Granulomatosis with Polyangiitis Presenting as Pauci-Immune Crescentic Glomerulonephritis in Pregnancy

Abstract

Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis rarely affects females of reproductive age. A 28-year-old African American woman presented at 8 weeks of gestation with intractable vomiting attributed to hyperemesis gravidarum. She was found to have acute kidney injury that was unresponsive to vigorous fluid resuscitation and urine sediment examination was suggestive of an underlying glomerulonephritis. Serum c-ANCA and PR3 were elevated and there was no peripheral eosinophilia. During her course she also developed one episode of small volume hemoptysis with right upper lobe infiltrates on CT Chest. There were no cutaneous manifestations of vasculitis or upper respiratory symptoms. Renal biopsy revealed a pauci-immune crescentic glomerulonephritis (PICGN). The diagnosis was consistent with granulomatosis with polyangiitis (GPA). Management initially comprised teratogen sparing agents; steroids, intravenous immunoglobulin; and plasma exchange. The response was suboptimal and she became dependent on daily renal replacement therapy. Ultimately the pregnancy was terminated allowing for traditional treatment approaches with dramatic effect. This is the first case of GPA presenting as PICGN in pregnancy and highlights the challenges of its management.

Successful treatment outcomes in pregnant patients with ANCA‐associated vasculitides: A systematic review of literature

“…Postmortem examination suggested active vasculitis . Nine patients received IVIG with good results . Plasma exchange was offered in 10 instances for life‐ or organ‐threatening disease during or before pregnancy …”

Disease activity assessment of rheumatic diseases during pregnancy: a comprehensive review of indices used in clinical studies

“…In the majority of the studies performed in AAV pregnant patients, disease activity was scored using the Birmingham Vasculitis Activity Score (BVAS) [73] whilst damage due to vasculitis was scored by the Vasculitis Damage Index (VDI) [74]; furthermore, most studies collected the characteristics of the patients (age, type of AAV, organ involvement, ANCA status, ongoing and previous treatment), maternal outcomes (renal function, arterial hypertension, PE), fetal and neonatal outcomes (fetal growth restriction (FGR), prematurity, birth weight, perinatal mortality), type of delivery [75][76][77][78][79][80][81][82][83]. In the majority of reported cases, women with AAV in remission had a favorable maternal and neonatal outcome with quiescent disease or only minor glucocorticoids-dose adjustment; conversely, outcomes were poorer when pregnancies were conceived during active disease or when AAV started during pregnancy [3,[75][76][77][78][79][80][81][82][83][84][85][86]. The most frequent complications were respiratory, cutaneous, articular and renal flares, arterial hypertension, PE, prematurity [3,[75][76][77][78][79][80][81][82][83][84][85][86].…”

“…In the majority of reported cases, women with AAV in remission had a favorable maternal and neonatal outcome with quiescent disease or only minor glucocorticoids-dose adjustment; conversely, outcomes were poorer when pregnancies were conceived during active disease or when AAV started during pregnancy [3,[75][76][77][78][79][80][81][82][83][84][85][86]. The most frequent complications were respiratory, cutaneous, articular and renal flares, arterial hypertension, PE, prematurity [3,[75][76][77][78][79][80][81][82][83][84][85][86]. By using BVAS during pregnancy, great attention should be paid to the evaluation of renal, central nervous system and constitutional symptoms that can be confounded by physiological (e.g.…”

A rare case of coexistence of Wegener’s granulomatosis and pulmonary tuberculosis with subsequent development of thrombosis of the cerebral veins

“…Whilst the health outcome of GPA patients is usually favorable if pharmaceutical therapy (relying on cyclophosphamide and methylprednisolone) is promptly started since the onset of the disease, the subsequent immune-suppression caused by the latter treatment regimen may increase the risk of infectious diseases [10][11][12].…”