scholarly journals Modifications of pulse therapy in pemphigus: a retrospective study of 72 patients

Author(s):  
Neela V. Bhuptani ◽  
Khushbu P. Chauhan ◽  
Monal M. Jadwani ◽  
Pooja Raja

<p class="abstract"><strong>Background:</strong> Pulse therapy defines as the administration of supra-pharmacologic doses of drugs in an intermittent manner to enhance the therapeutic effects and reduce the side effects. Dexamethasone-Cyclophosphamide pulse (DCP) therapy is known since 1986 but there are certain limitations due to side effects of cyclophosphamide.</p><p class="abstract"><strong>Methods:</strong> A retrospective study was carried out where 72 patients of pemphigus were treated with modified pulse therapy like DCP, DAP, DMP from 2006-2016. Modifications were made in DCP therapy protocol and substitution of cyclophosphamide with either azathioprine or methotrexate in few patients.<strong></strong></p><p class="abstract"><strong>Results:</strong> Male to female ratio observed was 1:0.7. Majority of them belonged to age group of 31-40 years (41.66%) followed by 41-50 years (33.3%). Maximum number of patients had pemphigus vulgaris (86.1%) followed by pemphigus foliaceous (12.5%) and IgA pemphigus (1.38%). Good response was observed in patients who took pulse therapy regularly.</p><p class="abstract"><strong>Conclusions:</strong> Modifications to the original DCP therapy protocol were found to be very effective, useful and it shortened the duration of phase I. Side effects were minimal and manageable.</p>

2013 ◽  
Vol 88 (4) ◽  
pp. 672-675 ◽  
Author(s):  
Nurimar Conceicao Fernandes ◽  
Mariana Menezes

In this study, five cases of pemphigus vulgaris and two cases of pemphigus foliaceus were treated with cyclophosphamide pulse therapy associated with prednisone, resulting in the need for a smaller maintenance dose of prednisone. In three cases of pemphigus vulgaris and one case of pemphigus foliaceus, dexamethasone and cyclophosphamide pulse therapy associated with prednisone helped the lesions to heal more rapidly. Neither treatment however prevented the recurrence of the disease. Amenorrhea, myelotoxicity and Stevens-Johnson syndrome were among the cyclophosphamide side effects. All the patients treated with prednisone experienced known side effects.


2021 ◽  
Vol 12 (5) ◽  
pp. 275-277
Author(s):  
Kriti Sareen ◽  
Fozia Sultana ◽  
Zabin Mirza ◽  
Kalpna Thakur ◽  
Ekta Parmar ◽  
...  

2020 ◽  
Vol 7 ◽  
Author(s):  
Roberta Scarpone ◽  
Wojciech Francuzik ◽  
Margitta Worm ◽  
Guido Heine

Pemphigus diseases are rare, and the treatment response differs between patients. Several therapy changes are often required to achieve disease control and avoid unwanted side effects. We aimed to analyze the treatment courses of pemphigus patients and the clinical responses regarding therapy changes. Pemphigus patients in our center were retrospectively examined according to the medication and dosage, disease activity, reason for treatment changes, and autoantibody concentrations. Therapy changes due to insufficient therapeutic effects or side effects were analyzed. Seventy-seven pemphigus patients with repeated consultations were identified (81% pemphigus vulgaris, 19% pemphigus foliaceus). Disease control was achieved in 66 patients (86%; score “almost clear” or “clear”), with an average of 4 different therapy regimens (range 1–18 changes), after an average of 2 years of treatment (range 0–11 years). Twenty-two patients (29%) with refractory disease received rituximab, of which 19 (86%) subsequently achieved remission. Anti-desmoglein-1 and−3 concentrations correlated with disease severity, but not with the number of treatment changes. The identification of an effective and safe therapy for the individual pemphigus patient is a challenge and often requires time, which is reflected by a high number of therapy changes. Predictive parameters are warranted to directly identify the safest and most efficient treatment regimen for an individual patient.


Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Hiroshi Katayama ◽  
Kanta Kishi ◽  
Ozaki Noriyasu ◽  
Yutaka Odanaka ◽  
Hiroshi Tamai

Background: The RAISE study showed that combination therapy with intravenous immunoglobulin (IVIg) and prednisolone (PSL) improved coronary artery outcomes in patients with severe Kawasaki disease (KD). We encountered KD cases in which fever and findings such as WBC were masked by the steroid administration in combination therapy. We hypothesized that combination therapy might delay second- and third-line therapy for severe KD. Purpose: The goal of the study was to investigate whether combination therapy with IVIg and PSL might delay second- and third-line therapy for severe KD patients. Methods: We retrospectively investigated clinical data from acute KD patients admitted to our hospital from January 2011 to September 2014. We examined the following: 1) prediction score for refractory KD 2) first-line therapy for KD (IVIg group, n=10; IVIg/PSL group, n=11); 3) additional rescue therapy such as additional IVIg, steroid pulse therapy, plasma exchange, and so on 4) period from KD onset to end of main therapy for KD; 5) day of increased albumin from previous examination; and 6) day of CRP reaching <1.0 mg/l. Results: Seventy-four acute KD patients were admitted to our hospital during the study period. Only 1 KD patient had coronary artery lesions (CAL) at 1 month after onset. Detailed data were as follows: 1) Twenty-one patients were at high risk for refractory KD. 2) number of patients with additional IVIg (IVIg, 6/10; IVIg/PSL, 6/11); 3) number of patients with steroid pulse therapy (IVIg, 4/10; IVIg/PSL, 4/11); 4) period from KD onset to end of main therapy (IVIg, 8.3 days; IVIg/PSL, 8.7 days); 5) day of increased albumin (IVIg, 12.0 days; IVIg/PSL, 11.8 days); 6) day of CRP reaching <1.0 mg/l (IVIg, 13.7 days; IVIg/PSL, 11.4 days). No differences between IVIg and IVIg/PSL groups were seen for any factor. Discussion and conclusion: In this study, overall frequency of CAL was 1.3%, lower than that reported from nationwide surveillance in Japan. This study found no differences between groups in regard to frequency or timing of additional therapy and response. We concluded that combination therapy with IVIg and PSL did not delay the timing of additional therapy for acute KD. The cohort for this retrospective study was small, and further investigation is thus needed.


Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 4951-4951
Author(s):  
C. Michael Jones ◽  
Tina M. Dickinson

Abstract Imatinib has recently been demonstrated to be an effective treatment in Polycythemia Vera. Initial response rates are high, approaching 80%. Patients enrolled in our trial, Novartis CST 1571B (US 144), were asked to complete a questionnaire identifying side effects of imatinib known from previous trials in chronic myeloid leukemia and GIST. Questionnaires were completed weekly for the first month, biweekly for the second month, and monthly for the remainder of the study. Mean time on study is now 1.1 years. Target enrollment was 20 patients. Mean age was 54 years; male to female ratio was 1.3: 1. Fourteen patients were Caucasian and 2 African American. Twelve patients were naïve to treatment and 4 patients were previously treated with hydroxyurea. Two dose escalations were allowed sequentially to 600mg and 800mg daily based on response using the Polycythemia Vera Study Group Criteria. Toxicity was graded according to the NCI Common Toxicity Criteria, version 2.0. Patient reported toxicities are expressed as the total number of events reported during the 120 week study period. Diarrhea was the most common reported toxicity at 53 events. Periorbital edema (33), pruritis (39), fatigue (29), arthralgias (20), headache (18), nausea (19), reflux (38), and dry mouth (15) were the next most commonly reported events on study. Other toxicities reported less commonly were chest pain (3), depression (4), cough (5), insomnia (6), weight gain (3), taste changes (7), and depigmentation (3). Most toxicities were mild at grade 1 and resolved spontaneously within 30 to 40 weeks from the time of treatment initiation. The exceptions to this were diarrhea, periorbital edema, and reflux esophagitis, which persisted in a number of patients throughout the study. The severity of gastrointestinal side effects was dose dependent. Previous studies of imatinib induced toxicity in patients with chronic myeloid leukemia have reported cutaneous manifestations as the most common event. Our study demonstrates the toxicities of imatinib in patients with polycythemia vera are generally mild and self limited, although resolution may take up to 35 weeks. In contrast with studies of imatinib and chronic myeloid leukemia, gastrointestinal toxicity was more common and tended to persist, but did not result in patient withdrawal from study. These differences from our findings may be the result of several factors: the use of a patient reported adverse events scale, small trial size, or differences in drug metabolism in a related myeloproliferative disorder.


Author(s):  
Vinay K. N. ◽  
Gouhare Afshan

<p class="abstract"><strong>Background:</strong> Pemphigus vulgaris is an autoimmune vesiculobullous disease characterized by vesicles and or bullae affecting skin and mucosa. Oral lesions are the first manifestation of the disease in majority of cases according to literature. This study is conducted to know the prevalence of oral lesions in pemphigus vulgaris patients at the time of presentation.</p><p class="abstract"><strong>Methods:</strong> This was a retrospective study. Departmental records were evaluated from January 2013 to January 2018. All the demographic data of pemphigus vulgaris patients at the time of first presentation, duration of disease, were assessed and tabulated. The clinical data of these patients during their subsequent visits to clinic were assessed for development of new oral lesions during course of disease.<strong></strong></p><p class="abstract"><strong>Results:</strong> Total number of patients were 55. The mean age was 42.5 years. 12 (21.8%) were males and 4 (78.1%) were females. Out of 55 patients 37 (67.27%) cases had oral involvement and 18 (32.72%) cases did not have oral lesions at the time of presentation. Out of 37 patients who had oral lesions, 34 (91.89%) patients had both oral and skin lesions and 3 (08.10%) patients had only oral lesions at the time of presentation. Out of the18 patients with no oral lesions, 3 (16.66%) patients developed oral lesions within 3 months. Total number of patients with oral lesions out of 55 were 40 (72.7%).</p><p class="abstract"><strong>Conclusions:</strong> Our study shows that oral lesions as initial manifestation of pemphigus vulgaris is 67.2%.</p>


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