1/179. Immunoablative high-dose cyclophosphamide without stem cell rescue in paraneoplastic pemphigus: report of a case and review of this new therapy for severe autoimmune disease.Paraneoplastic pemphigus (PNP) is a refractory and life-threatening autoimmune mucocutaneous disease. We have recently reported the effectiveness and safety of ablative intravenous cyclophosphamide (200 mg/kg daily over 4 days) without stem cell rescue in patients with refractory autoimmune diseases including systemic lupus erythematosus, autoimmune cytopenias, chronic inflammatory demyelinating polyneuropathy, and aplastic anemia. We report chronic lymphocytic leukemia-associated PNP in a patient who presented with extensive and debilitating painful oral ulcerations and received ablative therapy. The patient tolerated the regimen well and showed a slow but sustained improvement despite persistence of the underlying neoplasm. Eighteen months after therapy, the oral ulcerations were almost completely healed and the circulating autoantibodies became negative. Currently, the patient remains on cyclosporine and a low dose of prednisone. This provides further evidence for the efficacy and safety of this regimen in the management of severe autoimmune diseases including PNP.- - - - - - - - - - ranking = 1keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
2/179. 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 = 0.052403094539872keywords = lupus (Clic here for more details about this article) |
3/179. 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 = 4.8446281170301keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
4/179. thymoma associated with systemic lupus erythematosus and immunologic abnormalities.The association between Systemic Lupus Erythematosus (SLE) and thymoma occurs with a greater frequency than dictated by coincidence alone. The immunologic effects of thymectomy on the appearance and/or the course of SLE are still to be elucidated. We report one case of SLE diagnosed at the same time as thymoma, and two cases of thymoma associated with immunologic disorders in the absence of clinical signs and symptoms diagnostic of SLE.- - - - - - - - - - ranking = 4keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
5/179. An unusual cause of bruising in an 80-year-old woman.PRESENTATION: a previously fit 80-year-old woman presented with a 2-week history of spontaneous and extensive bruising affecting all four limbs. The severity was such that she required a transfusion of 8 units of blood. RESULTS OF INVESTIGATIONS: a markedly prolonged activated partial thromboplastin time which was only partially corrected with normal plasma; tests for lupus anticoagulant were negative. factor viii levels were reduced and the Bethesda assay indicated an acquired inhibitor to factor viii. She was treated with a combination of intravenous immunoglobulin and immunosuppression. OUTCOME: the response to treatment was excellent, with a marked reduction in anti-factor viii antibody levels and resolution of the bruising over the next few weeks.- - - - - - - - - - ranking = 0.052403094539872keywords = lupus (Clic here for more details about this article) |
6/179. Acquired pure red cell aplasia--a case report.A married female patient of 36 years with chronic anaemia, because of pure erythroid aplasia with a haemolytic component and hypothyroidism due to antithyroid auto-antibodies, was subsequently discovered as a case of systemic lupus erythematosus (SLE). She was treated with corticosteroid and immunosuppressive therapy and her anaemia was corrected. The response of erythroid aplasia to corticosteroid and other immunosuppressive agents suggests that immunological factors play a role in erythroid aplasia in SLE. The occurrence of red cell aplasia in association with a variety of immune phenomenon supports the concept that in SLE, erythroid aplasia may be of immune aetiology.- - - - - - - - - - ranking = 1keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
7/179. Pediatric autoimmune cardiovascular disease.Pediatric autoimmune cardiovascular disease can cause serious, sometimes life threatening sequelae on the pediatric population. Valvular, myocardial, and pericardial involvement causing morbidity and mortality can occur in association to rheumatic heart disease, systemic lupus erythematosus, and juvenile rheumatoid arthritis. Serious and potentially life threatening coronary artery involvement can occur in patients with childhood polyarteritis nodosa, takayasu arteritis, and Kawasaki disease.- - - - - - - - - - ranking = 1keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
8/179. ovulation induction and early pregnancy loss in a woman susceptible to autoimmune diseases: a possible interrelationship.We report a 34-year-old woman with sequentially occurring autoimmune diseases that are possibly triggered by numerous ovulation inductions. At the ages of 26-32 years, she experienced 27 uncontrolled ovulation induction cycles using clomiphene citrate (CC) or CC plus human menopausal gonadotropin plus human chorionic gonadotropin. She became pregnant at the ages of 27, 30 and 31 with subsequent pregnancy loss in the 28th, 8th and 10th week of gestation, respectively. insulin-dependent diabetes mellitus (IDDM) developed at the age of 28. During the second year of ovulation induction, at the age of 27, she developed arthralgia that worsened and became migratory from the age of 31. thrombocytopenia appeared at the age of 33. The diagnosis of systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS) was established at the age of 34. To the best of our knowledge, this is the first case of concurrent IDDM, SLE and APS in a patient associated with ovulation inductions. Excessive levels of estradiol achieved during the ovulation inductions could play a role in the expression of multiple autoimmune diseases in the susceptible woman.- - - - - - - - - - ranking = 1keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
9/179. Long-term follow-up of ischaemic retinopathy in the antiphospholipid syndrome with lupus-like disease.PURPOSE: antiphospholipid syndrome (APS), as an acquired prothrombotic disorder, is increasingly being recognised as an important cause of systemic venous and arterial thrombosis. The defining feature of the condition is the presence of raised levels of antibodies to negatively charged phospholipids in the serum. methods: We describe 2 cases of APS with ocular involvement and review the recent literature. Both patients experienced acute visual loss. It was the presenting symptom in one case--a finding that led to the diagnosis of the syndrome. RESULTS: Management with anticoagulation therapy, in which the International Normalised Ratio (INR) has been maintained at or above 3, resulted in reperfusion of the ischaemic retina and stabilisation of the retinopathy in one patient, whilst in the other case, where the INR was less than 3, irreversible visual loss occurred. CONCLUSION: Anticoagulation with warfarin appears to result in reperfusion of ischaemic retina with stabilisation of the neovascular process when the INR is greater than 3.- - - - - - - - - - ranking = 0.20961237815949keywords = lupus (Clic here for more details about this article) |
10/179. A causal role for parvovirus B19 infection in adult dermatomyositis and other autoimmune syndromes.BACKGROUND: infection with parvovirus B19 (B19) has been associated with connective tissue disease (CTD) stigmata, namely, a systemic lupus erythematosus (SLE)-like illness, seronegative polyarthritis resembling rheumatoid arthritis, and vasculitis. The dermatopathology and pathogenetic basis of such B19-associated CTD-like syndromes have not been elucidated. OBJECTIVE: We attempted to document persistence of the B19 genome in skin lesions of 7 patients with CTD-like symptomatology following B19 infection and to correlate systemic manifestations to dermatopathological findings. METHOD: In 7 prospectively encountered patients in whom history, clinical signs and/or serology supported a diagnosis of CTD in the setting of B19 infection, dermatopathological and clinical features were correlated. parvovirus B19 viral genome was sought in skin tissue using the polymerase chain reaction (PCR). RESULTS: Two patients had clinical features diagnostic of myopathic dermatomyositis (DM), 1 of whom is still symptomatic 1.5 years after the onset of her illness, and the other has had typical clinical features of DM for a duration of 3.5 years. A 3rd patient with SLE remains symptomatic 4 years after the onset of her illness. A 4th patient has persistent seronegative symmetrical polyarthritis of 6 years' duration and cutaneous lesions of granuloma annulare (GA). The 5th patient has a 1.5-year history of debilitating polyarthritis and cutaneous lesions with overlap features of DM and subacute cutaneous LE (SCLE). The 6th patient has had a persistent folliculocentric necrotizing vasculitis for 3 years. The 7th patient has a 1-year history of microscopic polyarteritis nodosa (PAN) with cutaneous vasculitis and persistent active renal disease. In 4 patients, exposure to children with fifth disease immediately preceded the onset of their CTD. parvovirus B19 infection was documented serologically in 6 patients with antibodies of IgG subclass in 6 and of IgM subclass in 1. Four of 6 patients questioned had a history of atopy. skin biopsies from patients with clinical features of SLE or DM demonstrated an interface dermatitis with dermal mucinosis. A necrotizing vasculitis with epithelial pustulation was seen in 2 patients. Interstitial GA-like infiltrates were seen in 5 cases. Immunofluorescent (IF) testing revealed a positive lupus band test (LBT) and epidermal nuclear and vascular staining for IgG and C5b-9 in the SLE patient. One DM patient had a negative LBT in concert with C5b-9 deposition along the dermoepidermal junction (DEJ) and within blood vessels while the other showed endomysial vascular Cs5b-9 deposition. In all patients, skin biopsy material contained B19 genome, which was absent in the serum of 4 patients analyzed. Symptomatic relief followed immunosuppressive and immunomodulatory therapy with agents including prednisone, cyclophosphamide, hydroxychloroquine, non-steroidal anti-inflammatory drugs and etanercept, but no patient has had complete symptom resolution. CONCLUSIONS: Persistent B19 infection may be of pathogenetic importance in certain prototypic CTD syndromes, to which underlying immune dysregulation associated with a blunted IgM response to viral antigen may predispose. Anti-viral therapy might be worthy of consideration since traditional immunosuppressive therapy was unsuccessful in our cases.- - - - - - - - - - ranking = 1.0524030945399keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
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