Research Results

(1)

Misdiagnosis Association & Society, Seattle, WA, USA

Keywords

Therapy-resistant pemphigusRituximabLab indicesIntravenous immunoglobulin (IVIG)CyclophosphamideMycophenolate mofetilMethotrexateAzathioprine

Results

Among 105 therapy-resistant pemphigus patients who received rituximab treatment in Razi Hospital, Tehran, Iran, from 2008 to 2012, only 39 patients managed to enter the study. The others were excluded due to inadequate data. Also in the included patient group, the maximum time interval between the last dosage of rituximab and follow-up was 1 month. The data of the remaining 39 patients were analyzed by Stata statistical software (StataCorp, Texas, USA), and the following results were obtained:

The age of the patients ranged from 16 to 67 with a mean of 36.46 years.
Their disease duration from the beginning of the disease until receiving rituximab ranged from 5 to 84 months with a mean of 39.30 months.
Of the patients, 25 (64%) were men and 14 (36%) were women. It does not seem that the sex difference is related to therapy-resistant pemphigus ; it is rather associated with the data collection method and exclusion of patients with incomplete files.

Investigation of the involved sites showed that 25 patients (64%) had mucosal involvement, 20 patients (51.3%) had upper body involvement, 18 patients (46.2%) had lower body involvement, 19 people (48.7%) had genitalia involvement, 23 people had facial involvement, 36 people (92%) had body involvement, and in 22 patients (56.4%) the scalp was involved. The lab result variations of the mentioned patients were investigated in terms of the involved sites.

The patients, before application of rituximab , were simultaneously under treatment with prednisolone and other adjoins. To summarize the unsuccessful treatments, 5 patients had cyclophosphamide , 18 of them received CellCept® (mycophenolate mofetil ), 7 people (17.9%) had intravenous immunoglobulin (IVIG) , 5 patients were treated with methotrexate , and 22 patients had azathioprine . All these patients did not respond to corticosteroids and had active disease.

In terms of variation in lab test results after receiving rituximab, the patients were investigated in terms of the previous adjuvants as well. Among 39 patients, 12 of them (30.8%) had systemic underlying diseases such as hypertension (HTN), diabetes mellitus (DM), ischemic heart disease (IHD), and many more.

The major variables were white blood cell (WBC), hemoglobin (Hgb), platelet (Plt), aspartate aminotransferase (AST), alanine aminotransferase (ALT), urea, and creatinine (Cr) before and after application of rituximab.

Before Receiving Rituximab

The WBC range was 4,000–14,800 with an average of 10,092.
The Hgb range was 9.1–16.8 with an average of 13.8.
The Plt range was 100,000–683,000 with an average of 243,384.
The AST range was 6–64 with an average of 24.56.
The ALT range was 10–143 with an average of 43.92.
The urea range was 12–145 with an average of 37.25.
The Cr range was 0.5–1.2 with an average of 0.87.

After Receiving Rituximab

The WBC range was 5,400–19,000 with an average of 9,964.
The Hgb range was 7.4–16.7 with an average of 13.42.
The Plt range was 110,000–440,000 with an average of 232,512.
The AST range was 10–121 with an average of 25.43.
The ALT range was 12–144 with an average of 48.46.
The urea range was 15–54 with an average of 29.12.
The Cr range was 0.6–1.2 with an average of 0.85.
The WBC had no statistically significant variations.
The Hgb had no statistically significant variations.
The Plt had no statistically significant variations.
The AST had no statistically significant variations.
The ALT had no statistically significant variations.
The urea had statistically significant variations.
The Cr had no statistically significant variations.

After receiving rituximab and adjusting for the effect of gender:

The WBC had no statistically significant variations.
The Hgb had no statistically significant variations.
The Plt had no statistically significant variations.
The AST had no statistically significant variations.
The ALT had no statistically significant variations.
The Cr had no statistically significant variations.
In the case of urea, we concluded that it depends on gender, as in men the variation was significant, while in women the variations were not statistically significant.

When investigating the results with adjustment of the involved sites, the following results were obtained:

In patients with lower body involvement, rituximab had no significant effect on WBC, Plt, AST, ALT, urea, and Cr, but it had significant impact on Hgb reduction.
In patients whose lower body was not involved, urea significantly increased after receiving rituximab.
In patients whose lower body was involved, rituximab caused a significant reduction in Cr, urea, and Hgb.
In patients whose upper body was not involved, rituximab had no significant effect on the variables.
In the patients with or without facial involvement, rituximab had no significant impact on any of the variables.
In patients whose genitalia region was involved, rituximab has no significant impact on any of the major variables.
In patients with no genitalia involvement, rituximab resulted in significant reduction of urea.
In patients with body involvement, rituximab resulted in significant reduction of urea.
In patients with scalp involvement, rituximab resulted in significant reduction of urea.

The adjustment of previous therapies was also addressed. As all the patients received prednisolone, the effect of adjoins (azathioprine, CellCept®, cyclophosphamide, IVIG , and methotrexate ) was addressed: