|
Authors/year of publication | Age, sex, ethnicity | Time from diagnosis of SLE to onset of DAH, years | Cumulative major organ SLE involvement | Previous SLE treatment | Positive serologies at DAH | Treatment | Outcome |
|
Favorable clinical outcome to rituximab therapy |
Gillis et al. [4]/2007 | 24-year-old, female, Pacific Islander | 1 | NR | IV cyclophosphamide Plasma exchange IVIG IV methylprednisolone Azathioprine | NR | Prednisone 60 mg | Survived |
Hydroxychloroquine |
Dapsone |
IV rituximab 375 mg/m2 on day 1, 7, 14, and 21 |
Maintenance: azathioprine |
|
Nellessen et al. [5]/2008 | 29-year-old, female, North African | 12 | Nephritis (class III) | Prednisone IV cyclophosphamide Mycophenolate mofetil | ANA Anti-dsDNA Low C3 Low C4 | IV prednisolone | Survived, no relapse |
IV cyclophosphamide |
Plasma exchange |
Prophylactic antibiotics and antifungals |
Discharged on: prednisolone and cyclosporine |
Relapse: same regimen as during first presentation combined with IV rituximab 500 mg (375 mg/m2) every 2 weeks for 6 weeks |
Maintenance: cyclosporine, mycophenolate mofetil, and prednisolone |
|
Pinto et al. [6]/2009 | 19-year-old, female, Hispanic | 4 | Nephritis (class IV) | NR | NR | IV hydrocortisone | Survived |
IV methylprednisolone pulse |
IV cyclophosphamide |
IV rituximab 375 mg/m2 |
Relapse: IV methylprednisolone pulse |
IV cyclophosphamide |
IV rituximab 375 mg/m2 |
Maintenance: chloroquine and prednisone |
|
Narshi et al. [7]/2009 | 52-year-old, female, NR | 12 | Nephritis (class IV) | IV methylprednisolone IV cyclophosphamide Prednisolone | ANA Anti-dsDNA Low C3 Low C4 | Broad-spectrum antibiotics | Survived, no relapse in 16 months |
IV methylprednisolone pulse |
IV rituximab 1000 mg on day 9 and 25 |
IV cyclophosphamide |
Maintenance: prednisolone |
|
Todd and Costenbader [8]/2009 | 24-year-old, female, Cambodian | 5 | Nephritis (class IV) | Corticosteroids Hydroxychloroquine Azathioprine IV cyclophosphamide IV rituximab 375 mg/m2/week for 4 weeks, 3 courses 6–9 months apart (for nephritis) | ANA Anti-dsDNA Low C3 Low C4 Anti-Ro/La | IV dexamethasone | No relapse, died after 10 months; cause of death unknown |
Empiric antibiotics |
IV methylprednisole pulse |
IV cyclophosphamide |
Plasmapheresis |
Recombinant activated factor VII |
IV rituximab 375 mg/m2 2 infusions, 2 weeks apart |
|
Pottier et al. [9]/2011 | 18-year-old, male, Caucasian | 6 | Nephritis | IV corticosteroids IV cyclophosphamide Mycophenolate mofetil | ANA Anti-dsDNA Low C3 Low C4 | IV methylprednisolone pulse | Survived, no relapse in 15 months |
Mycophenolate mofetil |
Plasma exchange |
Rituximab 715 mg (375 mg/m2) at days 6 and 21 |
Maintenance: prednisolone and mycophenolate mofetil |
|
Martinez-Martinez and Abud-Mendoza [10]/2012 | 23-year-old, female, NR | 4 | Nephritis (class IV) | Methotrexate Prednisone | ANA Anti-dsDNA | IV methylprenisolone pulse | Survived, no relapse in 12 months |
IV cyclophosphamide |
Discharged on: prednisone and mycophenolate mofetil |
Relapse: IV rituximab 2 doses of 1000 mg 2 weeks apart |
Maintenance: prednisone and azathioprine |
|
Gonzalez-Echavarri et al. [11]/2014 | 27-year-old, female, Caucasian | 2 | NR | Hydroxychloroquine | ANA Anti-dsDNA Low C3 Low C4 | IV methylprednisolone pulse | Survived, no relapse in 12 months |
IV cyclophosphamide |
IV rituximab 2 doses of 1000 mg 2 weeks apart |
Hydroxychloroquine |
Prednisone |
Maintenance: prednisone, azathioprine, and hydroxychloroquine |
|
Esper et al. [12]/2014 | 37-year-old, female, NR | NR | NR | Deflazacort | ANA Low C3 Low C4 | IV methylprednisolone pulse | Survived |
Recombinant activated factor VIII |
IV rituximab 500 mg x 1 |
|
Tse et al. [13]/2015 | 52-year-old, female, NR | 8 | Nephritis, S/P renal transplant | Prednisone Hydroxychloroquine Mycophenolate mofetil For renal transplant: IV methylprednisolone Thymoglobulin Tacrolimus | ANA Low C3 | Empiric antibiotics | Survived, no relapse in 6 months |
IV methylprednisolone pulse |
IVIG |
Plasma exchange |
Discharged on: mycophenolate and tacrolimus |
Relapse: IV methylprednisolone pulse |
Plasma exchange |
IVIG |
IV rituximab 550 mg (375 mg/m2) weekly x 3, the 4th dose seven days after discharge |
|
Na et al. [14]/2015 | 37-year-old, female, NR | NR | NR | NR | ANA Anti-dsDNA Anti-Smith Anti-Ro Anti-La | Empiric antibiotics | Survived, no relapse in 4 years |
IV methylprednisolone pulse |
IV rituximab 500 mg (375 mg/m2) on days 3 and 10 |
Maintenance: prednisolone |
|
Aakjær et al. [15]/2017 | 24-year-old, male, Caucasian | 15 | Nephritis (class IV) | Prednisolone IV cyclophosphamide Azathioprine Mycophenolate mofetil | Low C3 | Mycophenolate mofetil | Survived, no relapse in 8 years |
IV rituximab 2 doses of 1000 mg 2 weeks apart |
Relapse: IV rituximab 1000 mg yearly for one year, then every 6 months |
Maintenance: prednisolone, mycophenolate mofetil, hydroxychloroquine, and IV rituximab 1000 mg every 4 months |
|
Current case | 25-year-old, male, Hispanic | 0 | Central nervous system, nephritis | NA | ANA Anti-dsDNA Low C3 Low C4 | IV methylprednisolone pulse | Survived, no relapse in 8 months |
IV cyclophosphamide |
Plasmapheresis |
IV rituximab 375 mg/m2 weekly for 4 weeks |
Maintenance: prednisone and mycophenolate mofetil |
|
Unfavorable clinical outcome to rituximab therapy |
Verzegnassi et al. [16]/2010 | 13-year-old, female, NR | 0.3 | Nephritis | NA | ANA Anti-dsDNA Low C3 Low C4 | Empiric antibiotics | DAH relapse which responded favorably to plasmapheresis |
IV methylprednisolone pulse |
IV cyclophosphamide |
IV rituximab 375 mg/m2 weekly x 3 |
Maintenance: prednisone |
|
Ta et al. [17]/2016 | 16-year-old, female, African American | 0.2 | Myocarditis, nephritis | NA | ANA Anti-dsDNA Low C3 Low C4 | Corticosteroids | Died 25 days after admission |
Plasmapheresis |
IV cyclophosphamide |
IVIG |
IV rituximab (dose not specified) |
|