Clinicopathological features of graft versus host disease-associated myositis.

BACKGROUND AND OBJECTIVE: Chronic graft versus host disease (GVHD)-associated myositis targeting skeletal muscle is a relatively rare but potentially debilitating complication following allogeneic hematopoietic stem cell transplantation (HSCT). We reviewed the clinicopathological features of GVHD-associated myositis among patients receiving allogeneic HSCT to elucidate the cellular pathogenesis. METHODS: We retrospectively reviewed clinical data and muscle biopsy results from 17 consecutive patients diagnosed with GVHD-associated myositis at our institution between 1995 and 2019. Immunostaining findings of GVHD-associated myositis were compared to those of patients with anti-tRNA-synthetase antibody-associated myopathy (ASM) (n = 13) and dermatomyositis (DM) (n = 12). RESULTS: The majority of patients with GVHD-associated myositis showed subacute or chronic progression of mild to moderate limb weakness together with elevated serum creatine kinase. These patients also exhibited mild C-reactive protein elevation but were negative for myositis-related autoantibodies. Programmed death-1 (PD-1)-positive cells were observed in muscle interstitium adjacent to myofibers expressing human leukocyte antigen (HLA)-DR. The interstitium was also HLA-DR-positive, similar to biopsy samples from ASM patients but not DM patients. The proportions of HLA-DR-positive muscle fibers and PD-1-positive interstitial cells were significantly higher in GVHD and ASM samples than DM samples. The PD-1-positive cells were mostly CD-8-positive lymphocytes. DISCUSSION: GVHD-associated myositis is characterized by HLA-DR-positive myofibers and infiltration of PD-1-positive lymphocytes. These features distinguish GVHD-associated myositis from DM but not from ASM.

Total hip arthroplasty for a patient with angel-shaped phalango-epiphyseal dysplasia (ASPED). A case report.

Angel-shaped phalango-epiphyseal dysplasia is characterized by the angel shape of the middle phalanx and severe coxarthrosis in adult life. This osteochondrodysplasia shows a further variety of heterogeneous multiple epiphyseal dysplasias. It also shows a late and dysplastic development of the femoral head that leads to osteoarthrotic changes with severe hip pain and gait disturbance. In this report, we show a 35-year-old female with Angel-shaped phalango-epiphyseal dysplasia that was treated by bilateral total hip arthroplasty. She has suffered from her coxalgia since she was 27 and since the age of 30 has been able to walk with the aid of crutches. The radiographs of her bilateral hip showed severe osteochondrotic changes with a progressive disappearance of the joint space. Total hip arthroplasties were performed to treat the osteoarthrosis on the bilateral hip joint due to Angel-shaped phalango-epiphyseal dysplasia. One year after surgery, she is pain-free, and able to walk with a cane. Based on this case, we propose that total hip arthroplasty should be considered one of the treatments for the coxopathy in patients with Angel-shaped phalangoepiphyseal dysplasia.