Letter To The Editor [1] [cartas Ao Editor]

[No abstract available]38639

Evans Syndrome And Systemic Lupus Erythematosus: Clinical Presentation And Evolution [syndrome D'evans Et Lupus érythémateux Systémique: Présentation Clinique Et évolution]

[No abstract available]793241244Evans, R.S., Takahashi, K., Duane, R.T., Primary thrombocytopenic purpura and acquired haemolytic anemia: Evidence for a common etiology (1951) AMA Arch Intern Med, 87, pp. 48-65Kittaka, K., Dobashi, H., Baba, N., A case of Evans syndrome combined with systemic lupus erythematosus successfully treated with rituximab (2008) Scand J Rheumatol, 37, pp. 390-393Savasan, S., Warrier, I., Ravindranath, Y., The spectrum of Evans' syndrome (1997) Archives of Disease in Childhood, 77 (3), pp. 245-248Dhingra, K.K., Jain, D., Mandal, S., Evans syndrome: A study of six cases with review of literature (2008) Hematology, 13, pp. 356-360Sultan, S.M., Begum, S., Isenberg, D.A., Prevalence, patterns of disease and outcome in patients with systemic lupus erythematosus who develop severe haematological problems (2003) Rheumatology, 42 (2), pp. 230-234. , DOI 10.1093/rheumatology/keg069Domiciano, D.S., Shinjo, S.K., Autoimmune hemolytic anemia in systemic lupus erythematosus: Association with thrombocytopenia (2010) Clin Rheumatol, 29, pp. 1427-1431Tan, E.M., Cohen, A.S., Fries, J.F., The 1982 revised criteria for the classification of systemic lupus erythrematosus (1982) Arthritis and Rheumatism, 25 (11), pp. 1271-1277. , DOI 10.1002/art.1780251101The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes (1999) Arthritis Rheum, 42, pp. 599-608. , Acr Ad Hoc Committee On Neuropsychiatric LupusPirofsky, B., Clinical aspects of autoimmune hemolytic anemia (1976) Semin Hematol, 13, pp. 251-265Silverstein, M.N., Heck, F.J., Acquired hemolytic anemia and associated thrombocytopenic purpura: With special reference to Evans' syndrome (1962) Mayo Clin Proc, 37, pp. 122-128Karpatkin, S., Autoimmune thrombocytopenic purpura (1980) Blood, 56 (3), pp. 329-343Alger, M., Alarcon-Segovia, D., Rivero, S.J., Hemolytic anemia and thrombocytopenic purpura: Two related subsets of systemic lupus erythematosus (1977) J Rheumatol, 4, pp. 351-357Kameda, T., Dobashi, H., Kittaka, K., Susaki, K., Yamaoka, G., Arai, K., Tokuda, M., Ishida, T., Two cases of refractory thrombotic thrombocytopenic purpura associated with collagen vascular disease were significantly improved by rituximab treatment (2007) Clinical Rheumatology, 26 (12), pp. 2159-2162. , DOI 10.1007/s10067-007-0631-0Kokori, S.I.G., Ioannidis, J.P.A., Voulgarelis, M., Tzioufas, A.G., Moutsopoulos, H.M., Autoimmune hemolytic anemia in patients with systemic lupus erythematosus (2000) American Journal of Medicine, 108 (3), pp. 198-204. , DOI 10.1016/S0002-9343(99)00413-1, PII S0002934399004131Nossent, J.C., Swaak, A.J.G., Prevalence and significance of haematological abnormalities in patients with systemic lupus erythematosus (1991) Q J Med, 80, pp. 605-612Deleze, M., Alarcon-Segovia, D., Oria, C.V., Sanchez-Guerrero, J., Fernandez-Dominguez, L., Gomez-Pacheco, L., Ponce De Leon, S., Hemocytopenia in systemic lupus erythematosus. Relationship to antiphospholipid antibodies (1989) Journal of Rheumatology, 16 (7), pp. 926-930Alger, M., Alarcon-Segovia, Rivero, S.J., Hemolytic anemia and thrombocytopenic purpura: Two related subsets of systemic lupus erythematosus (1977) J Rheumatol, 4, pp. 351-357Rabinowitz, Y., Dameshk, W., Systemic lupus erythematosus after idiopathic thrombocytopenic purpura. A review (1960) Ann Intern Med, 52, pp. 1-28Durán, S., Apte, M., Alarcón, G.S., Features associated with, and the impact of, hemolytic anemia in patients with systemic lupus erythematosus: LX, results from a multiethnic cohort (2008) Arthritis Rheum, 59, pp. 1332-1340Pui, C.H., Wilimas, J., Wang, W., Evans syndrome in childhood (2006) Eur J Haematol, 76, pp. 526-530Mathew, P., Chen, G., Wang, W., Evans syndrome: Results of a national survey (1997) Journal of Pediatric Hematology/Oncology, 19 (5), pp. 433-437. , DOI 10.1097/00043426-199709000-00005Michel, M., Chanet, V., Dechartres, A., The spectrum of Evans syndrome in adults: New insight into the disease based on the analysis of 68 cases (2009) Blood, 114, pp. 3167-3172Sachetto, Z., Fernandes, S.R., Del Rio, A.P., Systemic Lupus Erythematosus associated with vasculitic syndrome (Takayasu's arteritis) (2009) Rheumatol Int, , doi:101007/s00296-009-1133-yNg, S.C., Evans syndrome: A report on 12 patients (1992) Clin Lab Hematol, 14, pp. 189-193Cambridge, G., Leandro, M.J., Teodorescu, M., Manson, J., Rahman, A., Isenberg, D.A., Edwards, J.C., B cell depletion therapy in systemic lupus erythematosus: Effect on autoantibody and antimicrobial antibody profiles (2006) Arthritis and Rheumatism, 54 (11), pp. 3612-3622. , DOI 10.1002/art.22211Norton, A., Roberts, I., Management of Evans syndrome (2006) British Journal of Haematology, 132 (2), pp. 125-137. , DOI 10.1111/j.1365-2141.2005.05809.

Juvenile Systemic Lupus Erythematosus: Clinical Manifestations, Laboratory Data, And Evolution In 59 Patients [lupus Eritematoso Sistemico Juvenil: Manifestacoes Clinicas, Laboratoriais E Evolutivas Em 59 Pacientes]

The aim of this study was to analyze clinical manifestations, laboratory data and mortality causes among 59 patients (48F/11M) with systemic lupus erythematosus (SLE) whose symptoms initiated by the age of 16. To this aim, the authors made a retrospective study of the medical records of patients seen at the Hospital das Clinicas da Universidade Estadual de Campinas (HC) between 1979 and 1995. Patients presenting discoid, drug-induced or neonatal lupus were not included. Patients were analyzed individually and in three groups, depending on the age at the beginning of first signs of the disease. All patients had four or more American College of Rheumatology (ACR) criteria for SLE. More frequent clinical manifestations were articular (91.5%), renal (71.1%), malar erythema (61%), alopecia (61%), fever (59.3%), and photosensitivity (52.5%). Laboratory results showed: Antinuclear antibodies (ANA) (94.9%), LE cells (71.1%), decrease of serum complement level (65.3%), anti-DNA (63.4%), hematuria (62.7%), and proteinuria (61%). The mortality rate was 23.7% (9F/5M). Death causes were infectious processes in eight patients (57.1%), central nervous system (CNS) disease in five (35.7%), and renal failure in one (7.2%). Clinical manifestations initiating before age 14 and the presence of nephropathy were bad prognosis factors for SLE.39525225

Bone Mineral Density In Patients With Systemic Lupus Erythematosus And Its Relation To Estrogen Levels [densidade Mineral Ossea Em Pacientes Com Lupus Eritematoso Sistemico E Sua Relacao Com Niveis Estrogenicos]

Objectives: 1) To evaluate bone mineral density (BMD) in patients with systemic lupus erythematosus (SLE); 2) to determine the role of corticosteroids and cytotoxic drugs; 3) to assess the effect of estrogen on BMD in SLE. Patients and methods: BMD (DEXA) at lumbar vertebrae (L2-L4) and at femoral neck in 60 pre-menopausal SLE patients and in 64 controls, and concentrations of estradiol were measured. Age, age at disease onset, body mass index (BMI), time of disease, disease activity (SLEDAI), prednisone dose at the evaluation, total cumulative and cumulative prednisone dose in the last year and cytotoxic drugs were also assessed. Results: Mean plasmatic estradiol was 175.98 pg/ml in patients and 149.9 in controls. BMD was lower in patients than in controls (p < 0.0001). The mean current, cumulative, and last year prednisone doses were respectively 19.17 mg/d, 28. 78 g, and 5.33 g. There was no association between corticosteroids or the cytotoxic drug used and bone loss (BL). The serum concentration of estradiol did not influence the bone mass loss. The body mass index and age at the disease onset showed influence on BMD at L2. Conclusion: BMD was significantly lower in SLE patients but not related to CE or to other drugs; the estradiol had no effect on BMD. Low bone mass index interacting with early onset of disease might influence the probability of loss of bone mass.404175182Bresnihan, B., Outcome and survival in systemic lupus erythematosus (1989) Ann Rheum Dis, 48, pp. 443-445Mills, J.A., Systemic lupus erythematosus (1994) N Engl J Med, 330, pp. 1871-1879Dykman, T.R., Gluck, O.S., Murphy, W.A., Hahn, B.H., Evaluation of factors associated with glucocorticoid-induced osteopenia in patients with rheumatic diseases (1985) Arthritis Rheum, 28, pp. 361-368Ramsey-Goldman, R., Schilling, E., Daugherty, C., Conte, C., Rairie, J., Manzi, S., Fractures in patients with lupus (1996) Arthritis Rheum, 39 (SUPPL. 9), pp. S89Dhillon, V.B., Davies, M.C., Hall, M.L., Assessment of the effect of oral corticosteroids on bone mineral density in systemic lupus erythematosus: A preliminary study with dual energy x-ray absorptiometry (1990) Ann Rheum Dis, 49, pp. 624-626Kalla, A.A., Van Wyk Kotze, T.J., Meyers, O.L., Metacarpal bone mass in systemic lupus erythematosus (1992) Clin Rheumatol, 11, pp. 475-482Kalla, A.A., Fataar, A.A., Jessop, S.J., Bewerunge, L., Loss of trabecular bone mineral density in systemic lupus erythematosus (1993) Arthritis Rheum, 36, pp. 1726-1734Formiga, F., Moga, I., Nolla, J.M., Pac, M., Mitjavila, F., Roig-Escofet, D., Loss of bone mineral density in premenopausal women with systemic lupus erythematosus (1995) Ann Rheum Dis, 54, pp. 274-276Houssiau, F.A., Lefebvre, C., Depresseaux, G., Lambert, M., Devogelaer, J.P., Nagant-De Deuxchans, C., Trabecular and cortical bone loss in systemic lupus erythematosus (SLE) (1996) Br J Rheumatol, 35, pp. 244-247Horslev-Petersen, K., Influence of disease activity and drug treatment on bone mineral density (BMD) in patients with systemic lupus erythematosus (1995) Rheumatology in Europe, 24 (SUPPL. 3), p. 11Sels, F., Bone density in systemic lupus erythematosus (1995) Rheumatology in Europe, 24 (SUPPL. 3), p. 353Sels, F., Dequeker, J., Verwilghen, J., Mbuyi-Muamba, J.M., SLE and osteoporosis: Dependence and/or independence on glucocorticoids (1996) Lupus, 5, pp. 89-92Sels, F., Dequeker, J., Verwilghen, J., Mbuyi-Muamba, J.M., SLE and osteoporosis: Dependence and/or independence on glucocorticoids (1996) Lupus, 5, pp. 89-92Rosin, M., Yarboro, C., Nuzzo, V., Klippel, J., Gourley, M., Corticosteroid (CS) effects on mineral density (BMD) in patients with systemic lupus erythematosus (SLE) (1996) Arthritis Rheum, 39 (SUPPL. 9), pp. S293Hearth-Holmes, M., Nandy, I., Elder, C., Wolf, R., Bone mineral density (BMD) in two groups of patients with systemic lupus erythematosus (1996) Arthritis Rheum, 39 (SUPPL. 9), pp. S292Petri, M., Osteoporosis in SLE: Prednisone affects lumbar spine more than other areas (1996) Arthritis Rheum, 39 (SUPPL. 9), pp. S138Tan, E.M., Systemic lupus erythematosus: Immunologic aspects (1985), pp. 1049-1054. , McCarty DJ (ed): Arthritis and Allied Conditions, Philadelphia, Lea and FebigerBombardier, C., Gladman, D.D., Urowitz, M.B., Caron, D., Chang, C.H., The committee on prognosis studies in SLE. Derivation of the SLEDAI: A disease activity index for lupus patients (1992) Arthritis Rheum, 35, pp. 630-640Cullun, I.D., Ell, P.J., Ryder, J.P., X-ray dual-photon absorptiometry: A new method for the measurement of bone density (1989) Br J Radiol, 62, pp. 587-592(1994), World Health Organization (WHO). Assessment of Fracture Risk and its Application to Screening for Postmenopausal Osteoporosis, Geneva, WHO (Reports series, 843), 130pMagaro, M., Tricerri, A., Piane, D., Generalized osteoporosis in non-steroid treated rheumatoid arthritis (1991) Rheumatol Int, 11, pp. 73-76Kroger, H., Honkanen, R., Saarikoski, A., Alhava, E., Decreased axial bone mineral density in perimenopausal women with rheumatoid arthritis: A population based study (1994) Ann Rheum Dis, 53, pp. 18-23Gough, A.K.S., Lilley, J., Eyre, S., Holder, R.L., Emery, P., Generalized bone loss in patients with early rheumatoid arthritis (1994) Lancet, 344, pp. 23-27Laan, R.F.J.M., Van Riel, P.L.C.M., Van Erning, L.J.T.H.O., Lemmens, J.A.M., Ruijs, S.H.J., Van de Putte, L.B.A., Vertebral osteoporosis in rheumatoid arthritis patients: Effect of low dose prednisone therapy (1992) Br J Rheumatol, 31, pp. 91-96Joffe, I., Epstein, S., Osteoporosis associated with rheumatoid arthritis: Pathogenesis and management (1991) Semin Arthritis Rheum, 20, pp. 256-272Kipen, Y., Buchbinder, R., Forbes, A., Strauss, B., Littlejohn, G., Morand, E., Prevalence of reduced bone mineral density in systemic lupus erythematosus and the role of steroids (1997) J Rheumatol, 24, pp. 1922-1929Liel, Y., Edward, J., Shary, J., Spicer, K.M., Gordon, L., Bell, N.H., The effects of race and body habitus on bone mineral density of the radius, hip and spine in premenopausal women (1988) J Clin Endocrinol Metab, 66, pp. 1247-1250Katzman, D.K., Bachrachl, K., Carter, D.R., Marcus, R., Clinical anthropometric correlates of bone mineral acquisition in healthy adolescent girls (1991) J Clin Endocrinol Metab, 73, pp. 1332-1339Heiss, C.J., Sanborn, C.F., Nichols, D.L., Bonnick, S.L., Alford, B.B., Association of body fat distribution, circulating sex hormones and bone density in postmenopausal women (1995) J Clin Endocrinol Metab, 80, pp. 1591-1596Formiga, F., Nolla, J.M., Mitjavila, F., Bonnin, R., Navarro, M.A., Moga, I., Bone mineral density and hormonal status in men with systemic lupus erythematosus (1996) Lupus, 5, pp. 623-626LoCascio, V., Bone loss in response to long-term glucocorticoid therapy (1990) Bone Miner, 8, pp. 39-51Adachi, J.D., Bensen, W.G., Hodsman, A.B., Corticosteroid-induced osteoporosis (1993) Semin Arthritis Rheum, 22, pp. 375-384Laan, R.F.J.M., Van Riel, P.L.C.M., Van Erning, L.J.T.H.O., Lemmens, J.A.M., Ruijs, S.H.J., Van de Putte, L.B.A., Low-dose prednisone induces rapid reversible axial bone loss in rheumatoid arthritis patients (1993) Ann Intern Med, 119, pp. 963-968Pacifici, R., Brown, C., Puscheck, E., Effect of surgical menopause and estrogen replacement on cytokine release from human blood mononuclear cells (1991) Proc Natl Acad Sci U S A, 88, pp. 5134-5138Lahita, R.G., Sex steroids and SLE: Metabolism of androgens and estrogens [Editorial] (1992) Lupus, 1, pp. 125-12

Bone Marrow Morphology In Systemic Lupus Erythematosus [morphologie Des Knochenmarks Bei Systematischen Lupus Erythematodes]

Autoimmune-mediated cell destruction in peripheral blood has been considered the most frequent cause of cytopenias seen in systemic lupus erythematosus. On the other hand, several bone marrow abnormalities have been documented by case reports as well as retrospective studies. In the present prospective analysis we addressed the question of the frequency and features of bone marrow abnormalities in SLE patients presenting peripheral blood cytopenias. In 12 of the 21 patients examined, we found foci of an exudative necrotic reaction characterized by edema, necrotic cells (nucleophagocytosis) and an infiltrate by lymphocytes, plasma cells and macrophages. Reticulin fibers were increased in these foci in 8 cases. Surprisingly, hemopoiesis was hyperplastic in only 6 patients, but was grossly diminished in 12 cases. In 14 patients atypical elements of one or more cell lines could be observed. Most commonly (11 cases), an increase in megakaryocytes with atypical elements showing small pleomorphic nuclei could be seen, together with normal nuclei. Cell atypia in the erythroid line was found in 8 cases, and abnormal granulocytic precursors were seen in 3 cases. No ring sideroblasts could be observed in any case. All these features provide persuasive evidence that the bone marrow is a common target organ affected by autoaggression in SLE. Central and peripheral mechanisms acting to produce peripheral cytopenias must be assumed in most cases.15529229

Ovarian Failure In Sle Patients Using Pulse Cyclophosphamide: Comparison Of Different Regimes

The objective of this study was to determine the frequency and risk factors of early ovarian failure in systemic lupus erythematosus (SLE) women treated with cyclophosphamide (CY). We further tried to determine if there was a reduction of ovarian failure in recent years, due to reduction in the CY dose. We reviewed the charts of all women below 40 years of age who received intravenous CY pulse therapy. In order to be included, the patients must have finished CY treatment before completing 40 years. Patients were divided into two groups: Group A (57 patients), patients who were treated with 0.75 mg/body surface; Group B (50 patients), patients treated with 0.5 mg/body surface. Fifty patients with similar age distribution who never received CY were selected from the database as a control group (Group C). The Chi-square test was applied to compare the categorical variables of the groups and whenever needed, the Fisher's Exact test was used. We observed similar age distribution and disease duration at disease onset between groups. Also, no differences regarding the age at menarche, total prednisone dose, and SLICC-ACR/DI scores were observed at disease onset between the three groups. In group A, ten (17.5%) patients refereed sustained amenorrhea, independently associated with treatment duration (P = 0.001), total intravenous cyclophosphamide (IV-CF) dose (P = 0.02), older age at disease onset (P = 0.04). Seven (12.3%) patients referred transient amenorrhea. Transient amenorrhea was related to CY treatment duration (P = 0.017). In group B, no patient reported sustained amenorrhea and 10 of 50 (20%) patients referred transient amenorrhea, related to CY treatment duration (P = 0.017). The most important risk factors for menstrual abnormalities were duration of treatment and cumulative dose of CY. Lower CY dose reduced the number of premature ovarian failures significantly in this cohort. © 2007 Springer-Verlag.286567571Cervera, R., Khamashtra, M.A., Font, J., Systemic lupus erythematosus: Clinical and immunological patterns of disease expression in a cohort of 1,000 patients (1993) Medicine, 72, pp. 113-124Balow, J.E., Austin, H.A., Tsokos, G.C., Antonovych, T.T., Steinberg, A.D., Klippel, J.H., NIH conference: Lupus nephritis (1987) Ann Intern Med, 106, pp. 79-94Neuwelt, C.M., Lacks, S., Kaye, B.R., Ellman, J.B., Borenstein, D.G., Role of intravenous cyclophosphamide in the treatment of severe neuropsychiatric systemic lupus erythematosus (1995) Am J Med, 98, pp. 32-41Boumpas, D.T., Austin, I.I.I.H.A., Vaughn, E.M., Klippel, J.H., Steinberg, A.D., Yarboro, C.H., Balow, J.E., Controlled trial of pulse methylprednisolone versus two regimens of pulse cyclophosphamide in severe lupus nephritis (1992) Lancet, 340, pp. 741-745Boumpas, D.T., Barez, S., Klippel, J.H., Balow, J.E., Intermittent cyclophosphamide for the treatment of autoimmune thrombocytopenia in systemic lupus erythematosus (1990) Ann Intern Med, 112, pp. 674-677Fukuda, M., Kamiyama, Y., Kawahara, K., Kawamura, K., Mori, T., Honda, M., The favorable effect of cyclophosphamide pulse therapy in the treatment of massive pulmonary hemorrhage in systemic lupus erythematosus (1994) J Pediatr, 153, pp. 167-170Schroeder, J.O., Euler, H.H., Loffler, H., Synchronization of plasmapheresis and pulse cyclophosphamide in severe systemic lupus erythematosus (1987) Ann Intern Med, 107, pp. 344-346Valeri, A., Radhakrishnan, J., Estes, D., D'Agati, V., Kopelman, R., Pernis, A., Flis, R., Appel, G.B., Intravenous pulse cyclophosphamide treatment of severe lupus nephritis: A prospective five-year study (1994) Clin Nephrol, 42, pp. 71-78Austin I.I.I.H.A.́, Klippel, J.H., Balow, J.E., Le Riche, N.G.H., Steinberg, A.D., Plotz, P.H., Decker, J.L., Therapy of lupus nephritis - Controlled trial of prednisone and cytotoxic drugs (1986) N Engl J Med, 314, pp. 614-619Houssiau, F.A., D'Cruz, D.P., Haga, H.J., Hughes, G.R.V., Short course of weekly low-dose intravenous pulse cyclophosphamide in the treatment of lupus nephritis: A preliminary study (1991) Lupus, 1, pp. 31-35Kumar, R., Biggart, J.D., McEvoy, J., McGeown, M.C., Cyclophosphamide and reproductive function (1972) Lancet, 1, pp. 1212-1214Martin, F., Lauwerys, B., Lefebvre, C., Devogelaer, J.P., Houssiau, F.A., Side-effects of intravenous cyclophosphamide pulse therapy (1997) Lupus, 6, pp. 254-257Mok, C.C., Lau, C.S., Wong, R.W.S., Risk factors for ovarian failure in patients with systemic lupus erythematosus receiving cyclophosphamide therapy (1998) Arthritis Rheum, 41, pp. 831-837McDermott, E.M., Powell, R.J., Incidence of ovarian failure in systemic lupus erythematosus after treatment with pulse cyclophosphamide (1996) Ann Rheum Dis, 55, pp. 224-229Langevitz, P., Klein, L., Pras, M., Many, A., The effect of cyclophosphamide pulses on fertility in patients with lupus nephritis (1992) Am J Reprod Immunol, 28, pp. 157-158Huong, D.L., Amoura, Z., Duhaut, P., Sbai, A., Costedoat, N., Wechsler, B., Piette, J.C., Risk of ovarian failure and fertility after intravenous cyclophosphamide. a study in 84 patients (2002) J Rheumatol, 29, pp. 2571-2576Warne, G.L., Fairley, K.F., Hobbs, J.B., Martin, F.I.R., Cyclophosphamide induced ovarian failure (1973) N Engl J Med, 289, pp. 1159-1162Uldall, P.R., Kerr, D.N.S., Tacchi, D., Sterility in cyclophosphamide (1972) Lancet, 1, pp. 693-694Wang, C.L., Wang, F., Bosco, J.J., Ovarian failure in oral cyclophosphamide treatment for systemic lupus erythematosus (1995) Lupus, 4, pp. 11-14Boumpas, D.T., Austin H.A.́, Vaughn, E.M., Yarboro, C.H., Klippel, J.H., Balow, J.E., Risk for sustained amenorrhea in patients with systemic lupus erythematosus receiving intermittent pulse cyclophosphamide therapy (1993) Ann Intern Med, 119, pp. 366-369Fogelman, I., The effects of oestrogen deficiency on the skeleton and its prevention (1996) Br J Obstet Gynaecol, 103, pp. 5-9. , 14Van Der Schoun, Y.T., Van Der Graaf, Y., Steyeberg, E.W., Eijkemans, J.C., Banga, J.D., Age at menopause as a risk factor for cardiovascular mortality (1996) Lancet, 347, pp. 714-718Tan, E.M., Cohen, A.J., Fries, J.F., The 1982 revised criteria for the classification of systemic lupus erythematosus (1982) Arthritis Rheum, 25, pp. 1271-1277Bombardier, C., Gladman, D.D., Urowitz, M., Caron, D., Chang, C., Derivation of the SLEDAI: A disease activity index for lupus patients (1992) Arthritis Rheum, 35, pp. 630-640. , The Committee on Prognosis Studies in SLEGladman, D., Glinzler, E., Goldsmith, C., The development and initial validation of the Systemic Lupus International Collaborating Clinics/American College of Rheumatology damage index for systemic lupus erythematosus (1996) Arthritis Rheum, 39, pp. 363-378Brandt, J.T., Triplett, D.A., Scharer, I., Criteria for the diagnosis of lupus anticoagulant: An update (1995) Thromb Haemost, 74, pp. 1185-1190Tincani, A., Cyclophosphamide therapy induces ovarian failure in premenopausal women affected by systemic lupus erythematosus (2001) Clin Exp Rheumatol, 19, pp. 490-491Blumenfeld, Z., Shapiro, D., Shteinberg, M., Avivi, I., Nahir, M., Preservation of fertility and ovarian function and minimizing gonadotoxicity in young women with systemic lupus erythematosus treated by chemotherapy. (2000) Lupus, 9, pp. 401-405Pasoto, S.G., Mendonca, B.B., Bonfa, E., Menstrual disturbances in patients with systemic lupus erythematosus without alkylating therapy: Clinical, hormonal and therapeutic associations (2002) Lupus, 11, pp. 175-18

Antithrombin, Protein S And Protein C And Antiphospholipid Antibodies In Systemic Lupus Erythematosus

Patients with systemic lupus erythematosus (SLE) have an increased risk of thrombosis, related to the lupus anticoagulant or anticardiolipin antibodies (ACL). Antithrombin (AT), protein C (PC) and protein S are natural anticoagulants with a very important role in the regulation of coagulation and fibrinolysis, and in the prevention of thrombosis. Total protein S (TPS), free protein S (FPS), AT and PC were measured in 53 unselected patients with SLE in order to verify their relation with previous history of thrombosis or the presence of anticardiolipin antibodies (ACL). The influence of prednisone use on the concentration of these natural anticoagulants was also analysed. The control group was formed by 20 blood donors. The PC concentration was increased in patients with ACL, when compared to controls (p < 0.01), or patients without ACL (p = 0.01). FPS levels were decreased in patients with SLE in relation to controls (p = 0.01), but not related to thrombosis or ACL. There was no difference in plasmatic concentration of AT and TPS, between patients and controls. PC, AT and TPS were increased in patients under prednisone use (p < 0.01). There was no association between concentration of natural anticoagulants and a history of thrombosis. Although decreased FPS was found, or results suggest that it seems not to be a risk factor for thrombosis in SLE. Since PC and AT were not decreased, they should not be involved with thromboembolic complications in these patients. The use of prednisone can influence concentration of natural anticoagulants in patients with SLE.435345348Hugues, G.R.V., Harris, E.N., Gharavi, A., The anticardiolipin syndrome (1986) J.Rheumatol, 13, pp. 486-489Love, P.E., Santoro, S.A., Antiphospholipid antibodies: Anticardiolipin and the lupus anticoagulant in systemic lupus erythemathosus (SLE) and in non-SLE disorders. Prevalence and clinical significance (1990) Ann Intern Med, 112, pp. 682-698Asherson, R.A., Cervera, R., Anticardiolipin antibodies, chronic biologic false-positive tests for syphilis and other antiphospholipid antibodies (1993) Dubois Lupus Erythemathosus. 4 Ed., pp. 233-245. , Wallace DJ, Hahn BH (eds): Lea Le: FebigerFreyssinet, J.M., Cazenave, J.P., Lupuslike anticoagulants, modulation of the Protein C pathway and thrombosis (1987) Thromb Haemost, 58, pp. 679-681Moreb, J., Kitchens, C.S., Acquired functional Protein S deficiency, cerebral venous thrombosis, and coumarin skin necrosis in association with antiphospholipid syndrome: Report of two cases (1989) Am J Med, 87, pp. 207-210Watter, T.S., Triplett, D.A., Javed, N., Musgrave, K., Evoluation of lupus anticoagulants: Antiphospholipid antibodies, endothelium associated immunoglobulin, endothelial prostacyclin secretion and antigenic protein S levels (1988) Thromb Res, 51, pp. 267-281Ruiz-Argüelles, G.J., Ruiz Argüelles, A., Alarcon-Segovia, D., Drenkard, C., Villa, A., Cabiedes, J., Natural anticoagulants in systemic lupus erythemathosus. Deficiency of protein S bound to C4bp associates with recent history of venous thrombosis, antiphospholipid antibodies, and the antiphospholipid syndrome (1991) J Rheumatol, 18, pp. 552-557Hasslear, P., Derksen, R.H.W.M., Blokzijl, L., Hessing, M., Nieuwenhuis, H.K., Bouma, B.N., Risk factors for thrombosis in lupus patients (1989) Ann Rheum Dis, 48, pp. 933-940Keeling, D.M., Campbell, S.J., Mackie, I.J., Machin, S.J., Isenberg, D.A., Total and free Protein S in systemic lupus erythemathosus (1990) Thromb Research, 60, pp. 237-240Cosgriff, T.M., Martin, B.A., Low functional and high antigenic antithrombin III level in a patient with the lupus anticoagulant and recurrent thrombosis (1981) Arthritis Rheum, 24, pp. 94-96Tan, E.M., Cohen, A.S., Fnes, J.F., Masi, A.T., McShane, D., Rothfield, N.F., The 1982 revised criteria for the classification of SLE (1982) Arthritis Rheum, 25, pp. 1271-1277Loizou, S., McCrea, J.D., Rudge, A.C., Reynolds, R., Boyle, C.C., Harris, E.N., Measurement of anticardiolipin antibodies by an enzyme-linked immunoabsorbents assay (ELISA): Standardization and quantitation of results (1985) Clin Exp Immunol, 62, pp. 738-745Comp, P.C., Doray, D., Esmon, C.T., An abnormal distribution of protein S occurs in functional protein S deficiency (1986) Blood, 67, pp. 504-508Park, A.L., Weinstein, R.E., Bona, R.D., Maier, D.B., Walker, F.J., The thrombotic diatesis associated with the presence of phospholipid antibodies may be due to lower levels of free protein S (1992) Am J Med, 93, pp. 49-56Ames, P.R.J., Tommasino, C., Iannaccone, L., Brillante, M., Cimino, R., Brancacio, V., Coagulation activation and fibrinolytic imbalance in subjects with idiopathic antiphospholipid antibodies - A crucial role for acquired free protein S deficiency (1996) Thromb Haemost, 76, pp. 190-194Cronlund, M., Hardin, J., Burton, J., Lee, L., Haber, E., Bloch, K.J., Fibrinopeptide A in plasma of normal subjects and patients with disseminated intravascular coagulation and systemic lupus erithematosus (1980) Thromb Haemost, 20, pp. 617-622Dorsch, C., Meyerhoff, C., Elevated plasma beta-thromboglobulin levels in systemic lupus erythematosus (1980) Thromb Haemost, 20, pp. 671-1622Comp, P.C., Viganó, S., Angelo, A., Acquired protein S deficiency occurs in pregnancy, the nephrotic syndrome and acute systemic lupus erythemathosus (1985) Blood, 66, pp. 348aViganó, S., Manucci, P.M., Angelo, A., Gelfi, C., Gensini, C., Rostagno, C., Protein C Antigen is not an acute phase reactantand is often high in ischemic heart disease and diabetes (1984) Thromb Haemost, 52, pp. 263-266André, E., Voisin, P.H., Brisquel, M.E., Stoltz, J.F., Martinet, N., Alexandre, P., Hemorrhagical and hemostatic parameters in children with nephrotic syndrome undergoing steroid therapy (1994) Nephron, 68, pp. 184-191Kobayashi, N., Takeda, Y., Studies of the effects of stradiol, progesterone, cortisol, thrombophebitis and thyphoid vaccine on synthesis and catabolism of antithrombin III in the dog (1977) Thromb Haemost, 37, pp. 11-1

Cumulative Organ Damage Evaluation Using The Slicc/acr-di In Brazilian Patients With Systemic Lupus Erythematosus [avaliação Do índice De Danos Permanentes Através Do Slicc/acr-di Em Pacientes Brasileiros Com Lúpus Eritematoso Sistêmico]

Objective: to evaluate the frequency and the different types of organ damage in relation to disease duration in Brazilian patients with SLE followed in a tertiary reference center through the application of the SLICC/ACR-DI. Methods: sixty SLE patients were enrolled in this study, and relevant data were obtained through medical history, physical and laboratory examinations reviewing the hospital records. The frequency and different types of organ damage were determined using the SLICC/ACR-DI. Statistics were performed through qui-square and t-tests. Multivariate regression was used to correlate damage with disease duration. Results: forty-one patients (68.3%) presented some cumulative organ damage. Non-caucasoid patients and patients with longer disease duration had a slight tendency to have more damage (p = 0.058). Skin (35.0%), neuropsychiatric (18.3%), ocular (15.0%), peripheral vascularization (16.6%) and cardiovascular (10.0%) systems were more frequently affected. Patients with more than 60 months of disease had a slight tendency to present greater ocular, neuropsychiatric, renal, skin and musculoskeletal damage than patients with shorter disease duration. Patients with more than 120 months of disease had greater pulmonary, cardiovascular and peripheral vascular damage. Conclusions: in this study, 68.3% of permanent damage was observed. Skin, neuropsychiatric, ocular, peripheral vascularization and cardiovascular systems were more frequently affected. Renal and gonadal involvement was not as frequent as previously described. Non-caucasoid patients had a tendency to present higher scores, but more studies are necessary to determinate if ethnic or economic factors are involved.442109114Cervera, R., Khamashta, M.A., Font, J., Morbidity and mortality in systemic lupus erythematosus during a 10-year period: A comparison of early and late manifestations in a cohort of 1,000 patients (2003) Medicine, 82, pp. 299-308. , (Baltimore)Moss, K.E., Ioannou, Y., Sultan, S.M., Outcome of a cohort of 300 patients with systemic lupus erythematosus attending a dedicated clinic for over two decades (2002) Ann Rheum Dis, 61, pp. 409-413Trager, J., Ward, M.M., Mortality and causes of death in systemic lupus erythematosus (2001) Curr Opin Rheumatol, 13, pp. 345-351Petri, M., Hopkins lupus cohort (2000) Rheum Dis Clin North Am, 26, pp. 199-213Gladman, D., Glinzler, E., Goldsmith, C., The development and initial validation of the Systemic lupus International Collaborating Clinics/ American College of Rheumatology damage index for systemic lupus erythematosus (1996) Arthritis Rheum, 39, pp. 363-378Zonana-Nacach, A., Camargo-Coronel, A., Yanez, P., Measurement of damage in 210 mexican patients with systemic lupus erythematosus: Relationship with disease duration (1998) Lupus, 7, pp. 119-123Tan, E.M., Cohen, A.S., Fries, J.F., The 1982 revised criteria for the classification of systemic lupus erythematosus (1982) Arthritis Rheum, 25, pp. 1271-1277Folstein, M.F., Folstein, S.E., Folstein, P.R., "Mini mental state" A practical method for grading the cognitive state of patient for the clinician (1975) J Psychiatr Res, 12, pp. 189-198Appenzeller, S., Fujisawa, G., Costallat, L.T.L., Distúrbios cognitivos no lúpus eritematoso sistêmico (1999) Rev Bras Reumatol, 39, pp. 12-18Rahman, P., Glagman, D.D., Urowitz, M.B., Early damage as measured by the SLICC/ACR damage index is a predictor of mortality in systemic lupus erythematosus (2001) Lupus, 10, pp. 93-96Stoll, T., Seifert, B., Isenberg, D.A., SLICC/ACR damage index is valid, and renal and pulmonary organ scores are predictors of severe outcome in patients with SLE (1996) Br J Rheumatol, 35, pp. 248-245Stoll, T., Stucki, G., Malik, J., Association of the SLICC/ACR damage index with measures of disease activity and health status in patients with SLE (1997) J Rheumatol, 24, pp. 309-313Zonana-Nacach, A., Barr, S.G., Magder, L.S., Damage in systemic lupus erythematosus and its association with corticosteroids (2000) Arthritis Rheum, 43, pp. 1801-1808Gorgos, L., Goldman, D., Petri, M., The ACR/SLICC damage index in SLE (1993) Arthritis Rheum, 36, pp. S68Karlson, E.W., Daltroy, L.H., Lew, R.A., The relationship of socioeconomic status, race, and modifiable risk factor to outcomes in patientes with SLE (1996) Arthritis Rheum, 40, pp. 47-5

Cognitive Disorders In Systemic Lupus Erythematosus [disturbios Cognitivos No Lupus Eritematoso Sistemico]

Objective: To determine the frequency and nature of cognitive disorders in systemic lupus erythematosus (SLE). Methods: 40 patients with systemic lupus erythematosus (SLE) (ARA's criteria, 1982) were analyzed with special emphasis to cognitive disorders. To find cognitive disorders the authors used several tests, including Mini-mental, logic memory, long-term memory; long- and short-term memory; verbal fluency. The control group, composed of 40 patients with Rheumatoid Arthritis (RA) (Arnett, 1989) and 40 persons without any chronic disease, went through the same inventory. Statistics were performed by chi-square tests and non-parametric tests. Results: Cognitive disorders were observed in 75% in SLE compared to 30% in RA (p=0.0001) and to 7.5% in the healthy group (p<0.0001). Conclusion: Even if a greater incidence of cognitive disorders were observed in SLE (75%) and RA (30%) when compared to the healthy group (7.5%), the difference between SLE and RA was statistically significant in all tests employed. There was no relation between cognitive disorders and duration or activity of illness or with the use of corticosteroid therapy: The use of specific tests augments the sensibility for the detection of cognitive disorders in SLE and in other chronic disease.392707

Hla Antigens And Susceptibility To Systemic Lupus Erythematosus In Brazilian Patients

Objective: The aim of this study was to investigate association between systemic lupus erythematosus (SLE) and HLA antigens in Caucasoid Brazilian population. Methods: The HLA-A, B, DR and DQ antigens typing was performed using microlymphocytotoxicity test. Fifty-six unrelated Brazilian Caucasoid patients with SLE were studied. Control population for HLA A and B antigen frequency consisted of one hundred-fifty nine unrelated healthy Brazilian Caucasoid and for HLA DR and DQ one hundred-forty one. SLE patients were divided in two groups: 1) with nephritis and 2) without renal involvement. Results: The frequency of HLA-B8 was significantly increased in SLE patients than in controls (25.4% vs 6.9%). HLA-DR2 was significantly increased in SLE patients than was in controls (41% vs 19.1%, RR = 2.95; p < 0.005). When SLE nephritis patients were compared to controls, the frequency of HLA-DR2 (44% vs 19.1%) and HLA-DR3 (32% vs 14.2%) were significantly increased. On the other hand, the frequency of HLA-DR2 was increased in the group of SLE patients without renal disease when compared with control group (37.5% vs 19.1%). Conclusion: Our data suggested that HLA-B8 and HLA-DR2 might have a role in the susceptibility to SLE and HLA-DR3 may be associated with nephritis in Caucasoid Brazilian population with SLE.38633233