Document Detail

Efficacy and safety of cyclophosphamide, azathioprine, and cyclosporine (ciclosporin) as adjuvant drugs in pemphigus vulgaris.
MedLine Citation:
PMID:  17428113     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Pemphigus vulgaris is a potentially life-threatening, autoimmune bullous disease of the skin and mucous membranes. Most commonly, the disease is treated with prednisone in combination with an immunosuppressant agent, frequently referred to as adjuvant drug. However, there is no consensus regarding the first-choice adjuvant drug for the treatment of pemphigus vulgaris or the recommended dosage. OBJECTIVE: To evaluate the efficacy and safety of prednisone as monotherapy and in combination with the three most popular adjuvant agents - azathioprine, cyclosporine (ciclosporin), and cyclophosphamide - in the treatment of pemphigus vulgaris. METHODS: This was a retrospective study with a follow-up of 7-21 years. The study was conducted in an academic hospital with an outpatient division for patients with bullous diseases. A total of 101 patients with moderate-to-severe mucocutaneous pemphigus vulgaris were included in the study. For assessment of disease severity a 'pemphigus score,' based on the percentage of involved skin or oral mucous membranes, was developed. At treatment initiation the average pemphigus score was comparable in all treated groups of patients. Four treatment regimens were evaluated: oral prednisone at an initial dose of 100mg (1.1-1.5 mg/kg) per day as monotherapy, and prednisone combined with adjuvant drugs, i.e. oral azathioprine at a dose of 100mg (1.1-1.5 mg/kg) per day; cyclosporine (ciclosporin) at a dose of 2.5-3 mg/kg/day; or cyclophosphamide at a dose of 100mg (1.1-1.5 mg/kg) per day. The main outcome measures were average time to clinical remission, average time to immunologic remission (non-detectable circulating pemphigus vulgaris antibodies), proportion of patients who remained free of clinical relapse within 5 years after discontinuation of therapy, time from treatment discontinuation until first relapse, and incidence of adverse effects. RESULTS: The average (+/- SD) time to clinical remission was 7.2 +/- 13.1 months in patients who received prednisone monotherapy, 6.8 +/- 10.5 months in patients receiving additional azathioprine, 8.1 +/- 11.8 months in the cyclosporine group, and 4.9 +/- 6.9 months (which was significantly shorter than all other treatment groups, p < 0.05) in patients receiving cyclophosphamide. The average (+/- SD) times to immunologic remission were 33 +/- 27 months, 28 +/- 24 months, 30 +/- 21 months, and 23 +/- 17 months for prednisone monotherapy, azathioprine, cyclosporine, and cyclophosphamide, respectively. The proportions of patients who remained free of clinical relapse within 5 years after discontinuation of therapy were 55%, 50%, 43%, and 69% for prednisone monotherapy, azathioprine, cyclosporine, and cyclophosphamide, respectively. In patents who experienced relapse, the average (+/- SD) time from treatment discontinuation to clinical relapse was 10.50 +/- 6.86 months in patients receiving prednisone monotherapy, 16.40 +/- 17.36 months in the azathioprine group, 12.44 +/- 6.48 months in the cyclosporine group, and 21.16 +/- 20.13 months in the cyclophosphamide group. The safety profiles of all treatment regimens were comparable. CONCLUSION: Oral prednisone with cyclophosphamide is the most effective treatment for pemphigus vulgaris. All therapy regimens had a similar safety profile. In our opinion, cyclophosphamide at a dose of 1.1-1.5 mg/kg/day should be the adjuvant drug of choice in the treatment of moderate-to-severe pemphigus vulgaris.
Malgorzata Olszewska; Zofia Kolacinska-Strasz; Jadwiga Sulej; Hanna Labecka; Jaroslaw Cwikla; Urszula Natorska; Maria Blaszczyk
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  American journal of clinical dermatology     Volume:  8     ISSN:  1175-0561     ISO Abbreviation:  Am J Clin Dermatol     Publication Date:  2007  
Date Detail:
Created Date:  2007-04-12     Completed Date:  2007-06-19     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  100895290     Medline TA:  Am J Clin Dermatol     Country:  New Zealand    
Other Details:
Languages:  eng     Pagination:  85-92     Citation Subset:  IM    
Department of Dermatology, Warsaw Medical School, Warsaw, Poland.
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MeSH Terms
Azathioprine / therapeutic use*
Cyclophosphamide / therapeutic use*
Cyclosporine / therapeutic use*
Drug Therapy, Combination
Glucocorticoids / therapeutic use
Immunosuppressive Agents / therapeutic use*
Middle Aged
Pemphigus / drug therapy*
Prednisone / therapeutic use
Remission Induction
Retrospective Studies
Treatment Outcome
Reg. No./Substance:
0/Glucocorticoids; 0/Immunosuppressive Agents; 446-86-6/Azathioprine; 50-18-0/Cyclophosphamide; 53-03-2/Prednisone; 59865-13-3/Cyclosporine

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