1/16. Multifocal choroiditis in patients with familial juvenile systemic granulomatosis.PURPOSE: To document clinical features of uveitis in patients with familial juvenile systemic granulomatosis. DESIGN: Retrospective chart review. methods: Ophthalmologic examination, medical history, and clinical course in 16 patients from eight families examined at six academic medical centers. RESULTS: Of the 16 patients, 15 had evidence of panuveitis with multifocal choroiditis. One patient had only an anterior uveitis. Ischemic optic neuropathy, presumably due to a small vessel vasculopathy, and retinal vasculopathy each occurred in one patient. Ocular complications were common, including cataracts in 11, glaucoma in six, band keratopathy in six, cystoid macular edema in six, and optic disk edema in six. All 16 patients had polyarthritis, and at least nine had skin rash. Often patients were misdiagnosed initially as having either juvenile rheumatoid arthritis or sarcoidosis. CONCLUSIONS: Familial juvenile systemic granulomatosis is an uncommon genetic disease characterized by polyarthritis and uveitis. panuveitis and multifocal choroiditis often may be present. patients with a diagnosis of juvenile rheumatoid arthritis but having a family history of the disease and multifocal choroiditis should be suspected of having familial juvenile systemic granulomatosis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/16. Chronic nondestructive arthritis associated with cutaneous polyarteritis.Two patients with arthritis of the knee joints associated with cutaneous polyarteritis have been followed for 20 and 5 years. The arthritis is characterized by mild to moderate pain and stiffness and inflammatory joint effusions with predominantly polymorphonuclear leukocytes. Despite its chronicity, there has been no clinical or radiologic evidence of joint destruction. Necrotizing inflammation was seen in arteries of the deep skin but not in the small vessels observed in the synovial biopsy specimens.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/16. Early onset sarcoidosis with aortitis--"juvenile systemic granulomatosis?".A syndrome of granulomatous arthritis-uveitis-rash has been described in children. Since the clinical features of arthritis in this syndrome closely resemble those seen in early onset sarcoidosis, and since large vessel vasculitis has been seen in children with sarcoidosis, it is possible that early onset sarcoidosis and granulomatous arthritis-uveitis-rash syndrome are part of the same clinical spectrum. Clinical features of a child with sarcoidosis and takayasu arteritis described in this report support this hypothesis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/16. Jaccoud's arthritis and panvasculitis in the hypocomplementemic urticarial vasculitis syndrome.We describe a woman who developed hypocomplementemic urticarial vasculitis syndrome, and who demonstrated 2 rare features. As well as vasculitis of the small cutaneous vessels, vasculitis affected the femoral, cystic and renal arteries. Her arthritis led to subluxation of the metacarpophalangeal joints, without radiological evidence of rheumatoid-like erosions. This deformity could be passively corrected, and therefore conformed to the pattern described by Jaccoud.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/16. The syndrome of palmar fibromatosis (fasciitis) and polyarthritis.A 45-year-old woman developed simultaneously a form of palmar and digital fibromatosis and an unusual polyarticular disorder with painful capsular contraction. The features of the bilateral shoulder involvement were consistent with the adhesive capsulitis/frozen shoulder syndrome. The other affected joints were painfully contracted and tender but without signs of inflammation in synovial fluid (knee) or associated abnormalities in hematologic status. Histologically, there was extensive fibrosis with increased numbers of fibroblasts, dilated blood vessels and scant perivascular lymphocytic infiltration. The clinical and pathological features were similar to cases described as palmar fasciitis and polyarthritis occurring in association with malignant tumors and with antituberculous chemotherapy. Several months after removal of an endometrial cyst of the ovary and after symptomatic treatment, the palmar fibromatosis and polyarticular disorder had almost completely resolved.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/16. Hypocomplementemic urticarial vasculitis syndrome responsive to dapsone.A 45-year-old woman with cutaneous urticaria-like lesions subsequently developed polyarthritis, glomerulonephritis, and chronic obstructive pulmonary disease. biopsy of skin showed leukocytoclastic vasculitis of superficial capillaries. biopsy of the kidney revealed mesangioproliferative glomerulonephritis, and lung biopsy revealed severe emphysema and thick-walled blood vessels with immunoglobulin deposition. Therapy with dapsone produced dramatic improvement of the patient's cutaneous vasculitis and arthritis. This case uniquely demonstrates the efficacy of dapsone for both the urticarial vasculitic and the arthritic components of this syndrome and reemphasizes the potentially severe pulmonary and renal complications.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/16. aortitis and large vessel arteritis in a newborn.Disseminated arteritis with extensive involvement of the aorta, as well as involvement of the arch vessels, coronary arteries, and pulmonary arteries, but not of the arteries within the liver, spleen, kidneys, or other organs, is unusual in a newborn. The presence of both acute and chronic inflammation with fibrosis, as well as calcification and focal ossification in the aorta, would suggest that the process had been present for some time before birth. This lesion should be described rather than designated by an eponym or included with such entities as Takayasu's disease. An additional finding of interest was widespread calcification in Bowman's capsules of numerous glomeruli.- - - - - - - - - - ranking = 5keywords = vessel (Clic here for more details about this article) |
8/16. Bowel bypass arthritis dermatitis syndrome: a histological and electron microscopical study.A case of dermal vasculitis with arthralgia after intestinal bypass surgery is reported. A 36-year-old woman developed arthralgia and skin rash, 1 year after an ileo-jejunal bypass operation was performed for overweight (130 kg). skin biopsy showed leucocytoclastic vasculitis in the dermis. E.M. study showed clumps of platelets around small dermis blood vessels, and polymorphous perivascular infiltration. The symptoms subsided after tetracycline treatment.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/16. Pachydermoperiostosis: studies on the synovium.Pachydermoperiostosis (primary hypertrophic osteoarthropathy) is a familial disorder characterized by clubbing, periosteal bone changes, and furrowing of the skin. A 40 year old male with this syndrome and chronic arthritis was recently studied. The synovial fluid was non-inflammatory. Histologically the synovium exhibited hyperplasia and microvascular alterations. Immunofluorescent staining failed to identify deposits of immunoglobulin. Electron microscopy revealed multilayered basement laminae around small subsynovial blood vessels but no electron-dense deposits as described in studies of hypertrophic pulmonary osteoarthropathy. The pathogenesis is obscure and appears to be non-immunological.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
10/16. Pigmented villonodular synovitis associated with psoriatic polyarthropathy: and electron microscopic and immunocytochemical study.A case of pigmented villonodular synovitis in a patient with psoriatic polyarthropathy was studied by means of light microscopy, electron microscopy and immunocytochemical techniques. The lesion consisted of mononuclear phagocytes with features intermediate between type A and type B cells, two types of multinucleate giant cells, an abnormal vasculature, extravasated fibrin and erythrocytes. An unexpected feature was the presence of a mononuclear cell whose cytoplasm contained intermediate filaments and reacted strongly to antisera to cytokeratins. Such a cell type has not previously been described in this condition and its significance is unclear. The findings support the theory that the pathogenesis of PVNS involves leakage of blood through abnormal vessels resulting in the local accumulation and proliferation of mononuclear phagocytes, connective tissue cells and unidentified cytokeratin positive mononuclear cells. The aetiology of the vascular damage, however, remains unknown.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
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