ABSTRACT
Several autoantigens related to inflammatory myopathy have been identified. Antimitochondrial antibody M2 (AMA-M2) is known as one of the serologic hallmarks of primary biliary cholangitis (PBC). There have been several reports on the association between AMA-M2 and various types of inflammatory myopathy, including cardiomyopathy. We report a case of a 58-year-old man with decompensated heart failure who also had PBC and skeletal inflammatory myopathy. Endomyocardial biopsy revealed severe fibrotic replacement of the myocardium without massive inflammatory infiltration, which was pathologically similar to what happens in dilated cardiomyopathy (DCM). Although the potential relationship between chronic autoimmune inflammation and DCM has been discussed, the concept of the inflammatory DCM has not yet been established. When we see elevated liver enzymes, and which is not simply due to congestive hepatopathy, we should consider the coexisting disease such as PBC.
Subject(s)
Cholangitis , Heart Failure , Liver Cirrhosis, Biliary , Liver Diseases , Muscular Diseases , Myositis , Autoantibodies , Autoantigens , Cholangitis/pathology , Heart Failure/complications , Heart Failure/pathology , Humans , Liver Cirrhosis, Biliary/complications , Liver Cirrhosis, Biliary/diagnosis , Liver Diseases/pathology , Male , Middle Aged , Mitochondria/pathology , Myositis/pathologyABSTRACT
Denosumab is reported to increase bone mineral density (BMD) among haemodialysis patients; however, hypocalcaemia is a serious adverse effect among chronic kidney disease (CKD) patients. Identifying which patients will show greater improvement in BMD is important. We enrolled 84 haemodialysis patients with osteoporosis in our study. 28 patients initiated denosumab treatment between October 2019 and October 2020. We assessed BMD changes and investigated the association between baseline bone turnover marker (BTM) levels and 6-month changes in BMD after denosumab treatment. BMD was increased at 6 months in denosumab-treated patients compared with patients not treated with denosumab (lumbar spine: 5.34% vs. - 0.49%; total hip: 2.43% vs. - 0.47%). Bone-specific alkaline phosphatase (BAP) and tartrate-resistant acid phosphatase-5b (TRACP-5b) at baseline were independently associated with increased BMD in the total hip (BAP: ß = 0.472, p value = 0.004; TRACP-5b: ß = 0.433, p value = 0.008) and lumbar spine (BAP: ß = 0.591, p value = 0.001; TRACP-5b: ß = 0.613, p value = 0.0008). BAP and TRACP-5b were also independent predictors of hypocalcaemic events (OR [95% CI] 1.747 [1.084-4.604] and 1.006 [1.000-1.015], respectively). BTMs may be associated with increased BMD and hypocalcaemic events after denosumab treatment. BTM measurement may be useful for assessing the effect of denosumab on BMD; however, careful monitoring of serum calcium levels is needed.
