50 patients falling in the age group of 18-60 years with histologically and immunologically confirmed oral pemphigus vulgaris who meet at least one of the following:

Patients were enrolled based on inclusion and exclusion criteria after obtaining written and informed consent. Pre-treatment assessments were conducted including a detailed review of the patient's history with pemphigus vulgaris, prior treatments and any accompanying medical conditions. A comprehensive clinical examination was also performed to evaluate the extent and severity of oral lesions. Complete hemogram, erythrocyte sedimentation rate, renal function tests, and liver function tests, serology for HIV infection, hepatitis B surface antigen, anti-HCV (hepatitis C virus) antibody, and anti-HBc (hepatitis B core) antibody, Mantoux test, chest X-ray, Cardiology evaluation, electrocardiography and echocardiogram (if advised by a cardiologist), Estimation of immunoglobulin (Ig) G level in serum, Baseline serum level of antidesmoglein 1 and 3 antibodies, Ultrasound abdomen (if indicated clinically based on symptoms and clinical examination) were conducted in the initial visit. Premedication with Tab Paracetamol 650 mg, Inj. Hydrocortisone 100 mg IV, Inj Pheniramine maleate 2 cc IV were given.

IV rituximab was administered at a dose of 500 mg in 500 ml NS with first dose of rituximab infusion starting at 50 ml/hr. 50 mg increments were made in infusion rate every 30 mins to a maximum of 400 mg/hr. Initially, the infusion was started at a rate of 30 ml per hour for the first 30 minutes, delivering a volume of 15 ml. This cautious beginning allows for monitoring of any immediate adverse reactions. Subsequently, the infusion rate was increased to 60 ml per hour for the following 30 minutes, delivering 30 ml of Rituximab. As the treatment progresses, the infusion rate was further escalated to 90 ml per hour until the total volume of 455 ml was infused over a period of approximately 5 hours. The entire infusion process for the first dose typically spans a total duration of 6 hours, ensuring the medication is administered safely and effectively while minimizing potential complications.

Patients were given 10 mg rituximab per session under local anesthesia (2% lidocaine) with dosages modified according to the severity of the lesion. Injections are targeted directly at the base and margins of oral lesions using a 30-gauge needle, employing multiple angles to surround the lesion effectively within the same plane.

The patient's vital signs including pulse rate, blood pressure, respiratory rate and pulse oximetry were monitored at 30-minute intervals during the administration of treatment. Symptoms such as fever, rigors, nausea, vomiting, headache, hypotension, and abdominal pain were closely observed for signs of infusion reactions. Emergency medications including epinephrine, hydrocortisone, chlorpheniramine maleate, and furosemide were prepared in appropriate doses as a precautionary measure to manage potential adverse reactions. In the event of serious adverse effects like angioedema or anaphylaxis, the infusion was immediately halted and the patient received prompt treatment tailored to the specific symptoms. Following such incidents, efforts were made to investigate and treat the underlying cause responsible for the reaction, ensuring comprehensive patient care and safety throughout the treatment process.

Throughout the treatment course for pemphigus vulgaris, patients were scheduled for visits initially every 15 days over 2 months, followed by monthly visits for the subsequent 6 months. Each visit included a thorough assessment and monitoring protocol aimed at ensuring effective disease management and patient well-being. During these visits, a thorough clinical assessment of oral lesions & documentation of lesion progression through photography for objective comparison was conducted. Patients were also closely monitored for infections, alongside performing regular complete blood count, liver function tests, and renal function tests to evaluate overall health and medication tolerance. At 3-month and 6-month intervals, additional assessments included measuring serum IgG levels and anti-desmoglein antibody titers to track treatment response and utilizing the Autoimmune Bullous Disease Quality of Life (ABQOL) questionnaire to assess the impact of the disease and treatment on the patient's quality of life. This comprehensive approach ensured continuous evaluation, adjustment of treatment strategies as needed, and comprehensive support for patients throughout their treatment journey.

The results were statistically analysed using Chi square test and statistical significance (p value) methods.

The study observed that the majority of oral pemphigus patients (38, 76.0%) belonged to the age group of 31–40 years, followed by 12 patients (24.0%) in the age group of 41–50 years. Oral pemphigus showed a higher prevalence in the age range of 31–40 years. The minimum age of patient was 31 years, and the maximum age was 47 years. The mean age of male patients was 36.87 years, and the mean age of female patients was 38.09 years. The mean age of all patients was 37.70 years. There was no statistically significant difference in age between genders (P > 0.05).

Also, the study observed that out of 50 oral pemphigus patients sampled, 45 (90.0%) had oral involvement, 4 (8.0%) showed minimal involvement, and 1 (2.0%) patient showed no involvement. Investigations were conducted on all 50 patients (100.0%).

Comparison of treatment response:

In evaluating treatment outcomes for pemphigus vulgaris, primary goals were to achieve complete remission (CR), characterized by the absence of new lesions and substantial healing of existing one. Partial remission (PR) was defined as a ≥50% reduction in lesions. Secondary endpoints focused on additional measures such as assessing the time required to achieve CR or PR, monitoring the 6-month relapse rate to gauge the necessity for increased therapy due to new lesions. These comprehensive endpoints collectively provided a thorough assessment of treatment effectiveness, safety, and patient outcomes in managing pemphigus vulgaris.

Complications that had been reported with rituximab therapy included infusion reactions, infections, hypogammaglobulinemia, tumor lysis syndrome, progressive multifocal encephalopathy, and Hepatitis B reactivation. However, in our study involving low-dose intravenous and intralesional rituximab therapy, we encountered only 3 incidents (6%) where patients complained of chills following the initiation of infusion. These reactions were promptly managed, and therapy continued without interruption.

Despite the promising outcomes, certain limitations of this study should be acknowledged. Our sample size of 50 patients, while providing valuable insights, may not be sufficient to detect rare adverse events or establish definitive long-term safety profiles. A significant limitation of rituximab therapy remains it’s high cost, which poses a substantial financial burden for many patients. Despite implementing a low-dose protocol to minimize expenses, the cost of treatment remains a significant barrier impacting both access to care and patient compliance.

The single-center nature of our study limited the diversity of patient demographics and socioeconomic backgrounds, which is particularly relevant when evaluating treatment affordability across different healthcare settings. A broader, more diverse patient population would provide more generalizable results regarding both efficacy and economic feasibility of this novel protocol.

Nil.

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published, and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Nil.

There are no conflicts of interest.