THERAPEUTIC MODALITIES AND COURSE OD THE DISEASE IN PATIENTS WITH AUTOIMUNE PEMPHIGUS (AP) TREATED AT THE CLINIC OF DERMATOVENEROLOGY IN THE PERIOD 2013-2018.

  • Šućro Madžgalj Medicinski fakultet, Univerzitet u Beogradu
  • Dragana Jakovljevic Medicinski fakultet Beograd
Keywords: Keywords: pemphigus, therapeutic modality, clinical course, side effects

Abstract


Introduction: Autoimmune pemphigus (AP) is one of the most severe dermatologic diseases. AP belongs to the group of organ-specific autoimmune disorders, with autoantibodies directed against cell- surface antigens on keratinocytes, mostly desmogleins. Three main types of AP have been described, depending on the patients' clinical and histologic features and the different target antigens: Pemphigus vulgaris (PV), Pemphigus foliaceus (PF), each with its own subtypes and paraneoplastic pemphigus (PNP). The clinical course and prognosis of AP depend on its subtype, therapeutic modalities and their side effects. The main goal of treatment is to achieve and maintain remission without any systemic therapy or with minimal therapeutic doses (“maintenance therapy”).

The aim: A retrospective study of hospitalized patients with various forms of AP, analysis of clinical course and evaluation of therapeutic modalities employed.

Material and methods: A total of 158 inpatients with diagnosed AP, admitted to the Clinic of Dermatovenereology, Clinical Centre of Serbia, from 2013 to 2018, with a subsequent follow-up of 6 to 12 months, were included in this study. Demographic and clinical data as well as treatment approaches were analyzed using descriptive and analytic statistics.

Results: The most common subtype encountered was PV (72.15%); in the largest number of patients (46.84%) the disease occurred between ages 39-59. Conventional corticosteroid treatment was most frequently used (67.41%), along with azathioprine (66.86%) as the most common immunosupressive drug. In 60.8% of patients no side effects or disease complications were recorded. During the follow-up, at the end of 2018, 48.1% of patients achieved remission

Conclusion: We have shown a high incidence of PV. Treatment approaches used in our patients were in accordance with the pemphigus subtype and course of the disease. A significant number of patients achieved clinical remission.

References

1. Joly P, Litrowski N. Pemphigus group (vulgaris, vegetans, foliaceus, herpetiformis, brasiliensis). Clin Dermatol 2011; 29: 432-6.
2. Ioannides D, Lazaridou E, Rigopoulos D. Pemphigus. J Eur Acad Dermatol Venereol 2008; 22: 1478-1496.
3. Ruocco V, Ruocco E, Lo Schiavo A, Brunetti G, Guerrera LP, Wolf R. Pemphigus: etiology, pathogenesis, and inducing or triggering factors: facts and controversies. Clin Dermatol 2013; 31; 374-381.
4. Brenner S, Goldberg I. Drug-induced pemphigus. Clin Dermatol 2011; 29: 455-457.
5. Murrell DF, Dick S, Ahmed AR, et al. Consensus statement on definitions of disease, end points, and therapeutic response for pemphigus J Am Acad Dermatol. 2008; 58: 1043–1046
6. Mimouni D, Nousari CH, Cummins DL, Kouba DJ, David M, Anhalt GJ. Differences and similarities among expert opinions on the diagnosis and treatment of pemphigus vulgaris, J Am Acad Dermatol 2003; 49: 1059–1062
7. Manson SC, Brown RE, Cerulli A, Vidaurre CF. The cumulative burden of oral corticosteroid side effects and the economic implications of steroid use. Respir Med 2009; 103: 975–994.
8. Meurer M. Immunosuppressive therapy for autoimmune bullous diseases. Clin Dermatol 2012; 30: 78-83.
9. Vyas N, Patel NS, Cohen GF. Mycophenolate mofetil as a first-line steroidsparing agent in the treatment of pemphigus vulgaris. J Drugs Dermatol 2013; 12: 210–216.
10. Kasperkiewicz M, Schmidt E, Zillikens D. Current therapy of the pemphigus group. Clin Dermatol 2012; 30: 84-94.
11. Chams-Davatchi C, Mortazavizadeh A, Daneshpazhooh M, et al. Randomized double blind trial of prednisolone and azathioprine, vs. prednisolone and placebo, in the treatment of pemphigus vulgaris, J Eur Acad Dermatol Venereol 2013; 27: 1285–1292.
12. Olszewska M, Kolacinska-Strasz Z, Sulej J, et al. Efficacy and safety of cyclophosphamide, azathioprine, and cyclosporine (ciclosporin) as adjuvant drugs in pemphigus vulgaris, Am J Clin Dermatol 2007; 8: 85–92.
13. Kawakami T, Koga H, Saruta H, et al. Four mild but refractory cases of pemphigus foliaceus successfully treated with intravenous immunoglobulin. J Dermatol 2013; 40: 869–873.
14. Tavakolpour S. Current and future treatment options for pemphigus: Is it time to move towards more effective treatments? Int Immunopharmacol 2017; 133-142.
15. Joly P, Maho-Vaillant M, Prost-Squarcioni C, et al. First- line rituximab combined with short- term prednisone versus prednisone alone for the treatment of pemphigus (Ritux 3): a prospective, multicentre, parallel-group, open-label randomised trial. Lancet 2017; 389: 2031–2040.
16. Scully C, Challacombe SJ. Pemphigus vulgaris: update on etiopathogenesis, oral manifestations, and management. Crit Rev Oral Biol Med 2002; 13: 397–408.
17. Pisanty S, Sharav Y, Kaufman E, Posner LN. Pemphigus vulgaris: incidence in Jews of different ethnic groups, according to age, sex, and initial lesion. Oral Surg Oral Med Oral Pathol 1974; 38: 382–387.
18. Tsankov N, Vassileva S, Kamarashev J, et al. Epidemiology of pemphigus in Sofia, Bulgaria. A 16-year retrospective study (1980–1995). Int J Dermatol 2000; 39: 104–108.
19. Kanwar AJ, Kaur S, Thami GP. Long-term efficacy of dexamethasone-cyclophosphamide pulse therapy in pemphigus. Dermatology 2002; 204: 228-31.
20. Saha M, Powell AM, Bhogal B, Black MM, Groves RW. Pulsed intravenous cyclophosphamide and methylprednisolone therapy in refractory pemphigus. Br J Dermatol 2010; 162:790-7
21. Murrell DF, Peña S, Joly P, et al. Diagnosis and Management of Pemphigus: recommendations by an International Panel of Experts. J Am Acad Dermatol 2018, doi: 10.1016/j.jaad.2018.02.021.
22. Harman KE, Brown D, Exton LS, et al. British Association of Dermatologists’ guidelines for the management of pemphigus vulgaris 2017. Br J Dermatol 2017; 177: 1170-1201.
Published
2022/01/11
Section
Original Scientific Paper