1/75. Interstitial granulomatous dermatitis with plaques.We report on the clinical and histopathologic findings of four patients who had asymptomatic, erythematous to violaceous plaques symmetrically distributed on the upper aspect of the thighs, lateral chest, and in two cases also on the abdomen and flexor surface of the elbows. All of the patients were women; two of them had arthritis, which in one case was associated with an autoimmune disorder, and another had autoimmune thyroiditis. Histopathologically, all cases showed similar changes consisting of an interstitial granulomatous dermatitis involving mostly the lower reticular dermis. histiocytes were the predominant cellular component, arranged interstitially and in small palisades around foci of degenerated collagen bundles in concert with large numbers of neutrophils and eosinophils. Interstitial granulomatous dermatitis can present different clinical expressions, including linear cords, papules, and, as in our cases, plaques. This peculiar histopathologic pattern falls into the spectrum of cutaneous extravascular necrotizing granuloma, a condition that is often associated with systemic autoimmune disease.- - - - - - - - - - ranking = 1keywords = interstitial (Clic here for more details about this article) |
2/75. Thoracoabdominal aortic aneurysm in Cogan's syndrome.Cogan's syndrome is an autoimmune disease of unknown etiology, clinically manifested as non-syphilitic interstitial keratitis and audiovestibular symptoms. Increasing evidence suggests that Cogan's may be a systemic vasculitis. In this report, we review the vascular manifestations of Cogan's syndrome and report two cases of thoracoabdominal aortic aneurysm in patients with this disorder.- - - - - - - - - - ranking = 0.5keywords = interstitial (Clic here for more details about this article) |
3/75. Effect of open label pulse cyclophosphamide therapy on MRI measures of disease activity in five patients with refractory relapsing-remitting multiple sclerosis.OBJECTIVE: To evaluate the response to cyclophosphamide (CTX) of five patients who failed an average three treatments with multiple other therapeutic agents, using serial monthly MRI measures. methods: Five patients with relapsing-remitting multiple sclerosis (MS) and documented MRI disease activity were started on monthly pulse intravenous CTX at a dose of 1 g/m2. CTX was administered without an induction phase according to the protocol similar to the treatment of lupus nephritis. The five patients were followed with monthly MRI and clinical evaluation for a mean of 28 months. RESULTS: All the patients showed a rapid reduction in the contrast-enhancing lesion frequency and in three patients there was a decrease in the T2 lesion load within the first 5 months after starting CTX treatment. The administration of CTX during overnight hospitalization was safe and well tolerated. CONCLUSIONS: These findings suggest that aggressive immunosuppressive therapy may be useful in some rapidly deteriorating refractory patients and further controlled study should be considered in order to full evaluate this type of treatment as a potential therapy in MS.- - - - - - - - - - ranking = 8.9569159028547keywords = nephritis (Clic here for more details about this article) |
4/75. Hypocomplementemic urticarial vasculitis or systemic lupus erythematosus?The 2 patients presented here showed the typical signs of hypocomplementemic urticarial vasculitis syndrome (HUVS). During follow-up, there was an inverse correlation between anti-C1q autoantibody titer and C1q antigen concentration in serum in both patients over a period of 2 years. The first patient had nephritis characterized by immune deposits in glomeruli and around the tubules. The histological findings, C1q deposits, and presence of tubuloreticular inclusions in capillary endothelial cells suggested a disease process identical to systemic lupus erythematosus (SLE). The second patient, after a lag phase of 2 years, fulfilled a fourth American College of rheumatology criteria for SLE when she developed anti-double-stranded dna antibodies. HUVS and SLE overlap, and the criteria for identifying HUVS as an entity distinct from SLE are lacking.- - - - - - - - - - ranking = 8.9569159028547keywords = nephritis (Clic here for more details about this article) |
5/75. Cogan's syndrome: an oculo-audiovestibular disease.Typical Cogan's syndrome is a rare disease of young adults consisting of flares of interstitial keratitis and sudden onset of Meniere-like attacks (nausea, vomiting, tinnitus, vertigo and hearing loss). life-threatening aortic insufficiency develops in 10% of reported cases. Atypical Cogan's syndrome (audiovestibular dysfunction with other types of inflammatory eye disease) is associated with vasculitis in 20% of cases and has a less favourable prognosis than typical Cogan's syndrome.- - - - - - - - - - ranking = 0.5keywords = interstitial (Clic here for more details about this article) |
6/75. Coexistent lymphoid interstitial pneumonia, pernicious anemia, and agammaglobulinemia.immunologic factors have been incriminated in the pathogenesis of lymphoid interstitial pneumonia. The discovery of a patient with coexistent lymphoid interestitial pneumonia, pernicious anemia, and common variable hypogammaglobulinemia focused attention on the possible autoimmune nature of this pulmonary disease. Extensive immunologic studies demonstrated a noticeably impaired bonemarrow-dependent (B cell) system and intact thymus-dependent (T cell) system. No evidence of humoral or cellular hypersensitivity to homologous lung determinants was found.- - - - - - - - - - ranking = 2.5keywords = interstitial (Clic here for more details about this article) |
7/75. Graves' disease and low-output cardiac dysfunction: implications for autoimmune disease in endomyocardial biopsy tissue from eleven patients.Classic high-output thyrotoxic heart disease is generally considered a direct effect of thyroid hormone. In contrast, the cause of the less common low-output heart failure is generally unknown. The aim of this study was to retrospectively evaluate available endomyocardial biopsy tissue from patients with coexistent Graves' disease and idiopathic low-output heart failure and determine whether the biopsy features were consistent with an autoimmune process. The study group consisted of 11 patients whose mean age was 47 years when they were diagnosed with hyperthyroidism and 52 years when diagnosed with cardiac dysfunction. Right ventricular endomyocardial biopsy tissue revealed severe lymphocytic myocarditis in a patient with severe ophthalmopathy and showed borderline myocarditis in a patient without ophthalmopathy. biopsy tissues from 6 other patients showed appreciable myocyte hypertrophy and interstitial fibrosis, consistent with dilated cardiomyopathy. Two patients had nondiagnostic biopsy specimens, and 1 patient had features suggestive of arrhythmogenic right ventricular dysplasia. In conclusion, for the 11 patients with Graves' disease and unexplained systolic dysfunction, only 2 (18%) had lymphocytic infiltrates consistent with an autoimmune process. Thus, among patients with Graves' disease, most cases of low-output cardiac dysfunction appear to be due to causes other than an active autoimmune inflammatory process.- - - - - - - - - - ranking = 0.5keywords = interstitial (Clic here for more details about this article) |
8/75. ANCA-associated necrotizing/granulomatous glomerulonephritis in an elderly patient; importance of the renal biopsy.ANCA-associated glomerulonephritis may present initially with renal symptoms, especially in elderly patients. We report a case of ANCA-associated glomerulonephritis in an 80-year-old female presenting with renal insufficiency and proteinuria. There was no evidence of systemic illness at admission. The major finding in the renal biopsy was the presence of granulomas. Additional testing for anti-neutrophil cytoplasmic antibodies (ANCA) was suggested. The ANCA test was positive confirming the diagnosis of ANCA-associated necrotizing/granulomatous glomerulonephritis. The patient responded well to adequate treatment with immunosuppressive therapy. This case demonstrates the importance of the renal biopsy in cases of ANCA-associated glomerulonephritis presenting with renal symptoms.- - - - - - - - - - ranking = 71.655327222837keywords = nephritis (Clic here for more details about this article) |
9/75. Interstitial granulomatous dermatitis with cutaneous cords and arthritis: a disorder associated with autoantibodies.Interstitial granulomatous dermatitis with arthritis is a new entity characterized by linear or arciform subcutaneous cords. We describe a patient presenting with typical cutaneous lesions extending from the anterior and posterior part of the axillae to the flank. The lesions had been present for 6 years with flares and remission. Histopathologic examination revealed a bandlike infiltrate of histiocytes in the reticular dermis with focal areas of palisading around necrotic collagen bundles. A few atypical histiocytes were also present. As previously described, it was associated with serologic findings of an autoimmune disease, especially high titers of anti-dna antibodies. The possible inclusion of the interstitial granulomatous dermatitis in the continuous spectrum of the palisaded neutrophilic and granulomatous dermatitis of immune complex disease is also discussed.- - - - - - - - - - ranking = 0.5keywords = interstitial (Clic here for more details about this article) |
10/75. Cogan's syndrome: organ-specific autoimmune disease or systemic vasculitis? A report of two cases and review of the literature.Cogan's syndrome is a rare disorder of unknown origin characterized by inflammatory eye disease and vestibuloauditory symptoms. Typically, young adults suffer from interstitial keratitis and sudden onset of tinnitus and hearing loss. Few cases (around 150) have been published and thus it is difficult to determine the percentage of patients with underlying systemic disorders such as systemic vasculitis. The variety of systemic manifestations is large and includes fever, splenomegaly, lymphadenopath, and musculoskeletal complaints. systemic vasculitis can be seen in around 10% of cases and may involve the large vessels, appearing as Takayasu-like vasculitis with affection of the aortic valve but also the coronary arteries and the small kidney vasculature. Evaluating the exact extension of the systemic features determines the choice of treatment. While corticosteroids have proved to be of short-term benefit, long-term treatment with immunosuppressive drugs is controversial. Auditory function in deaf patients has often been restored successfully with cochlear implants. To illustrate the nature of the syndrome, we present two patients with a wide clinical spectrum of symptoms from local disease restricted to the eyes and ears to a widespread vasculitis affecting arteries of the brain, kidney and the upper and lower extremities. We then review the typical aspects as well as the etiology of the disease.- - - - - - - - - - ranking = 0.5keywords = interstitial (Clic here for more details about this article) |
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