Rituximab (RTX) is a chimeric anti-CD20 antibody, approved for
rheumatoid arthritis (RA) patients who failed anti-
Tumor Necrosis Factor therapy. It has been used occasionally for life-threatening
autoimmune diseases (AID). We report our center experience in the use of RTX in life-threatening complications or refractory AID. Clinical charts of patients treated with RTX at our center were reviewed, cases treated for life-threatening complications or refractory AID were analyzed. Acute damage to vital organs such as lung, heart, kidney, nervous system with severe functional impairment were defined as life-threatening complications; treatment failure with high-dose
corticosteroids,
cyclophosphamide,
IVIG,
plasmapheresis was defined as refractory
autoimmune disease. During the years 2003-2009, 117 patients were treated with RTX, most of them for RA. Nine patients (6 females, mean age 51.5 years, mean disease duration 6.3 years) answered the criteria. The indications were as follows: pulmonary
hemorrhage (1 patient with cryoglobulinemic
vasculitis, 1 with
systemic sclerosis, 1 with
ANCA-associated vasculitis), catastrophic
anti-phospholipid syndrome (2 SLE patients), non-
bacterial endocarditis and
pulmonary hypertension (1 patient with
mixed connective tissue disease),
vasculitis and feet
necrosis (1 patient with
systemic lupus erythematosus), severe lupus demyelinative neuropathy and
acute renal failure (1 patient), and severe rheumatoid
lung disease with recurrent
empyema and
pneumothorax (1 patient). B cell depletion was achieved in all patients. The median time since starting of complications to RTX administration was 3 weeks (range 2-15 weeks). Complete remission (suppression of the hazardous situation and return to previous stable state) was seen in 7 out of 9 patients. Partial remission (significant improvement) was achieved in the remained. The median time to response was 3 weeks (range 1-8 weeks), mean follow-up 47.2 months (range 6-60 months). A rapid tapering off of
steroids was achieved in all patients. Two patients relapsed and were successfully retreated with RTX: the patient with severe RA lung relapsed after 3 years, one of the patients with
ANCA-associated pulmonary alveolar
hemorrhage relapsed after 10 months. There were no side effects during RTX infusion. Two episodes of serious
infections were registered: fatal Gram-negative
sepsis 6 months after RTX treatment, and septic
discitis 4 months after receiving RTX. RTX serves as a safe, efficient, and prompt rescue
therapy in certain life-threatening conditions and resistant to aggressive immunosuppression AID. RTX when administrated at an earlier stage, prevented irreversible vital organ damage, and allowed rapid
steroid tapering off in already severe immunodepressed patients.