonism in Caplan's syndrome.

Parkinsonism is a rare feature of immunological diseases. We describe a 67-year-old man with Caplan's syndrome (CS) and parkinsonism. CS is an immunologic disease characterised by the presence of rheumatoid arthritis associated with a specific form of pneumoconiosis. Parkinsonism as a manifestation of involvement of the central nervous system in this condition has never been reported. Following immunosuppressive treatment both the CS and the parkinsonian signs and symptoms showed a marked improvement. The role of immune mechanisms in these parkinsonian syndromes is discussed.

[New possibilities of making the diagnosis of Caplan syndrome]. / Nowe mozliwosci rozpoznawania zespolu Caplana.

We describe a 56-year old man with rheumatoid arthritis and pulmonary nodules detected by HRCT. Needle biopsy confirmed the diagnosis of Caplan syndrome.

[Caplan's syndrome: case report]. / Przypadek zespolu Caplana.

A case of a 56-years old male with rheumatoid arthritis and unclear tumoral radiological changes in the lungs was described. Since noninvasive diagnostic procedures failed to explain nature of the pulmonary changes, an open pulmonary biopsy was performed. Pathological examination revealed presence of rheumatoid nodules and pneumoconiosis, typical for Caplan's syndrome. In the past, the patient had been working in foundry industry for 16 years and he had been exposed to silica and iron dust. Articular symptoms were revealed prior to finding the lung changes even dust exposure had occurred many years earlier. In spite of the fact that rheumatoid arthritis is a relatively common disease and pneumoconiosis is also not rare entity, the coexistence of both conditions i.e. Caplan's syndrome has been rarely diagnosed and described.