ANCA-associated vasculitis has recurred, how to deal with it? Latest roundup

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Standard care for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) consists of two phases, starting with glucocorticoids (GC) plus cyclophosphamide or rituximab to induce remission, followed by remission maintenance therapy to prevent relapse
.
Still, there is a significant risk of
relapse.
This article discusses new strategies for the prevention and treatment of AAV recurrence, with a focus on granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA).

Evaluation for suspected recurrence of AAV

AAV has a tendency to spontaneously recur, sometimes severe and potentially organ-endangering or life-threatening
.
There is currently insufficient evidence to predict episodes or recurrence, and there are no reliable indicators to guide risk stratification
of recurrence.
Therefore, ongoing and regular clinical monitoring of patients with AAV after remission induction is recommended for early detection of recurrence, when symptoms and signs are generally milder
than when relapse is confirmed.

For patients with suspected recurrence of AAV, the clinical manifestations need to be determined first, then the diagnosis should be clarified, the differential diagnosis should be excluded, and the severity of the recurrence should be determined, and the corresponding treatment plan
should be formulated.
Once relapse is diagnosed, its severity must be thoroughly assessed for organ or vital manifestations
.
About one-third of relapses are severe conditions
that affect the kidneys and patient survival.

To help clinicians determine the best treatment strategy and avoid overtreatment, a five-factor score (FFS) can be used to assess patient outcomes
.
Five factors include proteinuria > 1 g/day, renal insufficiency with serum creatinine >1.
58 mg/dL, gastrointestinal involvement, cardiomyopathy, and central nervous system involvement
.
The higher the FFS score, the worse
the prognosis.

Principles of treatment of recurrence of AAV

Patients with relapse should follow the same principles of treatment as newly diagnosed patients, taking into account several factors: classification of disease, expected outcome, severity, previous treatment, comorbidities, and age
.
Its management includes rapid initiation of therapy to avoid organ damage and early death, followed by maintenance remission therapy
with less toxic immunosuppressive regimens.

Treatment with recurrence of organ-threatening or life-threatening manifestations of AAV

For recurrence of AAV with organ-threatening or life-threatening manifestations, it is recommended to reintroduce or intensify the GC dose in conjunction with cyclophosphamide or rituximab
.
Rituximab is preferred, in contrast, with a better response to treatment in patients with recurrent disease
.
After completion of induction with cyclophosphamide, maintenance therapy with cyclophosphamide should be spaced 2-4 weeks
apart, regardless of the choice of drug maintenance therapy.
Cyclophosphamide can be taken orally, but oral administration is not recommended as first-line therapy because oral administration is more likely to cause adverse effects
than infusion.