1/14. osteoporosis with underlying connective tissue disease: an unusual case.A 44-year-old male was initially seen by dermatologists, who noted an erythematous rash on sun-exposed areas, the back, shoulders, and upper arms. There was associated muscle weakness and significant weight loss. Investigation revealed mildly raised aspartate and alanine transaminases but normal creatine kinase. Inflammatory indices and antinuclear antibodies (ANAs) were normal. biopsy of the rash was reported as consistent with either dermatomyositis (DM) or acute lupus erythematosus. A diagnosis of DM was made, and prednisolone was given with improvement of the rash but deteriorating myopathy. The patient was referred to the rheumatology department, and further history revealed multiple vertebral fractures after falling from standing height; these had occurred six months prior to starting steroids. Besides smoking he had no other risk factors for osteoporosis. Examination showed normal muscle strength, no muscle tenderness, and no joint abnormality. Repeat muscle enzymes were normal, and ANAs were now 1 : 100, but dsDNA antibodies and extractable nuclear antigens were normal. Investigations for osteoporosis revealed a hypergonadotrophic hypogonadism picture. Further examination indicated scanty pubic and auxiliary hair, small testicles, and mild gynecomastia. He is married, though has no children of his own. The hormonal profile raised the possibility of Klinefelter's syndrome, which was subsequently confirmed with karyotyping of 47 XXY. hypogonadism has been established as a cause of osteoporosis in males, and in this case would explain the occurrence of fractures in the absence of other major risk factors. Systemic lupus erythematosus has been recognized in association with Klinefelter's syndrome; in view of the normal muscle enzymes, his rash is most likely due to acute discoid lupus with androgen deficiency causing muscle weakness.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/14. Middermal elastolysis. Report of a case and immunohistochemical studies on the dermal distribution of fibrillin, vitronectin and amyloid P component.A 39-year-old woman with demarcated wrinkled areas, histologically characterized by absence of elastic fibers in the middle and upper reticular dermis, is described. Immunoreactivity of vitronectin and amyloid P component, present at the periphery of elastic fibers in normal skin in adults, was absent from the middermis of lesional skin as were orcein stained fibers. C9 neoantigen immunoreactivity, associated with elastic fibers in sun-exposed skin of middle-aged and elderly individuals, was present in conjunction with elastic fibers in papillary and lower reticular dermis in lesional skin but was absent in the middermis. In contrast, a fibrillin immunoreactive network was present throughout the dermis, indicating that the elastin-associated microfibrils are retained in the absence of amorphous elastin in lesional skin of middermal elastolysis.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
3/14. Raynaud's phenomenon with digital necrosis as the first manifestation of undifferentiated connective tissue syndrome.A patient with severe Raynaud's phenomenon (RP) associated with the presence of antibodies against extractable nuclear antigen (ribonucleoprotein) in the context of undifferentiated connective tissue syndrome is presented. The rapid course of digital necrosis in her case resulted in the surgical amputation of 9 of her fingers. We conclude that massive digital necrosis accompanied by severe RP could constitute the first manifestation of a connective tissue disease.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
4/14. Familial sjogren's syndrome in the Japanese: immunogenetic and serological studies.Serological abnormalities and HLA haplotypes were studied in a Japanese family of two patients with sjogren's syndrome (SS) associated with other autoimmune diseases. In contrast to the reports in the U.S.A. and europe, we found a significant excess of HLA-DRw53 antigen in the family members. Two family members, mother and niece of the probands, had suffered from other connective tissue diseases. Although none of the siblings of probands had manifestations of connective tissue diseases, two siblings had several autoantibodies. No consistent segregation, however, was found between the HLA haplotypes and the serological abnormalities in the relatives. Therefore, HLA alone can not explain the familial clustering of autoimmune diseases including SS and of autoantibodies.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/14. Excessive hypercalcaemia and mixed connective tissue disease.A 23-year-old female with extreme hypercalcaemia was treated with calcitonin, mitramycin and parathyroidectomy and normocalcaemia was achieved after 4 weeks. Nevertheless, the patient later died of cutaneous necrosis, impaired circulation and multiple organ failure. serum immunoreactive parathyroid hormone was in the normal range and parathyroid tissue normal. mixed connective tissue disease was diagnosed on the basis of high titers of antibody to extractable nuclear antigen, moderately elevated levels of antibody to nuclear antigen and only marginal elevation of anti-double standed dna. The role of Cl. difficile toxin in the blood and an acinic cell tumour is unclear.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
6/14. A direct immunofluorescence study in elastosis perforans serpiginosa.Two cases of elastosis perforans serpiginosa were studied by direct immunofluorescence. In one case, homogeneous deposits of IgM, C3 and C4 on the abnormal elastic fibres in the papillary dermis were demonstrated. In addition, in both cases, along the basement membrane zone, coarse linear deposits of fibrinogen and properdin were observed inside and near the transepidermal channels. Groups of cytoid bodies stained for IgM and occasional weak linear deposits of IgG, IgA, IgM and C3 were also observed. The immune deposits on the abnormal elastic fibres might indicate antigenic alteration and activation of humoral immunological mechanisms in the process of their elimination. The other findings may reflect a non-specific binding by damaged tissue structures.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
7/14. The precipitating antibody to an acidic nuclear protein antigen, the Jo-1, in connective tissue diseases. A marker for a subset of polymyositis with interstitial pulmonary fibrosis.The clinical significance of antibodies to the Jo-1 antigen in connective tissue diseases was studied. Clinical diagnoses of 11 patients who had anti-Jo-1 antibody were: polymyositis 8, dermatomyositis 1, and overlap syndrome 2 (polymyositis--systemic lupus erythematosus 1, polymyositis--scleroderma 1). All the patients who had anti-Jo-1 antibody showed interstitial pulmonary fibrosis, and in 2 patients anti-Jo-1 antibodies were detected before the appearance of lung disease.- - - - - - - - - - ranking = 5keywords = antigen (Clic here for more details about this article) |
8/14. Multiple clonally-restricted immunoglobulins in human sera: disease associations.Diseases associated with multiple clonally-restricted serum immunoglobulin (Ig) abnormalities present at levels which, in most cases, preclude detection by conventional immunoelectrophoresis, were studied using methods for detection and characterization of homogeneous Ig that are approximately equal to 40 times more sensitive than either cellulose acetate zone electrophoresis or immunoelectrophoresis. patients with these Ig abnormalities had a high incidence of infectious disease (29% of total cases), malignancy (19%), connective tissue disease (14%) and liver disease (10%). The concentration of individual clonal products was found to wax and wane, but it could not be determined whether these clonally-restricted Ig species represent, wholly or in part, the products of dominant antibody-producing plasma cell clones involved in the patients' response to their disease. We conclude that multiple homogeneous serum Ig abnormalities occur in clinical situations where heightened antigenic stimulation and/or immune reactivity are thought to occur (e.g., infections, malignancies and autoimmunity). Laboratory evaluation of these Ig abnormalities could be useful for diagnostic and/or therapeutic monitoring purposes in situations where the specificity of the clonally-restricted Ig species can be established.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
9/14. immunosuppression and the rheumatic diseases.Ignorance of the basic nature of rheumatoid arthritis precludes the introduction of rational schemes for using cytotoxic drugs. It is still plausible that the autoimmune and other immunological abnormalities which accompany this disease are the secondary effects of persistent antigen, for example, related to microbial infections. In this event, cytotoxic drugs may diminish the inflammatory response but their effects on immune responses would be irrelevant or even undesirable. Should rheumatoid arthritis prove to be a primary immunoproliferative disorder, cytotoxic drugs may prove to be of value not because of their conventional immunosuppressive effects but because of their selective action on the proliferating cells. Indeed, current evidence suggests that these drugs enhance rather than depress conventional immune responses, at least in the doses given to patients with rheumatic disorders.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
10/14. Connective tissue panniculitis.Two patients with lobular lymphohistiocytic panniculitis had chronic lesions that produced subcutaneous atrophy and that were responsive to antimalarial drugs. Massive lymphocytic infiltrate was associated with caseation necrosis of the fat lobules. In both patients, an unusual antibody to extractable nuclear antigen was seen, and antinuclear antibody was sometimes present. The unusual manifestation, course of the disease, serologic findings, and response to therapy suggest that the panniculitis is related to (but does not evolve into) lupus erythematosus panniculitis or subcutaneous morphea or both. A satisfactory term for this entity would be "connective tissue panniculitis."- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
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