Table 1.

Treatment of CVID-GLILD with immunosuppressive therapy

Agent	RTX [17]	RTX  +  AZA [8]	RTX  +  MMF [8, 18]
Number of patients	3	29	15
Regimen used	375 mg/m2 weekly  ×  4, then every 6 months	RTX: 375 mg/m2 weekly  ×  4, then every 4–6 months  +  AZA: 1–2 mg/kg/day	RTX: 375 mg/m2 weekly  ×  4, then every 4–6 months  +  MMF: 250–1000 mg twice daily (N  =  14)
			RTX: 1 g  ×  2 dose 1 month apart  +  MMF: 1–2 g/day (N  =  1)
Duration of follow up	24 months	16 months	16 months
Outcome	Improvement in CT chest and PFT	Improvement in CT chest and PFT, death  =  1, relapse  =  6	Improvement in CT chest and PFT, death  =  1, relapse  =  3
Adverse events	None reported	Reversible hepatotoxicity, intolerance, pneumonia	Pneumonia

AZA azathioprine, MMF mycophenolate mofetil, RTX rituxan