Small intestinal biopsy findings consistent with celiac disease in patients with idiopathic inflammatory myopathy: Review of existing literature

Gunhild Alvik Nyborg,Øyvind Molberg

doi:10.1016/j.semarthrit.2021.07.012

Abstract

BackgroundCase reports have described patients with idiopathic inflammatory myopathies (IIM) and a concurrent diagnosis of celiac disease (CeD) for whom the muscle inflammation (myositis) component of IIM improves after the patients start standard treatment with gluten-free diet (GFD). A connection between IIM and CeD is not commonly recognized. AimIn this first systematic review of the topic, we aimed to explore all peer-reviewed publications of IIM cases and concomitant small intestinal biopsy findings consistent with CeD, published after 1975. MethodsSystematic literature searches were performed in MEDLINE, PubMed, and EMBASE, supplemented by screening of references and non-systematic searches via Google and Google Scholar. ResultsAltogether 30 cases published between 1976 and 2017 were uncovered. Information about gastrointestinal symptoms prior to CeD diagnosis was available for 19 patients, with 6/19 (32%) reporting no GI symptoms. CeD-related serological data were available in 23/30 patients. Endomysial antibodies were present in 10/18 (56%), while only 2/9 (22%) had antibodies against tissue transglutaminase. Serum antibodies to native gliadin were present in 16/18 (89%). Clinical effects of a GFD on the IIM were reported for 24 patients, with signs of improvement in 14/24 (58%), including three patients with otherwise therapy-resistant inclusion body myositis. Longitudinal follow-up data available from the published studies indicated that 7/24 (29%) remained in clinical IIM remission with GFD as the sole therapeutic intervention. ConclusionIn the IIM cases presented here, duodenal biopsy findings consistent with celiac disease was sometimes present without classical CeD symptoms or positive traditional CeD serology, and in the majority of cases, the IIM improved after introduction of a gluten-free diet. While extra vigilance towards CeD in IIM patients seems warranted, there is need for more research to clarify if GFD has effects on organ systems other than the small intestine in patients with IIM and small intestinal biopsy findings consistent with CeD.

Highlights

Case reports have described patients with idiopathic inflammatory myopathies (IIM) and a concurrent diagnosis of celiac disease (CeD) for whom the muscle inflammation component of IIM improves after the patients start standard treatment with gluten-free diet (GFD)
One case was included in spite of a normal duodenal biopsy: A 73 year old DQ2+, IgG-Anti-gliadin antibodies (AGA)+, anti-TG2- female with PM, in whom GFD as monotherapy led to elimination of AGA, significant improvement of muscle strength, and s-Creatine kinase (CK) reduction from 1058 at presentation to 51 IU/L
Seven (26%) of these come from studies screening for CeD in IIM patients [39,45,48] (Table 2)

Summary

Introduction

Case reports have described patients with idiopathic inflammatory myopathies (IIM) and a concurrent diagnosis of celiac disease (CeD) for whom the muscle inflammation (myositis) component of IIM improves after the patients start standard treatment with gluten-free diet (GFD). The IIMs are part of the systemic connective tissue disease (CTD) spectrum of disorders They are rare; with reported incidence estimates ranging from 1¢16 to 19/million/year and prevalence from 2¢4 to 33¢8 per 100 000 inhabitants [1]. Apart from esophageal motor abnormalities, which are highly common and cause troublesome dysphagia, there has not been much focus on gastro-intestinal tract involvement in IIM It is clear from small case series that some few patients develop severe GI complications, such as intestinal ischemia and/or intestinal perforations [4,5]

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