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. 2023 Jan 31;43(3):537–543. doi: 10.1007/s00296-023-05281-x

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