Table 3.
Case series when using rituximab therapy in the setting of Goopasture syndrome as a second-line (A) or first-line (B) therapy
| Article | Gender | Age | Clinical findings | Renal injury | Pulmonary symptoms | Creatinine μmol/L (GFR ml/min/1.73m2) | Anti-MBG at diagnosis or at initiation of rituximab | Previous treatment | Rituximab indication | Treatment associated with rituximab | Rituximab | Renal outcome | Pulmonary symptom | Anti-MBG U/ml |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| A. RITUXIMAB AS A SECOND-LINE THERAPY | ||||||||||||||
| Arzoo et al. | F | 73 | hypoxia, haemoptysis, mechanical ventilation, haematuria | no | yes | ND | 51(ELISA) | CYC,PE, CTC | relapse at one year, 2nd treatment failure | CYC PO, corticosteroids | 6 times weekly 375 mg/m2 | recovery | no | undetectable |
| Shah et al. | M | 54 | severe renal failure, anuria, hypertension | yes | yes | 1874 (dialysis dependent) | 680 (U/ml) | CYC IV (500 mg) one dose, PE = 50, corticosteroids | haematological complication of CYC after one dose (on day 5) | PE = 50, corticosteroids | 4 times weekly 375 mg/m2 | dialysis dependent | no | undetectable |
| M | 64 | nasal obstruction, nausea, weight loss, AKI | yes | ND | 536 (dialysis dependent) | 49 (ELISA) | CYC PO (50 mg/d) for 7 days, PE, corticosteroids | haematological complication of CYC after 7 days | PE, corticosteroids | 4 times weekly 375 mg/m2 | dialysis independent after 4th rituximab, creatinine 260 μmol/L | ND | undetectable | |
| Syeda et al. | F | 68 | renal failure, TTP | yes | no | 994(dialysis dependent) | 1/160 (IIF) | CYC (2 mg/kg/d) for 5 days, PE, corticosteroids | haematological complication of CYC after 5 days | PE, corticosteroids | D28, 4 times weekly 375 mg/m2 | dialysis dependent | no | undetectable |
| Touzot et al. | F | 21 | renal failure, pulmonary haemorrhage | yes | yes | GFR 27 | 0 (ELISA) | CYC IV (1000 mg) PE = 13, corticosteroids | severity of disease | corticosteroids | 4 times weekly 375 mg/m2 | GFR 35 | no | undetectable |
| F | 46 | renal failure | yes | no | dialysis dependent | 1/200 (IIF) | CYC IV (1000 mg) PE = 15, corticosteroids | severity of disease, PE dependency | corticosteroids | 4 times weekly 375 mg/m2 | dialysis dependent | no | undetectable | |
| F | 16 | renal failure, haemodynamic instability | yes | no | GFR 105 | 0 (ELISA) | CYC IV (2000 mg) PE = 1,1 corticosteroids | severity of disease (ECMO) | corticosteroids, MMF 720 mg/d | 4 times weekly 375 mg/m2 | GFR 103 | no | undetectable | |
| M | 65 | ND | yes | ND | GFR 17 | 1/40 (IIF) | CYC IV (2000 mg) PE = 9, corticosteroids | persistent anti-GBM antibodies | corticosteroids, MMF 720 mg/d | 4 weekly 375 mg/m2 | GFR 25 | no | undetectable | |
| F | 19 | ND | yes | ND | GFR 29 | 25(ELISA) | CYC IV (1400 mg) PE = 25, corticosteroids | PE dependency | CYC IV (500 mg) PE = 10 corticosteroids | 4 times weekly 375 mg/m2 | GFR 96 | no | undetectable | |
| M | 22 | ND | no | yes | GFR 126 | 19(ELISA) | CYC IV (4500 mg) PE = 0, corticosteroids | relapse at 3 years, alternative therapy | corticosteroids | 4 times weekly 375 mg/m2 | GFR 108 | no | undetectable | |
| F | 17 | ND | yes | yes | GFR 53 | 8 (ELISA) | CYC IV (3000 mg),PE = 0, Corticosteroids | Relapse at 6 months after decrease of corticosteroids | PE = 6,CYC = 60 0 mg ×3, Prednisone | 4 times weekly 375 mg/m2 | Dialysis dependent | no | undetectable | |
| F | 21 | ND | yes | no | GFR 46 | 40(ELISA) | CYC IV (900 mg) PE = 0, corticosteroids | severity of disease | CYC IV (900 mg) PE = 19 corticosteroids | 4 times weekly 375 mg/m2 | GFR 74 | no | undetectable | |
| Sauter et al. | M | 29 | renal failure, pulmonary haemorrhage | yes | yes | 140 | 43 | CYC, PE =17, corticosteroids | relapse at 18 months after transplantation | PE, MMF 3000 mg/d then replaced by CYC PO, corticosteroids | 3 weekly 375 mg/m2 | dialysis dependent, graft lost | no | undetectable |
| Bandak et al. | M | 24 | Haemoptysis haematuria, AKI | yes | no | 379 | 161 | CYC PO (150 mg/d) 6 months, PE = 17, corticosteroids | treatment failure | CYC+ corticosteroids | one dose, 1000 mg | creatinine 181 μmol/L | no | undetectable |
| B. RITUXIMAB USED AS A FIRST-LINE THERAPY | ||||||||||||||
| Wechsler et al. | M | 55 | Haematuria, AKI in HIV, diabetic patient with septic hip arthritis | yes | no | 310 | 8,6 | no | infectious risk | MMF 1000 mgx2/d, corticosteroids IgIV | 4 times weekly 375 mg/m2 | creatinine 106 μmol/L | no | undetectable |
| Shah et al. | M | 17 | nausea, weakness, haemoptysis, weight loss | yes | yes | 272 | 131 | no | fertility | PE = 17, corticosteroids | 2 times weekly 375 mg/m2 | creatinine 99 μmol/L | no | undetectable |
| Narayanan et al. | M | 21 | Oliguria, weakness, oedema, haemoptysis | yes | yes | 1126 | 191 | no | fertility | PE = 5, corticosteroids | 2 doses, two weeks apart | dialysis dependent | no | undetectable |
Abbreviations: ESRD end-stage renal disease, F female, M male, AKI acute kidney injury, IIF indirect immunofluorescence, ANCA anti-neutrophil-cytoplasm antibody, anti-GBM anti-glomerular basement membrane antibody, MMF mycophenolate mofetil, PE plasma exchange, GFR glomerular-filtration rate, CYC cyclophosphamide, IgIV immunoglobulin intravenous, PO per os, IV intravenous, ECMO extracorporeal membrane oxygenation