Madan Ujjwal, Goel Vishesh, Shah Jignesh, Ahmad Hameed, Cassol Clarissa, Edrees Amr
Division of Internal Medicine, University of Missouri, Kansas City, MO, 64108, USA.
Division of Internal Medicine, Lady Hardinge Medical College, New Delhi, 110001, India.
CEN Case Rep. 2025 Aug;14(4):567-572. doi: 10.1007/s13730-024-00959-y. Epub 2024 Dec 20.
Granulomatosis with polyangiitis is an ANCA-associated vasculitis that involves small to medium-sized vessels. The extent of renal involvement varies, which is also associated with disease prognosis, with aggressive renal involvement having worse outcomes. Rapidly progressive glomerulonephritis with severe inflammatory features and extensive crescent formation can be challenging to treat. Usually, induction regimes utilize a combination of pulse dose methylprednisolone followed by rituximab or cyclophosphamide. Resistant diseases pose additional treatment challenges, and individualized treatment regimens have been described without accumulated outcome data. Cyclophosphamide, rituximab, azathioprine, methotrexate, and mycophenolate with or without plasmapheresis have been variably used, but there is a lack of consensus on a standardized regime in literature. Our case adds to the existing literature on the treatment-refractory granulomatosis with polyangiitis, which was treated with high-dose corticosteroid in combination with rituximab, low-dose cyclophosphamide, plasmapheresis, and brief use of hemodialysis. It also reiterates that the use of a variety of low-dose cyclophosphamide with rituximab could be beneficial for treatment-refractory cases or patients with severe renal involvement, in addition to better tolerance with low dose cyclophosphamide in comparison with full-dose cyclophosphamide.
肉芽肿性多血管炎是一种与抗中性粒细胞胞浆抗体（ANCA）相关的血管炎，累及小到中等大小的血管。肾脏受累程度各异，这也与疾病预后相关，严重的肾脏受累预后较差。具有严重炎症特征和广泛新月体形成的急进性肾小球肾炎治疗颇具挑战性。通常，诱导治疗方案采用静脉注射甲泼尼龙冲击剂量后联合利妥昔单抗或环磷酰胺。难治性疾病带来了额外的治疗挑战，虽然已有个体化治疗方案的描述，但尚无累积的疗效数据。环磷酰胺、利妥昔单抗、硫唑嘌呤、甲氨蝶呤、霉酚酸酯，无论是否联合血浆置换，均有不同程度的应用，但文献中对于标准化治疗方案尚无共识。我们的病例补充了关于难治性肉芽肿性多血管炎的现有文献，该病例采用大剂量糖皮质激素联合利妥昔单抗、小剂量环磷酰胺、血浆置换及短期血液透析进行治疗。这也再次强调，对于难治性病例或严重肾脏受累的患者，使用多种低剂量环磷酰胺联合利妥昔单抗可能有益，此外，与全剂量环磷酰胺相比，低剂量环磷酰胺耐受性更好。
