ABSTRACT
The over-secretion of monoclonal immunoglobulin light chains by clonal B cells followed by the aggregation and extracellular deposition of fibrillar deposits are responsible forthe clinical course AL amyloidosis. It is well documented that silica significantly increases the number of immunoglobulin-secreting cells. In the present paper, we report on a coal miner with silicosis and fast progressing primary amyloidosis with predominantly heart, kidney, and lung manifestations. Severeheart failure due to myocardial hypertrophy resulted in the patient's death. We conclude that long-term environmental silica exposure and silica deposition may contribute to the development of monoclonal gammopathy and amyloidosis due to chronic stimulus and the dysregulation of the immune system.
Subject(s)
Amyloidosis , Silicosis , Coal , Dust , Humans , Silicon Dioxide , Silicosis/complicationsABSTRACT
INTRODUCTION: The Churg-Strauss syndrome is a necrotic eosinophilic vasculitis affecting small and medium vessels. The etiology of this disease known to have an autoimmune background remains unclear. The Churg-Strauss syndrome is progressive and may involve multiple organs. Clinical manifestations are variegate, impeding the diagnosis. Typical symptoms include asthma, eosinophilia, mono- and polyneuropathy, sinusitis, pulmonary lesions and heart involvement. MATERIAL AND METHODS: We present a case of a patient with bronchial asthma who developed chronic sinusitis of the ethmoid, maxillary, and paranasal sinuses at the age of 37 years and was operated four times for this reason. At this age the patient also developed bilateral exophthalmos; normal thyroid function stood against the diagnosis of thyroid orbitopathy. The patient was treated by an ophthalmologist with glucocorticosteroids for three years. Glucocorticosteroids were withdrawn at the age of 40 years because of chronic pancreatitis. The patient suffered myocardial infarction at the age of 41 years and was diagnosed with the Churg-Strauss syndrome at the age of 44 years. CONCLUSIONS: The Churg-Strauss syndrome is a rare systemic disease with a variable course and variegate clinical manifestations which may be the reason for diagnostic difficulties.
Subject(s)
Churg-Strauss Syndrome/diagnosis , Adult , Chronic Disease , Exophthalmos/drug therapy , Exophthalmos/etiology , Humans , Male , Middle Aged , Referral and ConsultationABSTRACT
Amyloidosis is a generic term for a heterogeneous group of disorders associated with deposition of protein in an abnormal fibrillar form. We report a case of kappa L-chain primary lung amyloidosis. A 44-year-old woman without symptoms. Roentgenogram and computed tomography of the chest showed several small nodular shadows. The mammography revealed tumor of the breast and the metastatic breast cancer have been suspected. After the quadrantectomy the breast tumor has been diagnosed as adenoma. The diagnosis of pulmonary amyloidosis was established by an open lung biopsy. Immunohistochemistry revealed amyloid light (AL) of kappa-light chain origin. Serum protein electrophoresis was negative for monoclonal gammapathy. Over the next 6 years, the patient remained relatively asymptomatic. The case illustrates the problems with diagnosis of multiple solitary nodules and the long and relatively benign course of primary pulmonary amyloidosis.
Subject(s)
Amyloid/metabolism , Amyloidosis/diagnosis , Amyloidosis/metabolism , Immunoglobulin kappa-Chains/metabolism , Solitary Pulmonary Nodule/diagnosis , Solitary Pulmonary Nodule/metabolism , Adult , Biomarkers/metabolism , Biopsy , Diagnosis, Differential , Female , Humans , Lung Neoplasms/diagnosis , Solitary Pulmonary Nodule/pathologyABSTRACT
The aims of this study were to determine the proportion of rheumatoid arthritis (RA) patients attending hospital in whom amyloid deposits were present in abdominal fat aspiration (AFA) samples, and to assess possible risk factors for amyloid development in RA. One -hundred and twenty-one patients (16 males, 105 females) with RA referred to the Department of Rheumatology in Wroclaw between 1996 and 2001 were studied regardless of RA duration or laboratory findings. Abdominal subcutaneous fine-needle aspiration was performed, and samples of adipose tissue stained with alkaline Congo red then examined by polarized light microscopy. The presence or absence of amyloid fat deposits (AFD) was determined according to whether typical apple-green birefringence was observed. Amyloid deposits were found in 35 (29%) patients. Amyloidosis was significantly more common in males and in patients with longer disease duration. Patients with AFD had previously undergone less treatment with disease-modifying antirheumatic drugs (DMARDs) than those without AFD, and significantly fewer patients with AFD had previously taken methotrexate than those without AFD (25% vs 45%; p<0.01). Renal involvement was found in 12 of 35 patients with AFD (34%). Using the AFA technique, amyloid deposits were found commonly in RA patients, particularly in males with longer disease duration and in patients not treated intensively with DMARDs, especially methotrexate. AFA has potential useful application as a method for detecting amyloidosis before the overt occurrence of renal or other pathology related to amyloid deposits.
