Intractable headache, one of the manifestations of neuropsychiatric systemic lupus erythematosus, is difficult to diagnose and determine an effective treatment. In addition to conventional therapy according to the type of headache, the treatment should be conducted considering the disease activity of systemic lupus erythematosus rather than the headache. We report two patients with intractable headache who were successfully treated using belimumab therapy. The headaches in both patients were relieved after 2 weeks of belimumab administration. The neutralization of B lymphocyte stimulator and reduced production of cytokines from B lymphocytes might contribute to the early effects. The potential benefits of using belimumab as an additional immunosuppressant for treating intractable headache complicated with systemic lupus erythematosus have been discussed.

Spontaneous regression is rare in patients with blastic plasmacytoid dendritic cell neoplasm (BPDCN). An 85-year-old man presented with pancytopenia and skin lesions, and the bone marrow exhibited 79.6% CD4+, CD56+, CD123+, and TCL-1+ abnormal cells, with a normal karyotype; he was thus diagnosed with BPDCN. While being followed without chemotherapy, he was admitted due to sepsis induced by Serratia marcescens, which was successfully treated with antibiotics. Notably, his blood cell counts improved, and the skin lesions disappeared. To our knowledge, this is the first reported case of spontaneous regression of BPDCN with a decrease in tumor cells in the bone marrow following sepsis.

Giant cell arteritis (GCA) mainly involves large-sized arteries, while microscopic polyangiitis (mPA), characterized by pauci-immune necrotizing vasculitis, mainly affects small-sized vessels. We report a very rare concomitant case of GCA diagnosed by temporal artery biopsy and mPA with a high titer of myeloperoxidase antineutrophil cytoplasmic antibody, exacerbation of interstitial pneumonia, and suspected rapidly progressive glomerulonephritis. The patient died by sudden rupture of the gastroepiploic artery (medium-sized vessel), which may have been triggered by GCA and/or mPA.

No abstract

Giant cell arteritis (GCA) mainly involves large-sized arteries, while microscopic polyangiitis (mPA), characterized by pauci-immune necrotizing vasculitis, mainly affects small-sized vessels. We report a very rare concomitant case of GCA diagnosed by temporal artery biopsy and mPA with a high titer of myeloperoxidase antineutrophil cytoplasmic antibody, exacerbation of interstitial pneumonia, and suspected rapidly progressive glomerulonephritis. The patient died by sudden rupture of the gastroepiploic artery (medium-sized vessel), which may have been triggered by GCA and/or mPA.