0
We're unable to sign you in at this time. Please try again in a few minutes.
Retry
We were able to sign you in, but your subscription(s) could not be found. Please try again in a few minutes.
Retry
There may be a problem with your account. Please contact the AMA Service Center to resolve this issue.
Contact the AMA Service Center:
Telephone: 1 (800) 262-2350 or 1 (312) 670-7827  *   Email: subscriptions@jamanetwork.com
Error Message ......
Research Letter |

Kinetics of Response to Conventional Treatment in Patients With Pemphigus Vulgaris FREE

Annette Czernik, MS; Jean-Claude Bystryn, MD
Arch Dermatol. 2008;144(5):682-683. doi:10.1001/archderm.144.5.682.
Text Size: A A A
Published online

It is difficult to evaluate the effectiveness of new therapies for pemphigus vulgaris (PV) because controlled trials are rarely performed. Comparison to historical trials is also problematic because little is known about the frequency and time it takes for active pemphigus to respond to conventional treatment.1,2 To address this problem, we evaluated the kinetics of response in PV to conventional treatment with prednisone.

A retrospective study was conducted in 29 sequential patients with PV based on clinical, histological, and immunofluorescence criteria. All patients met the following inclusion criteria: (1) they had experienced a flare in disease activity (≥ 3 new lesions per week) while undergoing treatment with stable or tapering doses of prednisone; (2) they had been treated only with systemic steroids prior to the flare; and (3) the flare was treated only with prednisone. The median disease duration was 7 months (range, 2 months to 11 years). The median prednisone dose at the time of the flare was 2.5 mg/d (range, 0-32 mg/d). Patients were treated by increasing the dose of prednisone in steps of 10 to 20 mg/d every 1 to 2 weeks until disease activity was controlled (<1 new lesion per week). No adjuvant therapy was added. The dose that controlled disease activity was maintained until approximately 80% of the lesions were healed (end of consolidation phase), at which point the dose was gradually tapered, usually by 5 mg every 2 to 3 weeks.

The median time to control disease activity was 3 weeks (range, 1-17 weeks), and the median maximum dose of prednisone required was 40 mg/d (range, 5-90 mg/d) (Table 1). Disease activity was controlled 2 weeks after raising the prednisone dose in 41% of patients (n = 12) and after 3 weeks in 62% (n = 18) (data not shown). The end of the consolidation phase was reached after a median of 4 weeks from treatment of the flare (range, 1-17 weeks). The prednisone dose could be reduced by 50% of the maximum level required to control disease activity after a median of 7 weeks from the start of treatment (range, 1-30 weeks). After 4 months, 38% of patients achieved partial remission (no lesions for ≥1 month with <15 mg/d of prednisone) (n = 14); after 9 months, 62% (n = 18) (data not shown). Complete remission (no lesions for ≥1 month with no systemic treatment) was achieved in 24% of patients at 11 months (n = 7).

Table Graphic Jump Location1. Kinetics of Response to Conventional Treatment

The prednisone dose required to control disease activity and the kinetics of response were related to the baseline dose of prednisone at the time the flare occurred (Table 2). A median maximum prednisone dose of 20 mg/d was required to control disease activity in patients whose disease flared while they were not undergoing treatment with systemic steroids, while 45 mg/d was required for those on a prednisone regimen of 10 mg/d or higher. At 11 months, complete remission was induced in 36% (n = 4) and 0% of the 2 groups, respectively. Patients who were not undergoing systemic therapy prior to a flare reached the end of the consolidation phase and had a 50% decrease in their maximum prednisone dose a median of 2 weeks earlier in each case than those on a prednisone regimen greater than 10 mg/d (data not shown). There was 1 grade 2 (localized infection) and no grade 3 toxic effects during this 11-month period.3

Table Graphic Jump LocationTable 2. Relation Between Baseline Prednisone Dose and Kinetics of Response

These results indicate that flares in the activity of pemphigus vulgaris respond rapidly to conventional treatment with systemic steroids. Flares in disease activity were controlled a median of 3 weeks after increasing the steroid dose. After a median of 4 weeks, the steroid dose began to be tapered, and after 7 weeks, the dose could be tapered to half the maximum dose.

The rate of complete remission was 24% at 11 months (n = 7). This is consistent with a prior study, which found that complete remission occurred in 25%, 50%, and 75% of patients at 2, 5, and 10 years, respectively, following diagnosis.1 Partial remission was 76% at 11 months (n = 22), similar to a 71% remission rate after treatment with mycophenolate and prednisone after 13 months.4

Patients receiving a lower baseline dose of systemic steroids required less prednisone (lower maximum dose and increased rate of taper) and were more likely to achieve complete remission than those taking a higher baseline dose. These results suggest that the kinetics of response to treatment is related to the baseline dose of medication and that this variable needs to be considered when judging response to new therapies. Furthermore, these results provide a benchmark against which the results of newer therapies can be evaluated.

ARTICLE INFORMATION

Correspondence: Dr Bystryn, New York University School of Medicine, 550 First Ave, Tisch Hospital, H313, New York, NY 10016 (bystryn@nyu.edu).

Author Contributions:Study concept and design: Czernik and Bystryn. Acquisition of data: Czernik. Analysis and interpretation of data: Czernik. Drafting of the manuscript: Czernik. Critical revision of the manuscript for important intellectual content: Czernik and Bystryn. Statistical analysis: Czernik. Administrative, technical, and material support: Czernik. Study supervision: Bystryn.

Financial Disclosure: None reported.

Funding/Support: This study was supported in part by US Food and Drug Administration grant 1R01FD-03343-01 (Dr Bystryn).

Additional Contributions: Albert Einstein College of Medicine provided assistance for this study.

Herbst  ABystryn  JC Patterns of remission in pemphigus vulgaris. J Am Acad Dermatol 2000;42 (3) 422- 427
PubMed Link to Article
Bystryn  JCRudolph  JL Pemphigus. Lancet 2005;366 (9479) 61- 73
PubMed Link to Article
National Cancer Institute, Common terminology criteria for adverse events v3.0 (CTCAE). http://ctep.cancer.gov/forms/CTCAEv3.pdf. Accessed June 19, 2007
Mimouni  DAnhalt  GJCummins  DLKouba  DJThorne  JENousari  HC Treatment of pemphigus vulgaris and pemphigus foliaceus with mycophenolate mofetil. Arch Dermatol 2003;139 (6) 739- 742
PubMed Link to Article

Figures

Tables

Table Graphic Jump Location1. Kinetics of Response to Conventional Treatment
Table Graphic Jump LocationTable 2. Relation Between Baseline Prednisone Dose and Kinetics of Response

References

Herbst  ABystryn  JC Patterns of remission in pemphigus vulgaris. J Am Acad Dermatol 2000;42 (3) 422- 427
PubMed Link to Article
Bystryn  JCRudolph  JL Pemphigus. Lancet 2005;366 (9479) 61- 73
PubMed Link to Article
National Cancer Institute, Common terminology criteria for adverse events v3.0 (CTCAE). http://ctep.cancer.gov/forms/CTCAEv3.pdf. Accessed June 19, 2007
Mimouni  DAnhalt  GJCummins  DLKouba  DJThorne  JENousari  HC Treatment of pemphigus vulgaris and pemphigus foliaceus with mycophenolate mofetil. Arch Dermatol 2003;139 (6) 739- 742
PubMed Link to Article

Correspondence

CME
Also Meets CME requirements for:
Browse CME for all U.S. States
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
Please click the checkbox indicating that you have read the full article in order to submit your answers.
Your answers have been saved for later.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.

Multimedia

Some tools below are only available to our subscribers or users with an online account.

515 Views
2 Citations
×

Related Content

Customize your page view by dragging & repositioning the boxes below.

Articles Related By Topic
Related Collections
PubMed Articles
Jobs