1/21. The relationship between facial annular erythema and anti-SS-A/Ro antibodies in three East Asian women.A distinct annular erythema developed on the cheeks of three East Asian women who had anti-SS-A/Ro (SSA) antibodies. The erythema was characterized by a wide, elevated border and central pallor. Histologically, there was a coat-sleeve-like infiltration of lymphocytes around the blood vessels, appendages, and secretory gland cells in the dermis. Immunohistological analysis clarified that the majority of infiltrating lymphocytes were CD4-positive T cells. Abnormal expression of hla-dr antigens in the perivascular, appendage, and secretory gland cells in the dermis was also observed. The differential diagnosis of the three patients lay between Sjogren syndrome (SjS), Sjogren/systemic lupus erythematosus overlap syndrome and an asymptomatic clinical state. These results are consistent with recent findings of major histocompatibility complex class II expression on target organs in various autoimmune diseases. Based on these findings, erythema appears to represent a broad cutaneous manifestation of these diseases. Furthermore, the presence of SSA antibodies, aberrant HLA-DR expression, and sun exposure may be responsible for the development of erythema.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/21. Neuropsychological, neuroimage and psychiatric aspects of primary sjogren's syndrome.We report a case of a 49-year-old woman diagnosed with primary Sjogrens Syndrome (pSS) who was submitted to extensive neuropsychobiological assessment. Examination revealed a Wechsler adult intelligence Scale-Revised (WAIS-R) Full Scale IQ of 97 with no Verbal/Performance IQ discrepancy and performance below estimated premorbid levels on arithmetic skills, visual tracking, naming and delayed paired associate learning/memory. CT scans of the brain were normal. However, there were subcortical hyperintensities on MRI and left parieto-temporal hypoperfusion on SPECT. Neuropsychological impairment is consistent with the pattern of neuroimage findings. We hypothesize that the pathophysiological mechanisms of pSS involve direct immune attack on neurons in addition to indirect effects through small-vessel angiopathy and thereby induce natural fracture lines in behavior according to location in the central nervous system.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/21. Severe pulmonary hypertension associated with primary sjogren's syndrome.Severe pulmonary hypertension is one of the fetal complications in various connective tissue diseases. We report a case of severe pulmonary hypertension associated with primary sjogren's syndrome. In a lung biopsy specimen, there were findings of intimal and medial hypertrophy with narrowing vessel lumina and plexiform lesions. Moreover, deposits of immunoglobulin m, immunoglobulin a and complement protein C1q were found in the pulmonary arterial walls. Although pulmonary hypertension was refractory to oral prostacyclin, steroid therapy improved the clinical and hemodynamic conditions. In the present case, the immunological etiology may be related to the mechanisms of pulmonary hypertension associated with sjogren's syndrome.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/21. Caring for patients with leg ulcers and an underlying vasculitic condition.It is not uncommon for nurses in the community to encounter patients with leg ulceration combined with rheumatic disease, particularly rheumatoid arthritis (RA). The aetiology of the leg ulcers in these cases is rarely straightforward, and the management of the ulcers is correspondingly complex. Management may be further complicated in the presence of vasculitis, an uncommon disorder in which inflammatory changes cause degradation of blood vessels. Rapid deterioration and pain are the main challenges with these cases. This article discusses the aetiology of vaculitic ulcers, and presents two case studies which were successfully managed using a new hydrogel dressing.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/21. Peripheral neuropathy associated with sjogren's syndrome: pathologic and immunologic study of two patients.Two cases of sjogren's syndrome accompanied by peripheral neuropathy are reported. The level of anti-endothelial cell antibody was increased in both patients. Immunofluorescent deposits of immunoglobulin and C3 component were detected in the vasa nervorum of both cases. The pathological findings showed damage to the endothelial cells in the same vessels. The findings suggest that injury from immune complex and anti-endothelial cell antibody may be the immunological factor in the induction of peripheral neuropathy.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/21. ibuprofen-induced fever in sjogren's syndrome.A 68-year-old woman with a medical history significant for Sjogren syndrome and leukocytoclastic vasculitis of small vessels presented to the emergency department with chills, malaise, a temperature of 39 degrees C, nausea, vomiting, and hypotension. Fifteen minutes earlier she had taken ibuprofen for flu-like symptoms. She was treated with a perfusion of intravenous saline, paracetamol, and ciprofloxacin with improvement 24 hours later. Three months later, she had a similar episode, without hypotension. An oral challenge test with ibuprofen in the hospital produced the same symptoms 3 hours after the last dose. She was treated with metamizole and paracetamol and was asymptomatic the next day. This is the first report of a febrile reaction to ibuprofen in a patient with sjogren's syndrome.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/21. Primary amyloidosis with dry eyes and dry mouth--a case report.We report a rare case of dry eyes and dry mouth caused by primary amyloidosis. A 66-year-old woman with keratoconjunctivitis sicca and xerostomia died of acute respiratory failure. Shirmer's test, gum test, and sialography indicated sjogren's syndrome. lip biopsy revealed amyloid deposition around the salivary ducts. Bence-Jones protein was noted in the urine. At autopsy, amyloid deposition was identified histochemically in many organs, mainly on the vessel walls. Primary amyloidosis should be considered as a differential diagnosis of sjogren's syndrome.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/21. The treatable dementia of sjogren's syndrome.Progressive dementia developed during a 15-month period in a 56-year-old woman with serologically and clinically documented primary sjogren's syndrome. Findings from magnetic resonance imaging and angiography were normal, but a brain biopsy disclosed perivascular lymphocytic inflammation in leptomeningeal and parenchymal vessels. Treatment with high-dose corticosteroids produced rapid and nearly complete resolution of the dementia.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/21. Hypergammaglobulinemic purpura in patients with sjogren's syndrome: a report of nine cases and a review of the Japanese literature.Nine patients with hypergammaglobulinemic purpura associated with primary sjogren's syndrome were presented. All patients were female (mean age 45.6) and showed recurrent purpura on the lower extremities and typical findings of sjogren's syndrome with high gammaglobulin and IgG levels, rheumatoid factors, anti-SSA/SSB antibodies (5/5) and anti-nuclear antibodies (6/9). vasculitis was seen in 6 patients, 4 were of the mononuclear cell type and 2 of the neutrophilic cell type. Six patients had intermediate complexes between 7S and 19S shown by serum ultracentrifugation. Immunofluorescent staining showed immunoglobulin deposition along the blood vessel walls of the skin in these patients. These data suggest that hypergammaglobulinemic purpura in sjogren's syndrome is vasculitis caused by immunologic processes.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
10/21. Annular erythema: a possible association with primary sjogren's syndrome.In four patients with primary sjogren's syndrome a distinct annular erythema developed, which was characterized by a wide, elevated border (which can be likened to a doughnut ring) and central pallor. Histologically, there was a coat sleeve-like infiltration of lymphocytes around blood vessels and nuclear debris in the connective tissue. vasculitis or epidermal changes suggestive of lupus erythematosus were not observed, and there was no immunoglobulin deposition along the basement membrane zone. This annular erythema may be a cutaneous manifestation of sjogren's syndrome.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
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