Muscular pathological features and morphological analysis of facioscapulohumeral muscular dystrophy / 中国医师杂志

ABSTRACT

Objective:To analyze the pathological features of facioscapulohumeral muscular dystrophy (FSHD). For better characterization of inflammatory response in FSHD, we performed histochemical morphological analysis for FSHD and polymyositis (PM) muscle biopsies.In order to provide a reference for guide targeted therapeutic interventions.Methods:The clinical and myopathological data of 30 patients with FSHD from January 2006 to January 2019 in the Sixth Hospital of Shanxi Medical University and the first hospital of Jilin University were retrospectively analyzed. The patients were divided into non-inflammatory infiltration group (16 cases) and inflammatory infiltration group (14 cases) according to the presence or absence of inflammatory cell infiltration. For better characterization of inflammatory response in FSHD, we performed histochemical morphological analysis for two groups of muscles: FSHD and PM muscle biopsies, using Image-Pro plus bioanalytical software.Results:In 30 cases of FSHD, 14 cases showed intramuscular and interstitial inflammatory cell infiltration, especially around the blood vessels. Immunohistochemical staining confirmed that the infiltration of inflammatory cells was mainly CD4 + T lymphocytes. Morphometric analysis showed that there were no significant differences in muscle fiber surface area, density, diameter, inflammatory cell infiltration, regeneration and necrosis between FSHD patients and PM patients ( P>0.05). The total area of myointerstitium in FSHD group was significantly larger than that in PM group ( P=0.03). Conclusions:The pathological morphometric analysis showed that the proliferation of interstitial connective tissue in FSHD inflammatory cell infiltration group was significantly more than that in PM group. Clinicians can identify the two from pathology and provide help for clinical practice.