Cutting-Edge Strategies for Renal Tumour-like Lesions in Granulomatosis with Polyangiitis: A Systematic Review.

BACKGROUND: Granulomatosis with polyangiitis (GPA) is characterised by granulomatous inflammation and small-to-medium vessel necrotising vasculitis, mainly affecting respiratory tract and kidneys. Renal involvement presenting as tumour-like lesions poses diagnostic and treatment challenges. METHODS: Following the observation of a GPA patient presenting with multiple renal tumour-like lesions, we conducted a systematic literature review on MEDLINE/PubMed, EMBASE, and Cochrane databases. Data gathered from the literature were analysed to summarise the diagnostic approach, management, and outcome of renal GPA-related tumour-like lesions. RESULTS: a 49-year-old female presented with persistent constitutional symptoms and multiple bilateral renal lesions. Renal biopsy showed chronic interstitial inflammation with necrotising granulomas. Laboratory tests disclosed positive anti-proteinase 3 (PR3) anti-neutrophil cytoplasmic antibody (ANCA) leading to a final diagnosis of GPA. She was effectively treated with high-dose glucocorticoids and rituximab. Literature search yielded 41 articles, concerning 42 GPA patients with renal masses, presenting bilaterally in 23.8% of the cases. Positive PR3-ANCA was observed in 86.5% of the cases. Half of 42 patients showed kidney abnormalities. Treatment with glucocorticoids (83.3%) and immunosuppressive agents (80.9%) resulted in an overall good remission rate and favourable prognosis. CONCLUSIONS: GPA should be considered in the differential diagnoses of kidney tumour-like lesions. The diagnosis is challenging, and histological examination greatly contributes to the diagnostic work-up.

Combination of monoclonal antibodies targeting type 2 inflammation for severe asthma and eosinophilic granulomatosis with polyangiitis.

Monoclonal antibodies targeting type 2 inflammation are promising treatments for eosinophilic-associated diseases. There is growing interest in the potential benefits of combining two biologics to treat patients with poorly controlled conditions. We present a case of a 54-year-old female patient affected with a relapsing-refractory ANCA myeloperoxidase positive eosinophilic granulomatosis with polyangiitis (EGPA), presenting with difficult-to-treat asthma and rhino-sinusitis manifestations. She failed several biologics, including omalizumab 300 mg, mepolizumab 100 mg, and benralizumab 30 mg every 8 weeks. A switch to dupilumab led to significant eosinophilia (7.69 × 109/L) as well as systemic symptoms, and a deterioration of asthma control. Therefore, a combination of dupilumab-benralizumab was started, leading to better nasal and ear outcomes, asthma control and decrease in blood eosinophils. During the 12-month treatment, no adverse effects were observed. We conducted an extensive literature search in MEDLINE for original articles published until August 1st, 2023 reporting the combination of anti-type 2 biologics. A total of 51 cases were retrieved from the literature. Omalizumab was the most frequently combined drugs (34 cases). Combination therapy led to reduction of asthma exacerbations and glucocorticoid intake, though was ineffective only for one EGPA patient. Only one patient on omalizumab-mepolizumab therapy reported a mild adverse reaction. Combination biologic therapies for conditions which share pathogenic pathways appears to be both safe and effective. This approach may benefit patients with uncontrolled conditions and counter side effects of biologics, like dupilumab-related hypereosinophilia.