Page 71 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

Overview of Primary and Secondary Vasculitides 47

[69] and Jones [218] and co-investigators demonstrated the superiority and safety of
rituximab. Although 75 to 90% of patients with GPA and other AAV achieve remission with
oral or intravenous cyclophosphamide, few data are available on therapeutic strategies for
patients with disease refractory to this first-line therapy.

Table 3. Recommendations for the Treatment of Vasculitides in the Literature

LARGE VESSEL VASCULITIS
GCA, TAK: CS [15, 230], AZA [18], RTX [208], infliximab [231], anti-TNF?, anti-IL-6R [232, 233],
tocilizumab [234]; and MM [235].
Adjunctive therapy: ASA [236] and AC [237].
MEDIUM VESSEL VASCULITIS
PAN, KD: CS and CYC [31-33]; MM [49].
SMALL VESSEL VASCULITIS-AAV Type
GPA, EGPA, MPA: Induction with CS + CYC [50, 238, 239], CS + RTX [69, 218, 240-243], or CS + MM
[244] and maintenance with RTX [63-68], AZA [69-71, 226], or MM [224-226].
SMALL VESSEL VASCULITIS- IC Type
CV: MM [247]; INF-alpha [108, 246, 247] and PegINF-alpha plus ribovarin [108, 111] or RTX [248] in
HCV-associated MC.
IgAV: CS [249] and [250] or MM [251]; and supportive care.
Hypocomplementic-C1q: Antihistamines, IVIg, PE.
VARIABLE VESSEL VASCULITIS
Cogan Syndrome: CS [252].
BD: CS [253], MM [254]; colchicine or anti-TNF? [255].
SINGLE ORGAN VASCULITIS-ISOLATED AORTITIS, PACNS
Isolated Aortitis: CS [208, 209], AZA, MM, MTX [209]
PACNS: Induction with CS [184, 187, 196, 256] or CS +CYC [184, 187, 190, 196, 257]; [187, 188, 190,
257], followed by maintenance with AZA [196], MTX or MM [258]
VASCULITIS ASSOCIATED WITH SYSTEMIC COLLAGEN VASCULAR DISEASE-SLE, RAV
SLE: CS [116]; MM [259]; and AC [123]
RAV: CS [141], RTX [143, 260], infliximab [145, 146], and AZA or MTX [261]
VASCULITIS ASSOCIATED WITH ILLICIT SUBSTANCE ABUSE
Avoid illicit substance.
VASCULITIS ASSOCIATED WITH INFECTION
Antimicrobial agents chosen specifically to treat a given etiologic organism.
Abbreviations: AC, anticoagulation; ASA, aspirin; AZA, azathioprine; BD, Behçet disease; CS,

corticosteroids, CV, cryoglobulinemic vasculitis; CYC, cyclophosphamide; EGPA, eosinophilic
granulomatosis with polyangiitis; GCA, giant cell arteritis; GPA, granulomatosis with polyangiitis;
HCV, hepatitis C virus; IC, Immune complex; IgAV, IgA vasculitis; INF, interferon; IL,
interleukin; IVIg, intravenous immune globulin; KD, Kawasaki disease; MC, mixed
cryoglobulinemia; MM, mycophenolate mofetil; MPA, microscopic polyangiitis; MTX,
methotrexate; PACNS, primary angiitis of the central nervous system; PAN, polyarteritis nodosa;
PE, plasma exchange; RAV, rheumatoid arthritis vasculitis; RTX, rituximab; SLE, systemic lupus
erythematosus; TAK, Takayasu arteritis; TNF, tumor necrosis factor.

Its favorable effect on vasculitis derives from the preferential T-cell lysis resulting from
the inhibition of hematopoietic precursors in the bone marrow, leaving stem cells unharmed.
At high doses, this inhibition favors repopulation of the marrow and thus the cellular immune
system. After an intravenous dose of cyclophosphamide, the nadir of peripheral leucopenia,
which corresponded with peak marrow suppression, occurred in 7 to 18 days. Less than 20%
of labeled cyclophosphamide is excreted unchanged in the urine. The toxic side effects

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