Prepubertal-Onset Vulvar Lichen Sclerosus: The Importance of Maintenance Therapy in Long-Term Outcomes

2015 ◽  
Vol 32 (4) ◽  
pp. 461-467 ◽  
Author(s):  
Elizabeth Ellis ◽  
Gayle Fischer
2010 ◽  
Vol 106 (1) ◽  
pp. 56-60 ◽  
Author(s):  
T.V. Parran ◽  
C.A. Adelman ◽  
B. Merkin ◽  
M.E. Pagano ◽  
R. Defranco ◽  
...  

2020 ◽  
Vol 22 (1) ◽  
pp. 99-114
Author(s):  
Alessandro Borghi ◽  
Monica Corazza

Background: Vulvar Lichen Sclerosus (VLS) is a chronic inflammatory disease with a huge impact on a person’s quality of life. A correct therapy is required for relieving symptoms, reversing signs and preventing further anatomical changes. Objective: The main objective of the present paper is to provide suggestions for the best treatment approach, based on the available evidence. Treatment strategies are divided on the basis of the treatment phase, distinguishing options for initial, acute or attack treatment and those for long-term, maintenance treatment. Methods: An electronic search was performed using the National Library of Medicine PubMed database. All the studies evaluating treatment of vulvar lichen sclerosus published in the English literature were analyzed, including controlled studies, case series, guidelines and reviews. Results: Current evidence identifies ultra-potent and potent corticosteroids, administered for 12 weeks, as the first-line recommended treatment for active VLS. Topical calcineurin inhibitors, tacrolimus and pimecrolimus, are effective and safe alternatives. Long-term maintenance strategies aimed at preventing recurrences are required, after the initial treatment phase. Maintenance treatment mostly consists in topical corticosteroids, administered i) on an “as needed” basis (“reactive” scheme), ii) on a continuative regimen, iii) on a low-dose, intermittent regimen (“proactive” scheme). Further investigations are needed for better defining the placement of other options within the VLS therapeutic algorithm, including retinoids, physical and systemic treatments. Conclusion: The available evidence provides useful indications for the management of VLS. Both the identification of new therapeutic targets and the optimization of the available options represent the main objectives of future research.


Rheumatology ◽  
2017 ◽  
Vol 56 (10) ◽  
pp. 1684-1693 ◽  
Author(s):  
Hubert de Boysson ◽  
Jean-Jacques Parienti ◽  
Caroline Arquizan ◽  
Grégoire Boulouis ◽  
Nicolas Gaillard ◽  
...  

Lupus ◽  
2020 ◽  
Vol 29 (8) ◽  
pp. 845-853
Author(s):  
Narayan Prasad ◽  
Jithu Kurian ◽  
Vikas Agarwal ◽  
Dharmendra Bhadauria ◽  
Manas Behera ◽  
...  

Introduction Lupus nephritis (LN) has a considerable impact on the morbidity and mortality of systemic lupus erythematosus (SLE) patients. Long-term comparative outcome data from the Indian subcontinent on treatment regimens with cyclophosphamide (CYP) and mycophenolate mofetil (MMF) are sparse. We assessed renal and patient survival for these patients in terms of the types of induction – CYP or MMF – and the two maintenance therapies – MMF or azathioprine (AZA). Methods We retrospectively analysed outcomes of 100 LN patients, 67 treated with CYP (26 class III, 25 class IV, 6 class III + V and 10 class IV + V; 40 Euro lupus regimen and 27 National Institutes of Health regimen) and 33 treated with a MMF-based regimen with steroids between July 2008 and June 2018. Data regarding demographic, clinical and histopathological features and the treatment given to all patients were extracted. Outcomes between the two regimens CYP and MMF were compared in terms of remission, dialysis and patient survival. Results The clinical characteristics were similar in both groups, except that the activity index was higher in CYP patients (6.13 ± 4.48 vs. 4.61 ± 2.80). However, the chronicity index was similar. The overall remission rate was 70% at the end of induction. The rates of complete remission, partial remission and non-responders in the CYP group were 46.2%, 23.9% and 29.9%, respectively. However, in the MMF group, the corresponding rates were 57.6%, 12.1% and 30.3%, respectively. The 1-, 2-, 3-, 4-, 5- and 10-year patient survival rates in the CYP group were 89.5%, 86.2%, 86.2%, 83.8%, 83.8% and 83.8%, respectively. In the MMF induction group, the corresponding rates were 93.9%, 93.9%, 89%, 89%, 89% and 89%, respectively. At the end of the study, rates of end-stage renal disease in the MMF group and CYP group were 7.5% and 12.1%, respectively. The death-censored and non-censored renal survival rates were also similar in the long term. With regard to maintenance therapy, 3/56 (5.3%) in the MMF group and 7/34 (20.5%) in the AZA group experienced doubling of serum creatinine ( p = 0.03). Conclusions Long-term outcomes in terms of patient and renal survival of LN patients treated with CYP and MMF induction are similar. Doubling of serum creatinine occurred more with AZA-based maintenance therapy than with MMF-based maintenance therapy. Most deaths occurred during induction, and sepsis was the most common cause of death.


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