1/24. Pneumatosis intestinalis in association with connective tissue disease.Pneumatosis intestinalis in association with connective tissue diseases is an unusual combination whose pathogenesis is not yet understood. Furthermore, steroid medication, often used to treat these diseases, may itself cause pneumatosis. Three cases of scleroderma, systemic lupus erythematosus, and amyloidosis in association with pneumatosis and without prior steroid therapy are presented. The small vessel occlusive pathologic processes in these diseases may cause focal areas of mucosal ischemia resulting in small, perhaps transient ulcerations that allow gas to enter the gut wall from the lumen.- - - - - - - - - - ranking = 1keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus (Clic here for more details about this article) |
2/24. The occurrence of various collagen diseases in one family: a sister with ISSc, PBC, APS, and SS and a brother with systemic lupus erythematosus.We encountered siblings who had collagen diseases and related symptoms. Case 1 was a 53-year-old woman who had limited cutaneous systemic sclerosis (ISSc) associated with primary biliary cirrhosis (PBC), antiphospholipid antibody syndrome (APS), and subclinical sjogren's syndrome (SS). Case 2 was a 48-year-old man, her younger brother, with systemic lupus erythematosus (SLE) that developed at 32 years of age. Investigation of their family revealed that their mother had Raynaud's phenomenon, arthritis, and subclinical sjogren's syndrome, and that another younger brother of Cases 1 and 2 had Raynaud's phenomenon and general fatigue. HLA analysis revealed that the sister and brother had some identical hla antigens in common, including A2, A33 (19), B67, B44 (12), Cw7, DR2, DR6, DR52, and DQ1. The sister, brother and their mother had common hla antigens including A2, B67, Cw7, DR2, and DQ1. Although Cases 1 and 2 shared the same HLA system, they presented different phenotypes of collagen disease.- - - - - - - - - - ranking = 5keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus (Clic here for more details about this article) |
3/24. Cardiac valvular disease in chronic inflammatory disorders of connective tissue. Factors influencing survival after surgery.Three cases of cardiac valvular disease complicating chronic inflammatory disorders of connective tissue are described. Each underwent surgical valve replacement. The outcome in the first case, a patient with aortitis complicating Reiter's syndrome and in good health otherwise, has been excellent. The other two patients, one with rheumatoid arthritis and vasculitis, the other with systemic lupus erythematosus and nephritis, died after operation as the result of their systemic disease. The importance of systemic features in assessing this type of patient is emphasized.- - - - - - - - - - ranking = 1keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus (Clic here for more details about this article) |
4/24. Gastrointestinal cytomegalovirus infection in collagen diseases.cytomegalovirus infection of the gastrointestinal tract is a rare serious complication in patients with collagen diseases receiving immunosuppressive agents. We report 3 such cases diagnosed by endoscopy followed by proper treatment. The patients include 38 and 53 years old females with systemic lupus erythematosus. They presented epigastric pain after pulse steroid therapy and combination therapy with steroids and cyclophosphamide, respectively. Their endoscopical findings were multiple small gastric erosions. The other patient was a 60-year-old female with polymyositis who developed rectal bleeding after steroid and imuran therapy. Her endoscopical finding was a discrete, irregular rectal ulcer. The diagnosis of all the patients was confirmed by biopsies of those lesions showing giant cell inclusion bodies and positive staining with anti- cytomegalovirus -antibodies. All patients were treated properly with ganciclovir. We should always keep in mind of a cytomegalovirus infection of the gastrointestinal tract in a patient with collagen disease receiving immunosuppressive agents.- - - - - - - - - - ranking = 1keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus (Clic here for more details about this article) |
5/24. tuberculosis or systemic lupus erythematosus? A diagnostic and therapeutic dilemma.The diagnosis of patients with fever of unknown origin (FUO) is often problematic because the range of possible differential diagnoses is broad. We report on a case in which a patient presented with FUO and was subsequently found to have both a collagen vascular disease and an intercurrent infection. Treatment for the collagen vascular disease with corticosteroids exacerbated the intercurrent infection. The problems in the diagnosis and management of such cases are discussed.- - - - - - - - - - ranking = 4keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus (Clic here for more details about this article) |
6/24. Dystrophic breast calcifications in patients with collagen diseases.We report the mammographic and ultrasonographic findings of characteristic dystrophic breast calcifications in three patients with collagen disease: The diagnoses were scleromyositis, dermatomyositis, and systemic lupus erythematosus. We regard the coarse, bizarre, lacy, and reticular patterns of breast calcifications seen on mammography and located in the subcutaneous fat layer on ultrasonography as characteristic findings for these patients.- - - - - - - - - - ranking = 1keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus (Clic here for more details about this article) |
7/24. Bilateral infrapopliteal artery aneurysms.The authors report the case of a 37-year-old man with bilateral posterior tibial artery aneurysms and concomitant collagen vascular disease. The patient initially presented with pain and swelling of the calves. The diagnosis was made by duplex scan and confirmed with arteriography. Diagnostic studies later verified the presence of a lupus-like syndrome. To date, 10 patients with aneurysms of the infrapopliteal arteries have been reported in the literature; four of these have had associated systemic diseases. These reports are reviewed; their clinical manifestations are discussed, and a treatment plan for these uncommon lesions is presented.- - - - - - - - - - ranking = 0.032457828413465keywords = lupus (Clic here for more details about this article) |
8/24. Acquired perforating dermatosis. Transepidermal elimination of dna material and possible role of leukocytes in pathogenesis.A patient had acquired perforating dermatosis and suffered from renal disease, diabetes mellitus, and lupus vulgaris. Histologic and immunohistochemical studies revealed that the bulk of the coarse granular basophilic material being extruded by transepidermal elimination was of nuclear origin obviously derived from polymorphonuclear leukocytes that were particularly abundant in an early, nonperforated lesion. At the lower boundary of the material being eliminated transepidermally, leukocytes were seen to accumulate, to undergo pyknosis and karyorrhexis, and to transform into nuclear debris. As a minor component, the material contained collagen fibers with altered staining qualities and, in an early lesion, elastic fibers. We speculate that accumulation, disintegration, and enzyme release from polymorphonuclear leukocytes may represent an important, hitherto disregarded driving force in transepidermal elimination. Lysosomal enzymes may later be responsible for the alteration of staining properties in collagen fibers, the degradation of elastic fibers, and for opening up the transepidermal route by impairing intercellular keratinocyte cohesion.- - - - - - - - - - ranking = 0.032457828413465keywords = lupus (Clic here for more details about this article) |
9/24. Effects of prostaglandin E1 on collagen diseases with high levels of circulating immune complexes.Prostaglandin E1 (PGE1) was administered to 4 patients with collagen diseases presenting with high levels of circulating immune complexes (CIC) in sera. Our study patients had progressive systemic sclerosis, systemic lupus erythematosus, polyarteritis nodosa, and rheumatoid arthritis. In all 4 patients, CIC levels significantly decreased after administration of PGE1 by continuous infusion at 10/ng/kg/min via central venous catheter for 72 h. In addition, the skin ulcer in a patient with PSS healed completely, and the finger necrosis in a patient with RA improved. These results suggest that PGE1 given by continuous venous infusion is effective in reducing CIC, in addition to improving peripheral vascular disorders.- - - - - - - - - - ranking = 1keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus (Clic here for more details about this article) |
10/24. argon laser treatment of cutaneous vascular lesions in connective tissue diseases.Two patients with juvenile dermatomyositis, 5 with chronic discoid lupus erythematosus, and one with Rothmund-Thomson's Syndrome were treated for their teleangiectasias of the face with argon laser. The results were highly satisfactory with an almost normal appearance of treated skin in 4 patients. Two patients showed satisfactory results with 60-70% blanching, while 2 patients showed some improvement, but not a completely cosmetically satisfactory result. The most impressive results were in the patients with juvenile dermatomyositis and Rothmund-Thomson's Syndrome. The only side effects observed were a slight scarring and an insignificant pigmentation. No patient displayed any signs of disease activation.- - - - - - - - - - ranking = 0.82500638702648keywords = lupus erythematosus, erythematosus, lupus (Clic here for more details about this article) |
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