Case Reports in Rheumatology

Case Report

Efficacy of Rituximab in a Systemic Lupus Erythematosus Patient Presenting with Diffuse Alveolar Hemorrhage

Table 1

Demographic features, clinical manifestations, treatment, and outcome in SLE patients presenting with diffuse alveolar hemorrhage (DAH) treated with rituximab.


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)

SLE: systemic lupus erythematosus; IV: intravenous; ANA: anti-nuclear antibodies; NR: not reported; IVIG: intravenous immunoglobulin; NA: not applicable; ENA: extractable nuclear antigen.