Background: Chronic spontaneous urticaria (CSU) is an autoimmune skin disorder that lasts for >6 weeks and may last for years. It is a disabling skin disease that impairs quality of life. Set-up treatment with anti...Background: Chronic spontaneous urticaria (CSU) is an autoimmune skin disorder that lasts for >6 weeks and may last for years. It is a disabling skin disease that impairs quality of life. Set-up treatment with antihistamines, immunosuppressives, immune modulators and lately Omalizumab are expensive or have significant side effects. In this retrospective study, we describe our experience with the use of hydroxychloroquine (HCQ) as a maintenance therapy for those with severe forms of CSU after Corticosteroids (C) induction phase. Patients and Methods: 16 adult patients (aged 44 ± 7) with severe CSU for 5 ± 1 months, were included in the study. Eight patients had attacks of angioneurotic oedema. Their previous treatments were antihistaminic and short-courses of C. Results: After 2 weeks of remission with C and HCQ 200 mg twice daily, the dose of C was tapered down and discontinued by the end of the first month. The seven days Urticaria Activity Score decreased from 30 ± 3 to 6 ± 1 by the first month and remained low at 3 ± 1 by the end of 2 years of follow-up. Moreover, IgE levels and CRP had similar trends. Remission persisted after 37 ± 9 months of follow-up. Conclusion: HCQ is a safe, efficacious and inexpensive drug for the treatment of CSU.展开更多
Background: Systemic secondary amyloidosis (SSA) is associated with chronic inflammatory disorders and/or chronic infections. Patients and Methods: Over the past 10 years;a total of 21 patients, with long-term (17 mon...Background: Systemic secondary amyloidosis (SSA) is associated with chronic inflammatory disorders and/or chronic infections. Patients and Methods: Over the past 10 years;a total of 21 patients, with long-term (17 months) and extensive psoriasis (P) with psoriasis area severity index (PASI) >29, were evaluated. Results: Two patients had nephrotic syndrome (proteinuria 3.9 and 3.6 g/day) and decrease creatinine clearance (46 and 62 ml/minute). Their renal biopsy revealed Congo-red (+) nodular glomerulosclerosis that lacked immune-deposits and resisted wash with K-permanganate wash indicating SSA. Three months subsequent to Cyclosporin A (CyA) therapy with 100 mg twice daily;psoriasis improved in all patients with decrease in (PASI) from 29.5 to 3.5 1. In the 2 patients with SSA;proteinuria decreased to 2.1 and 1.8 g/day and creatinine clearance improved to 51 and 69 ml/minute. Such improvement persisted up to 2 years of follow up and up to 78 months in patients with SSA. Conclusion: psoriasis-induced SSA is an autoimmune disease, with genetic predisposition that is amenable to CyA therapy.展开更多
Background: Idiopathic Bullous Pemphigoid (IBP) is a rare blistering autoimmune disease. Its morbidity and mortality have remained high owing to complications of extensive skin involvement as well as its conventional ...Background: Idiopathic Bullous Pemphigoid (IBP) is a rare blistering autoimmune disease. Its morbidity and mortality have remained high owing to complications of extensive skin involvement as well as its conventional steroid therapy. We reviewed the medical literature and found indicators of an autoimmune etiology for its pathogenesis triggering genetically predisposed patients. Objective: to evaluate, prospectively, the role of Rituximab (R) therapy in its persistent, severe and extensive form. Patients and methods: A total of 12 patients, with disease duration of 6 ± 1 months, were treated with yearly R infusions (1 g followed by 1 g 2 weeks later). Results: Significant clinical improvement was achieved as documented by decrease in total score of Bullous Pemphigoid Disease Area Index from 60 ± 3 to 6 ± 2 that persisted for 26 ± 11 months of follow up. Moreover, IBP autoantibodies (anti-BP 180 and anti-320 IgG) levels fell from to 91 ± 3 and 81 ± 2 to 8 ± 2 and 9 ± 2, respectively. Conclusions: R is a safe and effective treatment for severe IBP and such response further confirms its autoimmune pathogenesis.展开更多
文摘Background: Chronic spontaneous urticaria (CSU) is an autoimmune skin disorder that lasts for >6 weeks and may last for years. It is a disabling skin disease that impairs quality of life. Set-up treatment with antihistamines, immunosuppressives, immune modulators and lately Omalizumab are expensive or have significant side effects. In this retrospective study, we describe our experience with the use of hydroxychloroquine (HCQ) as a maintenance therapy for those with severe forms of CSU after Corticosteroids (C) induction phase. Patients and Methods: 16 adult patients (aged 44 ± 7) with severe CSU for 5 ± 1 months, were included in the study. Eight patients had attacks of angioneurotic oedema. Their previous treatments were antihistaminic and short-courses of C. Results: After 2 weeks of remission with C and HCQ 200 mg twice daily, the dose of C was tapered down and discontinued by the end of the first month. The seven days Urticaria Activity Score decreased from 30 ± 3 to 6 ± 1 by the first month and remained low at 3 ± 1 by the end of 2 years of follow-up. Moreover, IgE levels and CRP had similar trends. Remission persisted after 37 ± 9 months of follow-up. Conclusion: HCQ is a safe, efficacious and inexpensive drug for the treatment of CSU.
文摘Background: Systemic secondary amyloidosis (SSA) is associated with chronic inflammatory disorders and/or chronic infections. Patients and Methods: Over the past 10 years;a total of 21 patients, with long-term (17 months) and extensive psoriasis (P) with psoriasis area severity index (PASI) >29, were evaluated. Results: Two patients had nephrotic syndrome (proteinuria 3.9 and 3.6 g/day) and decrease creatinine clearance (46 and 62 ml/minute). Their renal biopsy revealed Congo-red (+) nodular glomerulosclerosis that lacked immune-deposits and resisted wash with K-permanganate wash indicating SSA. Three months subsequent to Cyclosporin A (CyA) therapy with 100 mg twice daily;psoriasis improved in all patients with decrease in (PASI) from 29.5 to 3.5 1. In the 2 patients with SSA;proteinuria decreased to 2.1 and 1.8 g/day and creatinine clearance improved to 51 and 69 ml/minute. Such improvement persisted up to 2 years of follow up and up to 78 months in patients with SSA. Conclusion: psoriasis-induced SSA is an autoimmune disease, with genetic predisposition that is amenable to CyA therapy.
文摘Background: Idiopathic Bullous Pemphigoid (IBP) is a rare blistering autoimmune disease. Its morbidity and mortality have remained high owing to complications of extensive skin involvement as well as its conventional steroid therapy. We reviewed the medical literature and found indicators of an autoimmune etiology for its pathogenesis triggering genetically predisposed patients. Objective: to evaluate, prospectively, the role of Rituximab (R) therapy in its persistent, severe and extensive form. Patients and methods: A total of 12 patients, with disease duration of 6 ± 1 months, were treated with yearly R infusions (1 g followed by 1 g 2 weeks later). Results: Significant clinical improvement was achieved as documented by decrease in total score of Bullous Pemphigoid Disease Area Index from 60 ± 3 to 6 ± 2 that persisted for 26 ± 11 months of follow up. Moreover, IBP autoantibodies (anti-BP 180 and anti-320 IgG) levels fell from to 91 ± 3 and 81 ± 2 to 8 ± 2 and 9 ± 2, respectively. Conclusions: R is a safe and effective treatment for severe IBP and such response further confirms its autoimmune pathogenesis.
