Methods: A retrospective chart review was performed of 3 CVID patients with elevated IL-1β.
Results: Case 1 is a 56 year-old female with CVID and Hashimoto’s thyroiditis who developed muscle stiffness, arthralgias and daily fevers (Tmax 104F) which persisted despite intravenous immunoglobulin (IVIG), antibiotics, corticosteroids, NSAIDs and rituximab. An autoinflammatory disorder was suspected when she was found with elevated IL-1β 22.9 pg/mL (nl < 3.9) and responded to anakinra (IL-1 receptor antagonist) with resolution of symptoms and normalization of IL-1β levels.
Case 2 is a 49 year-old male with CVID complicated by incapacitating arthritis and nodular lymphoid hyperplasia of the intestine with malabsorptive diarrhea which improved with IVIG. An elevated IL-1β 68.7 pg/mL was found when he presented with recurrent low grade fevers, myalgias and upper extremity muscle weakness with fasciculations. Anakinra was considered, but symptoms resolved and IL-1β normalized on high-dose IVIG (1.5 grams/kg).
Case 3 is a 26 year-old female with CVID on IVIG and stiff-person’s syndrome with limited improvement on corticosteroids, benzodiazepines and rituximab. She was found with an elevated IL-1β 18.6 pg/ml after complaining of daily flu-like symptoms with fevers (Tmax 101F) which improved with anakinra but her muscle stiffness still persists.
Conclusions: Patients with CVID and autoimmune complications may have altered levels of pro-inflammatory cytokines including IL-1β. They may be responsive to treatment with anakinra or high-dose IVIG.