Intracranial vascular stenosis in giant cell arteritis successfully treated by two balloon angioplasty procedures.

Intracranial vascular stenosis is rarely associated with giant cell arteritis (GCA), and the prognosis for stroke caused by GCA is poor. Despite its unfavourable outcome, the strategy to manage this involvement and the indication of endovascular treatment are not well defined in the latest guidelines or recommendations. Here, we present a case in a 68-year-old woman, which was refractory to medical therapy, but successfully treated by two balloon angioplasty procedures. She was admitted to our department with lower extremity stiffness and left visual disturbance. GCA was clinically diagnosed by the wall thickening of the temporal artery and the aorta. Hemiparesis and motor aphasia developed shortly after intravenous methylprednisolone pulse therapy, and magnetic resonance imaging revealed acute cerebral infarction with severe stenosis at the end of the left internal carotid artery. Balloon angioplasty was tried initially with improvement in her symptoms and additionally performed to treat restenosis without any significant adverse events. Her symptoms markedly improved with no recurrence until 8 months after discharge. We also review 10 similar cases reported in the literature. Although further evidence is needed to confirm the usefulness and safety of balloon angioplasty for intracranial GCA, this case report provides valuable information about the endovascular therapy for GCA.

A Rare Case of Pembrolizumab-Induced Dermatomyositis in a Patient with Cancer of Unknown Primary Origin.

BACKGROUND Pembrolizumab is a humanized monoclonal antibody against programmed cell death-1 protein. Pembrolizumab sometimes causes immune-related adverse events (irAEs). Dermatomyositis is a rare irAE of immune checkpoint inhibitors. The presentation is usually acute, and symptoms include edema with erythema of the eyelids, erythema of the forehead, and muscle weakness in both thighs. CASE REPORT Here we report a case of pembrolizumab-induced dermatomyositis in a 71-year-old Japanese woman with cancer of unknown primary origin, who experienced a high fever and had difficulty walking after her sixth course of pembrolizumab. General physical examination revealed edema with a heliotrope rash, V-neck signs, and nonspecific erythema of the forehead. Laboratory evaluation revealed that myogenic enzymes were within normal ranges. Autoantibody tests revealed that antinuclear antibodies were negative, and autoantibodies related to myositis and anti-acetylcholine receptor antibodies were also negative. A magnetic resonance imaging scan of the thighs revealed signal abnormalities in the left lateral and distal vastus medialis muscle. The patient was treated with corticosteroids, subsequently followed by intravenous immunoglobulin therapy, which led to an improvement of the symptoms. CONCLUSIONS Pembrolizumab-induced dermatomyositis is rare. Corticosteroids have been administered in many cases, and this case also suggests the efficacy of intravenous immunoglobulin therapy in treating immune checkpoint inhibitor-related dermatomyositis. This case highlights practical management of pembrolizumab-induced dermatomyositis.

Nintedanib and intensive immunosuppressive therapy to treat rapidly progressive interstitial lung disease presenting anti-ARS antibodies.

We describe a case of fulminant onset, rapidly progressive-interstitial lung disease (RP-ILD) with anti-ARS antibodies (anti-PL-7). The patient was successfully treated with nintedanib in addition to intensive immunosuppressive therapies, including intravenous cyclophosphamide. Nintedanib has just been approved for treatment of progressive fibrosing ILD, but to date, no reports of RP-ILD treated with nintedanib have been published. This case report may advance discussions regarding the use and timing of nintedanib in treating RP-ILD.