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.

Silicosis pulmonar: hallazgos radiológicos en la tomografía computarizada / Silicosis: computed tomography findings

La silicosis es una enfermedad pulmonar ocupacional, causada por la inhalación de sílice, que afecta a un amplio abanico de profesiones. Existen varias formas clínicas. La silicosis aguda, que resulta de la exposición a cantidades muy grandes de sílice en un período inferior a 2 años. La silicosis crónica simple, el tipo más frecuente que podemos ver en la actualidad, resulta de la exposición a bajas cantidades de sílice durante un período de entre 2 y 10 años. La silicosis crónica complicada, con conglomerados silicóticos. En muchos casos el diagnóstico se realiza por los datos epidemiológicos y radiológicos, sin confirmación histológica. Es importante conocer las distintas manifestaciones radiológicas de la silicosis para diferenciarla de otras enfermedades pulmonares y reconocer sus posibles complicaciones concomitantes. El objetivo de este trabajo es describir los hallazgos radiológicos, típicos y atípicos, de la silicosis y sus complicaciones en la TC de tórax helicoidal y de alta resolución (TCAR) (AU)

Silicosis is an occupational lung disease, which is caused by the inhalation of silica and affects a wide range of jobs. There are many clinical forms of silicosis: acute silicosis, results from exposure to very large amounts of silica dust over a period of less than 2 years. Simple chronic silicosis, the most common type that we see today, results from exposure to low amounts of silica between 2 and 10 years. Chronic silicosis complicated, with silicotic conglomerates. In many cases the diagnosis of silicosis is made according to epidemiological and radiological data, without a histological confirmation. It is important to know the various radiological manifestations of silicosis to differentiate it from other lung diseases and to recognize their complications. The objective of this work is to describe typical and atypical radiological findings of silicosis and their complications in helical and high resolution (HRCT) thorax CT (AU)

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.