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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.

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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.

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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.

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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.

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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.

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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.

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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.

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The authors describe two cases suffering from Caplan s syndrome, i.e. a combination of pneumoconiosis and rheumatoid arthritis, in miners of deep coal mines in southern Moravia. The finding on the lungs preceded the manifestation of articular symptoms. The disease progressed even after the risk of fibrogenic dust was eliminated. In both patients occupational diseases were involved.

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We report the case of a 59-year old patient with the rare combination of silicosis and rheumatoid arthritis, which is called Caplan's syndrome. The patient presented with right heart failure caused by a pericardial tumor compressing the right and the left ventricle. By means of several imaging techniques it was possible to elucidate the topographic relations of the tumor. Definite signs of malignant growth were not found. Despite his elevated perioperative risk we decided to operate on the patient. During the operation macroscopic and histologic evidence revealed that the tumor was not a neoplastic process but consisted of an organized hemopericardium. This demonstrates that pericarditis in rheumatoid arthritis can be hemorrhagic and can mimick a malignant pericardial tumor. The decision to operate was first supported by the findings of the applied imaging techniques, the normal endomyocardial biopsy, and the clinical course and were later confirmed in situ.

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Rheumatoid changes in the lungs are seen in 30% of cases of rheumatoid arthritis. Three cases with different patterns of pulmonary involvement in the course of rheumatoid arthritis (RA) are presented. In all cases pleural involvement was seen, in two subjects since the beginning, in the remaining in the course of developing interstitial lung disease. In two cases the dominant changes in the respiratory system were interstitial pneumonitis. In one case the interstitial pneumonitis took a fulminating course, being resistant to treatment with prednisone, endoxan, penicillamine. In the third case, the interstitial pneumonitis was of chronic character, despite the fact that the subject did not receive any treatment, it did not progress. In the first case the main features were pulmonary cavities. The could have evolved from necrosis of rheumatoid nodules or vessel changes.

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Thirteen cases of Caplan's syndrome were investigated by chest X-ray, rheumatic and immunological tests, heart and lung physiology and pathological-anatomical specimens. No positive correlation was found between exposure time to silica, roentgenolgical findings, degree of rheumatoid factor or physiological findings. In nine of the cases a rapid roentgenological progress took place. Although the combination of lung infiltrations and rheumatic disorders makes an immunological reaction probable, no correlation was found between the degree of rheumatic disorder and roentgenological progress. The hypothesis that silica acts as an adjuvant, which may strengthen an antigen-antibody reaction, was not reflected in rheumatic parameters. The roentgenological examination appeared to be the most sensitive method for following the disease.

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