Patients with HHV-8-negative/idiopathic multicentric
Castleman disease (iMCD) experience systemic inflammatory symptoms and polyclonal lymphoproliferation due to an unknown etiology. Schnitzler's syndrome (SS) is characterized by recurrent urticarial
rash, monoclonal
IgM gammopathy, and other clinical signs of
inflammation. To our knowledge, we report the first case of iMCD associated with SS and the fourth case of
anakinra inducing a complete response for an iMCD patient. A forty-four year old woman with a history of a recurrent urticarial
rash, presented to our hospital complaining of 6 months of night sweats,
fever,
chronic urticaria, iliac bone
pain, and generalized
lymphadenopathy. An
IgM Kappa monoclonal component was measured at 7.8g/L. A lymph node biopsy revealed histopathological features consistent with the plasma cell variant of iMCD. She was diagnosed with SS and iMCD. Anti-IL-1 treatment with
anakinra (100mg/day) was introduced. Within 48h, we observed improvement in the
fever and the urticarial
rash. By one month, we considered the patient
in complete remission. Two years later, the remission is persistent while the patient is still under
therapy. Though this is only the fourth reported case of
anakinra in iMCD, this is yet another case demonstrating the effectiveness of anti-IL-1 blockade in SS. We hypothesize that uncontrolled
cytokine production is responsible for both the SS and the iMCD. The etiologies of SS and iMCD are unknown, and future research is necessary.