Trial of canakinumab, an IL-1β receptor antagonist, in patients with inclusion body myositis.

Objective: To assess whether canakinumab, a monoclonal antibody against IL-1β approved for autoinflammatory diseases, is effective as target-specific therapy in patients with sporadic inclusion body myositis (sIBM).

Methods: Because in sIBM IL-1β colocalizes with amyloid precursor protein and upregulates amyloid aggregates enhancing degeneration, targeting IL-1β with canakinumab may arrest disease progression. On this basis, 5 ambulatory patients with sIBM participated in an institutional review board--approved open-labeled study with 150 mg canakinumab [4 bimonthly, then monthly subcutaneous injections] for a mean period of 15.

Cutaneous ulcers: An unusual manifestation of inherited thrombophilia.

We report two cases with cutaneous lesions found to be associated with factor V Leiden mutation and low S protein levels. At the time of presentation, no other symptoms were reported. Histopathology of both lesions revealed the presence of widespread intravascular thrombi.