علاج داء كاسلمان متعدد المراكز مجهول السبب دون خلل وظيفي حشوي شديد

References

DOI: 10.1182/blood-2018-07-862334

• iMCD patients who are not severely sick are typically diagnosed in the outpatient setting and have a good performance status without evidence of abnormal organ function, whereas other patients are more symptomatic and often exhibit an IL-6–driven inflammatory response that interferes significantly with their ability to function and work.
• Patients should be classified as nonsevere iMCD if the above criteria are not met.
• We recommend (category 1) using anti–IL-6 mAb therapy with siltuximab (11 mg/kg every 3 weeks) for all patients with nonsevere iMCD based on the high proportion of responders, the rigorous nature of the studies underlying the evidence base, and the low side-effect profile relative to other interventions.
• If siltuximab is not available, tocilizumab (8 mg/kg every 2 weeks) may be used (category 2A).
• If needed, first-line therapy with anti–IL-6 mAb should be accompanied by corticosteroid therapy for initial disease control.
• We recommend rituximab (375 mg/m2 × 4-8 doses) as a first-line alternative to anti–IL-6 mAb therapy for patients with nonsevere iMCD who do not have marked cytokine-driven symptomatology based on a more limited data set (category 2B evidence).