Iranian guideline for rituximab therapy in pemphigus patients

Abstract

Pemphigus vulgaris (PV) is an autoimmune blistering disease affecting the skin and/or mucosa. Rituximab (RTX) has been approved recently by US FDA as an effective and safe treatment of PV. The high incidence of PV in Iran encouraged our team to prepare a consensus guideline for RTX administration based on literature review and a decade experience of an expert panel. RTX is recommended for the treatment of new cases of PV as well as patients not responding to conventional therapy. Contraindications include history of anaphylaxis or IgE‐mediated hypersensitivity to murine proteins of RTX, severe active infections, pregnancy, breastfeeding, severe heart failure, and arrhythmia. Prophylactic antiviral therapy is recommended in patients at risk of reactivation of HBV and isoniazid for those at risk of reactivation of tuberculosis. Concomitant use of systemic corticosteroids is recommended as a rule. Except for methotrexate, the combination with other immunosuppressive drugs is discouraged. Intravenous immunoglobulin is recommended for those at risk of infections or with extensive disease. The recommended dosage of RTX for the first cycle is 2 g either 500 mg weekly or 1 g biweekly. There is no general consensus whether the next doses of RTX be administered upon relapse or as maintenance therapy. We strongly recommend RTX sooner in the course of pemphigus.

Rituximab in practice: Clinical evaluation of patients with pemphigus after rituximab administration

“…In our previous study, we defined it as more than a 10‐point increase in PDAI score within 3‐month following RTX administration (19). Although post‐RTX exacerbation may be controlled by increasing the corticosteroid dose or using intravenous immunoglobulin (IVIg), resuming the discontinued cycle of RTX is not recommended in the severe cases of disease exacerbation 10,28,29 …”

“…However, most infusion reactions are tolerable and can be managed by repeating premedication and continuing the infusion at a slower rate. Severe infusion reactions such as urticaria and anaphylaxis that cause stopping of RTX infusion are rare while using desensitization protocol can make the subsequent infusions feasible 10 . Acute respiratory distress syndrome is another rare, life‐threatening infusion adverse effect and should be monitored during the infusion 44…”

Intralesional injection of biosimilar rituximab in recalcitrant mucocutaneous lesions of patients with pemphigus vulgaris: A pilot study

“…Individuals were not included in the study in the following situations: evidence of active or dormant infections such as hepatitis B, hepatitis C or human immunodeficiency virus, pregnancy or breastfeeding, progressive disorder of the nervous system, angina, heart arrhythmias, kidney disease, hyperphosphatemia, hypocalcemia, hypercalcemia, anemia, thrombocytopenia, or leukemia. 18 Additionally, the patients that received intravenous RTX for 6 months before the start of the study were excluded. 16,18…”

“…18 Additionally, the patients that received intravenous RTX for 6 months before the start of the study were excluded. 16,18…”

“…After obtaining an informed consent form, the results of the initial tests (complete blood count, tuberculin test [PPD], viral markers, liver, and kidney function test), and counseling the patients about cardiovascular, and infectious diseases were considered for the contraindications to RTX. 18 Then topical anesthesia with lidocaine 10% spray was applied and intralesional RTX (Zytux, AryoGen Pharmed) at a dose of 5 mg/cm 2 was injected on the first and 15th day similar to the dose in the previous study by Vinay et al 16 Before injection of RTX, intravenous hydrocortisone (100 mg), intramuscular chlorpheniramine, and acetaminophen oral tablet (500 mg) were administrated. Patients were followed up for 6 months after injections.…”