Table 1.
Clinical features of cases describing EGPA and GPA overlap syndrome
Case | Sex/age | GPA | EGPA | ANCA | Treatment | Outcome |
---|---|---|---|---|---|---|
Henochowichz 1986 [20] | 25 F | Arthralgia, pulmonary nodules, purpuric lesions | Rhinitis, renal biopsy-proved necrotizing glomerulonephritis, eosinophilic vasculitis, renal and nose tissue eosinophilia | N/A | CCs, CYC | Remission |
Krupsky 1993 [21] | 43 M | Pulmonary nodules | Marked eosinophilia in the peripheral blood, pleural effusion | c-ANCA | CCs, CYC | Remission |
Potter 1999 Case 1 [22] |
M 29 | Nasal ulcerations, alveolar hemorrhage | Sinus congestion, peripheral eosinophilia, eosinophilic vasculitis | PR3 + | CCs, MTX, trimethoprim/sulfamethoxazole | Remission |
Potter 1999 Case 2 [22] |
F 30 |
Alveolar hemorrhage |
Peripheral eosinophilia, perivascular eosinophilic inflammatory infiltrates, sinus congestion | PR3 + | CCs, MTX, trimethoprim/sulfamethoxazole | Remission |
Lane 2002 Case 1 [23] | N/A | Crusting, Nasal obstruction, sinusitis, lung infiltrates | Renal biopsy proved eosinophilic infiltrate | c-ANCA | N/A | N/A |
Lane 2002 Case 2 [23] | N/A | Crusting, Nasal obstruction, deafness, bloody nasal discharge | Renal biopsy proved eosinophilic infiltrate | c-ANCA, PR3 + | N/A | N/A |
Lane 2002 Case 3 [23] | N/A | Lung infiltrates | Peripheral eosinophilia, renal biopsy proved eosinophilic infiltrate | c-ANCA | N/A | N/A |
Lane 2002 Case 4 [23] | N/A | Pulmonary nodules | Peripheral eosinophilia, Renal biopsy proved eosinophilic infiltrate | c-ANCA, PR3 + | N/A | N/A |
Lane 2002 Case 5 [23] | N/A | Lung infiltrates | Renal biopsy proved eosinophilic infiltrate | c-ANCA | N/A | N/A |
Shoda 2005 [24] | F 25 | Pulmonary hemorrhage, suprascleritis; granulomatous inflammation of the nasal mucosa | Paranasal sinus abnormality, eosinophilia in the blood and around the blood vessels in the biopsy samples; elevated IgE levels | PR3 + | CCs, MTX, CYC | Remission |
Uematsu 2014 [25] | F 78 |
Nasal obstruction with epistaxis; focal panarteritis with necrotizing vasculitis and histiocytic proliferation without eosinophilic infiltration at skin biopsy, microhaematuria |
Peripheral eosinophilia, asthma | PR3 + | CCs, CYC | Remission |
Surendran 2017 [26] | F 45 |
Rapidly progressive renale failure, Proptosis of right eye, saddle nose deformity; diffuse alveolar hemorrhage |
Asthma; renal biopsy proved eosinophilic infiltrate | PR3 + | CCs, CYC | Remission |
Quan 2018 [27] | F 50 | Cavitary pulmonary lesions |
Peripheral neuropathy; long-standing asthma; chronic sinusitis; peripheral eosinophilia; elevated IgE levels, eosinophilic infiltration in the pulmonary parenchyma |
neg |
Anti-IL-5 AZA CCs |
Remission |
N/A not available, MTX methotrexate, CCs steroids, CYC cyclophosphamide, AZA azathioprine