1/10. Primary and secondary sjogren's syndrome in children--a comparative study.sjogren's syndrome is a chronic inflammatory systemic autoimmune disease mainly affecting the exocrine and, particularly, the salivary and lacrimal glands. The condition usually occurs in adults. In 1994, the criteria for this syndrome were redefined in a multicenter European study. In children, sjogren's syndrome is a rare and probably underdiagnosed disease. To date, sjogren's syndrome in children has only been described in case reports and in the comparative presentation of various study results. So far, no study of a comparative classification into primary and secondary sjogren's syndrome has been carried out in a patient population of any size. sjogren's syndrome should be considered in the differential diagnosis of children with recurrent parotitis, keratoconjunctivitis sicca, or pronounced and early tooth decay associated with xerostomia. In this study of 23 children and adolescents under the age of 16 with the clinical symptoms and laboratory findings of sjogren's syndrome, we differentiate between primary and secondary sjogren's syndrome. The value of the individual methods of assessing the oral and the ophthalmological components and the manifestation of the underlying rheumatic condition are discussed on the basis of the EULAR criteria. The EULAR diagnostic criteria are of limited applicability in children because reliable anamnestic data are frequently lacking. Another problem in diagnosing sjogren's syndrome is the short-term detection of serological alterations and clinical symptoms. Even if young patients do not completely fulfill the required criteria, sjogren's syndrome can be assumed or confirmed in the presence of positive testing for oral and ocular manifestations and recurrent salivary gland enlargement.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
2/10. Inflammatory pseudotumor of the submandibular gland: report of a case presenting with autoimmune disease-like clinical manifestations.We report a rare case of inflammatory pseudotumor arising in the submandibular gland, which presented with autoimmune disease-like clinical manifestations. A 70-year-old Japanese man developed masses in both submandibular regions. Laboratory tests revealed polyclonal hypergammaglobulinemia, high titers of antinuclear antibody, and a positive thyroid test. Histologically, the lesion was composed of multiple nodules separated by thick fibrous bands and contained a few atrophic lymphoid follicles and residual ductal structures. At higher magnification, the nodules contained numerous mature plasma cells mixed with myofibroblasts, lymphocytes, and histiocytes. Occasionally, the myofibroblasts were arranged in poorly formed fascicles and in a storiform pattern. polymerase chain reaction analysis failed to demonstrated the rearrangement of the immunoglobulin heavy-chain gene. The patient was free of disease after 72 months follow-up. Marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) type arising from salivary glands occasionally showed prominent plasma cell differentiation. The present case indicates that inflammatory pseudotumor should be added to the list of different diagnoses for mucosa-associated lymphoid tissue-type lymphoma of the salivary glands.- - - - - - - - - - ranking = 3.5keywords = gland (Clic here for more details about this article) |
3/10. Pulmonary cystic disorder related to light chain deposition disease.light chain deposition disease (LCDD) is a rare disorder that very uncommonly affects the lung. We report three cases of severe cystic pulmonary LCDD leading to lung transplantation. Such a presentation has never been previously reported. The three patients present with a progressive obstructive pulmonary pattern associated with numerous cysts diffusely distributed in both lungs. The disease was histologically characterized by non-amyloid amorphous deposits in the alveolar walls, the small airways and the vessels. It was associated with emphysematous-like changes and small airway dilation. Monotypic kappa light chain fixation was demonstrated on the abnormal deposits and along the basement membranes. Electron microscopy revealed coarsely granular electron-dense deposits in the same localizations. Mild extrapulmonary deposits were found in salivary glands in one patient. No immunoproliferative disorder was identified. We conclude that LCDD may primarily affect the lung, present as a pulmonary cystic disorder, and lead to severe respiratory insufficiency.- - - - - - - - - - ranking = 0.5keywords = gland (Clic here for more details about this article) |
4/10. Hereditary periodic fever with systemic amyloidosis: is hyper-IgD syndrome really a benign disease?We report a case of amyloidosis in association with hyperimmunoglobulinemia D syndrome (HIDS). The patient showed typical clinical features of HIDS. He had crescentic glomerulonephritis progressing to end-stage renal disease at age 13 years. Eight years later, he developed an AA-type amyloidosis with extensive involvement of the intestine, respiratory tract, and thyroid gland. These unusual complications of HIDS seriously challenge the assumption that the disease is associated with a good prognosis.- - - - - - - - - - ranking = 0.5keywords = gland (Clic here for more details about this article) |
5/10. Hyperimmunoglobulin E syndrome: treatment with isotretinoin.A patient with hyperimmunoglobulin E syndrome is described. Recurrent cutaneous staphylococcal abscesses were a prominent feature and had been resistant to treatment with antibiotics and surgical drainage. Following a 4-month course of treatment with isotretinoin, he developed no further abscesses over a 6-month follow-up period. Detailed immunological investigations were undertaken before and after treatment and no change was found to account for his improvement. We consider that the beneficial effects of isotretinoin in this situation are likely to be due to a reduction in sebaceous gland size and activity, rather than to any effect on the underlying disorder.- - - - - - - - - - ranking = 0.5keywords = gland (Clic here for more details about this article) |
6/10. A case of sjogren's syndrome with valvular diseases.The case of a 61 year old woman with sjogren's syndrome with aortic and mitral stenosis is reported. She suffered from rheumatic fever at a young age. Physical and echocardiographic examinations showed findings of mitral and aortic valve stenosis. In addition, she had experienced xerostomia, a gritty sensation in the eyes and Raynaud's phenomenon. Blood examination showed hypergammaglobulinemia, positive rheumatoid factor, antinuclear and anti-Ro (SS-A) antibodies. The diagnosis of sjogren's syndrome was confirmed by sialography and biopsy of the labial salivary gland. The combination of valvular disease and sjogren's syndrome is rare and the etiological correlation is discussed.- - - - - - - - - - ranking = 0.5keywords = gland (Clic here for more details about this article) |
7/10. IgE induces secretion of prostaglandin E2 by human monocytes.IgE was isolated from a patient with the hyper IgE, recurrent infection syndrome by immunoadsorption on sepharose bound goat anti-human IgE. Addition of this IgE to a monolayer culture of human monocytes resulted in a dose-dependent increase in PGE2 secretion. The addition of F(ab')2 fraction of goat anti-human IgE in the presence of sub-stimulating doses of IgE markedly increased PGE2 secretion; whereas addition of F(ab')2 fragment of irrelevant goat IgG had no effect. Similar activation of monocytes which could be enhanced by anti IgE was observed in the presence of the patient's serum. No such effect was seen in the presence of normal human serum. These results indicate that IgE may activate human monocytes and induce PGE secretion.- - - - - - - - - - ranking = 2keywords = gland (Clic here for more details about this article) |
8/10. Non-IgM monoclonal gammopathy in patients with sjogren's syndrome.Two Japanese patients with sjogren's syndrome with non-immunoglobulin m(IgM) class monoclonal gammopathy are described. The monoclonal IgA lambda detected in the serum and saliva was confirmed to possess rheumatoid factor activity in the first patient with a hypergammaglobulinemic purpura and hyperviscosity syndrome. Idiotype specificity was present on the surface membrane of peripheral blood lymphocytes as well as in the cytoplasm of infiltrating cells in the salivary glands. Common idiotypic specificity was found in four of 60 other patients who had rheumatoid factors. In the serum and saliva of the other patient, a monoclonal immunoglobulin g, kappa type (IgG kappa), was detected. Kappa type IgG was found in most of the infiltrating cells in the salivary glands and also in the saline extract from a resected submandibular gland. Our findings indicate that non-IgM class monoclonal gammopathy is also one of the complications of sjogren's syndrome.- - - - - - - - - - ranking = 1.5keywords = gland (Clic here for more details about this article) |
9/10. Systemic capillary leak syndrome and monoclonal IgG gammopathy; studies in a sixth patient and a review of the literature.The clincical and laboratory features of a sixth patient with periodic systemic capillary leak syndrome are reported. During an attack metabolic studies demonstrated a marked shift of plasma (10 to 70%) from the intravascular to the extravascular space resulting in hemoconcentration (highest hematocrit of 82). At the termination of the attack there was a return of the electrolytes, water and proteins to the intravascular compartment. The cardiovascular, renal and endocrine compensation was appropriate to this insult and no underlying abnormalities were demonstrated in these systems. The effector pathways of coagulation, complement, bradykinin generation, prostaglandins and histamine metabolism did not appear to be responsible for the altered capillary permeability. The patient was not missing inhibitors of these same pathways. The only persistently abnormal finding was a monoclonal IgG gammopathy. However, further studies of this paraprotein did not uncover a link between it and the abnormal capillary permeability. Five similar cases are reviewed; at least four and possibly all of these patients also had an IgG paraprotein. Treatment of these attacks was unsuccessful. Attemps to prevent the episodes with a wide variety of therapeutic agents failed. Treatment of the acute attacks with administration of intravenous fluids, did not maintain an adequate intravascular volume and may lead to fluid overload upon return of normal capillary integrity. Pressor agents were of no apparent value and may cause increased cardiac irritability. Although the clinical features and pathophysiology of the capillary leak syndrome have been defined, the etiology remains unknown.- - - - - - - - - - ranking = 0.5keywords = gland (Clic here for more details about this article) |
10/10. Aplastic anemia complicating sjogren's syndrome.A 47-year-old woman was referred to our hospital because of severe anemia and polyclonal gammopathy. She developed sicca syndrome after admission. Laboratory data revealed pancytopenia (white blood cells, 2,800/microliter; hemoglobin, 6.4 g/dl; platelets, 6.1 x 10(4)/microliter) and hyper gamma globulinemia (5.2 g/dl), and bone marrow was hypoplastic. histology of the salivary gland showed infiltration of lymphocytes. We report a good response to immunosuppressive therapy in a rare case of aplastic anemia complicating sjogren's syndrome.- - - - - - - - - - ranking = 0.5keywords = gland (Clic here for more details about this article) |
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