Ghid de tratament al vasculitelor primare sistemice

5.1.1. Imunoglobulina intravenos : 2g/kg timp de 5zile. Înaintea administrării lor, concentraţiile serice ale imunoglobulinelor trebuie să fie determinate, deoarece pacienţii cu deficit selectiv de IgA pot dezvolta o reacţie anafilactică la primirea imunoglobulinelor intravenoase (IVIG) sau o hiper-γ-globulinemie pre-existentă se poate agrava care va duce la o stare de hipervâscozitate

5.1.2. Mycofenolat mofetil : 2g/zi

Utilizat in studii ce vizau mentinerea remisiei (68-70), mycophenolate mofetil este una din optiunile terapiei remisive alternative in boala refractara.

5.1.3. Terapia biologica (infliximab, rituximab) poate reprezenta o solutie pentru boala refractara sau recidivanta

Medicament	Doză	Referinţă
Imunoglobulină intravenos	2g/kg timp de 5 zile	Muso et al, Jayne et al
15-Deoxyspergualin	0,5mg/kg/zi până când valoarea cea mai mică a leucocitelor este de 3000/μl, apoi se va aştepta până când leucocitele ajung la ≥4000/ μl şi repetaţi doza 6 cicluri	Burke et al
Globulina anti-timocite	2,5mg/kg/zi pentru 10 zile, doză ajustată în funcţie de numărul leucocitelor: fără globulina anti-timocite dacă L<150/ μl, 1,5mg/kg/zi daca L=150-300/ μl şi doza maximă dacaă L>300/ μl	Schmitt et al
Infliximab	3-5mg/kg/perfuzie la fiecare 1-2 luni	Booth et al
Mycophenolat mofetil	2g/zi	Koukoulaki et al Stassen et al
Rituximab	375mg/m^2 SC săptămânal pentru 4 săptămâni	Keogh et al, Keogh et al, Stasi et al, Brihaye et al, Eriksson et al

IV. Tratamentul PAN cu hepatita B asociata

Glucocorticoizii, terapia antivirala si plasmafereza reprezinta terapia combinata recomandata in PAN si infectie virala B asociata capabila sa induca remisiunea bolii. (nivelul 3 de dovezi , gradul de recomandare C) (35). Există date limitate privind utilizarea rituximab în cazurile refractare. (71). Tratamentul acestor afecţiuni trebuie să fie stabilit împreună cu un medic hepatolog.

V. Tratamentul in vasculita crioglobulinemică esenţială mixtă (non-virală) nu beneficiaza de studii clinice. Consensul comisiei EULAR este că această boală ar trebui să fie tratată în acelaşi mod ca şi celelalte vasculite ale vaselor mici, discutate în aceste recomandări (GW, MPA şi CSS), cu agenţi imunomodulatori şi glucocorticoizi (nivel de dovezi 4, gradul de recomandare D) (35). Rituximabul utilizat la pacienţii cu vasculită crioglobulinemică cu hepatită C asociată ar putea fi deasemenea util în vasculitele esenţiale crioglobulinemice non-viral asociate. (71)

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