The limited role and risky profile of Rituximab in nephritis associated with Henoch-Schönlein purpura

Adult-onset IgA vasculitis or Henoch-Schönlein purpura (HSP) is a systemic vasculitis characterized by IgA1-dominant deposits.  The symptoms include cutaneous purpura, ankle arthritis, enteritis and nephritis.  HSP nephritis (HSPN) can be severe and refractory to corticosteroids with/without immunosuppressive agents.  The concept of depletion of antibody producing B cell with Rituximab is appealing despite the uncertainity of HSP pathogenesis.  In the present case report; we describe our experience with Rituximab treatment in a patient with this disease.  Our patient had different triggering factors for her relapses and lately Rituximab itself.  Review of the literature indicates that autoantibodies to Gd-IgA1 did not decrease with Rituximab therapy and others indicated an inherited predisposition for higher levels in patients with progressive disease.  Our findings confirm the limited role and risky profile of Rituximab in treatment of HSP.  Keywords: HSP, IgA, Rituximab, vasculitis

The spectrum of adverse drug reactions in a multidisciplinary kidney clinic

Introduction: Data on adverse drug-reactions (ADR) in the medical field are rare. Objective: To report on the pattern of such problem in a multidisciplinary kidney clinic Patients and Methods: Medical records of patients were reviewed retrospectively for such phenomenon in the past 6 years Results: A total of 4834 patients were included for analysis.   The unit is responsible for a large proportion of patients with acute and chronic kidney diseases of diverse etiologies and multiple co-morbid conditions.  Acute and maintenance dialysis as well as immunosuppressive treatment for idiopathic glomerulopathy and autoimmune systemic diseases were common practice.  Results: A total of 70044 ADR were diagnosed in 4438 patients.  Most patients were adults (39+14) and had median follow up of 38 months. Nearly half of the ADR were due to drug-side effects while idiosyncrasy accounted for 1.2%.  The former is due to misuse/abuse of medications while the latter is due to genetic, co-morbid conditions or synergetic between 2 drugs or a drug and disease.  Details of drugs ADR are outlined with their respective prevalence.  Our study indicates the need for careful auditing of patient’s response during follow up to improve their drug-compliance. Keywords: adverse drug reaction, allergy, medical clinic, kidney disease

Rituximab therapy for Severe and Persistent Lichenoid drug-reaction

We report on a patient with severe and fixed lichenoid drug-reaction.  She did not respond to 2 months treatment with high-dose corticosteroids.  Hence, Rituximab was given.  One month later, her lesions improved and remained stable for 14 months of follow up.    Keywords: fixed drug reaction, Prednesone, Rituximab

Long-term maintenance therapy with Cyclosporine A in adults with generalized pustular psoriasis

Generalized pustular psoriasis (GPP) is a rare and serious immune-mediated skin disorder that is characterized by a widespread eruption of sterile and subcorneal pustules.  In the present study we investigated the efficacy of Cyclosporine A (Cy A) in treatment of 9 adults with drug-refractory GPP viz. topical Corticosteroids, retinoids, methotrexate and narrow-band ultraviolet light exposure (UVB).  Initially; they were resuscitated as burn patients. Cy A was administered on day 1 at a dose of 100 mg twice daily either in the form of syrup or Neoral capsules.  In most patients, skin lesions had healed by 6 weeks and the dose of Cy A was reduced to minimum to prevent further recurrence.  Seven patients had required 50 mg twice daily and 2 were controlled with 50 mg am and 25 mg pm.  On follow up, there was no serious relapse, liver and kidney disease.  Minor complications included; hirsutism and dark skin (n: 5) and gingival hyperplasia (n: 2). Trial to replace Cy A with Tacrolimus (Prograf) failed to maintain remission.  In conclusion; Cy A is a safe and effective treatment for GPP. Keywords: Cyclosporin A, treatment, psoriasis, pustular

Acquired Perforating Dermatosis: A Disorder Treatable with Mycophenolate Mofetil

Acquired perforating dermatosis (APD) is an adult skin disease characterized by an umbilicated papulonodular rash with transepidermal elimination of dermal components such as collagen and/or elastin. It is frequently associated with multiple medications and diseases such as diabetes and chronic renal failure. It is a disabling disease with severe pruritus in 83.3% of cases and generalized ulcerating lesions that are associated with infections and scarring. Nearly 10% of renal patients are affected. Supportive measurements of disease activity and previous medications failed to halt its natural progression. In our study, we documented significant improvements in the severity of the disease as measured by the eczema area and severity index (EASI), in 32 patients with the renal disease through the use of mycophenolate mofetil (MMF), with EASI decreasing from 31 [interquartile range (IQR) = 4] to 3 (IQR = 4) by the 3rd month. Moreover, such changes persisted for up to 2 years despite a decrease in the dose of MMF to half after 1 year. In conclusion, our study showed that MMF is a safe and effective immunosuppressive drug for short- and intermediate-term therapy of severe APD and confirmed its autoimmune etiology