Elsevier

Neuromuscular Disorders

Volume 31, Issue 9, September 2021, Pages 870-876
Neuromuscular Disorders

Case report
A rare case of sporadic inclusion body myositis and rheumatoid arthritis exhibiting ectopic lymphoid follicle-like structures: a case report and literature review

https://doi.org/10.1016/j.nmd.2021.07.002Get rights and content

Highlights

Abstract

Sporadic inclusion body myositis (sIBM) is a degenerative, intractable, inflammatory myopathy with an immune pathomechanism. We report on a case of a 44-year-old Japanese man who began developing progressive muscle weakness at age 40. Rheumatoid arthritis symptoms manifested at 43 with strongly positive anti-cyclic citrullinated peptide antibodies. Along with typical sIBM pathology, a muscle biopsy revealed dramatic inflammation with prominent perivascular B-cell infiltration forming ectopic lymphoid follicle-like structures (ELFLSs). Exome sequencing identified no causative variants of hereditary myopathy or immune disorders. A combination of immunotherapy slowed the progression of the muscular symptoms. This unusual form of sIBM, including earlier age at onset, a partial response to immunotherapy, and a histopathology presenting B-cell infiltrate with ectopic lymphoid follicle-like structures, indicates a possible association of rheumatoid arthritis and heterogeneity with the autoimmune involvement of sIBM. We review the clinical and pathological features of patients with rheumatoid arthritis associated sIBM in the literature.

Introduction

Sporadic inclusion body myositis (sIBM) is a degenerative, intractable, inflammatory myopathy with an immune pathomechanism [1]. sIBM develops slowly and insidiously, with 5 to 6 years elapsing between symptom onset and diagnosis; immunosuppressive therapy is usually not effective. A previous analysis of inflammatory cell composition revealed that endomysial mononuclear cells predominantly consisted of CD8+ T-cells, with few or no B-cells [2]. Although autoimmune features and coexisting autoantibodies are relatively common in sIBM [3], [4], [5], the disease has been associated with six cases of rheumatoid arthritis (RA) [6], [7], [8], [9], [10], and the clinicopathological characteristics and pathogenesis of RA-associated sIBM have not been elucidated.

In this study, we describe a patient with sIBM and subsequent RA whose muscle biopsy showed B-cell-predominant perivascular infiltration forming ectopic lymphoid follicle-like structures (ELFLSs) and sIBM pathology. We also review reports of clinical and pathological features of patients with RA-associated sIBM in the literature.

Section snippets

Case report

This study was approved by the Ethics Committee of the Tohoku University School of Medicine (No. 20774).

A 44-year-old Japanese man presented with a 4-year history of slowly progressive muscle weakness and atrophy in his hands, arms, and thighs. He was a heavy smoker, and his medical history included mild hypertension and hyperuricemia; his family history did not include any neuromuscular, cardiovascular, or autoimmune diseases. He first noticed the numbness of his left hand and difficult left

Literature review of sIBM patients with RA

Table 1 summarizes the clinical and pathological characteristics of seven sIBM patients with RA (our patient (case 1) and six from the literature [6], [7], [8], [9], [10]). The median age of sIBM onset was 53.5 years, with five male and two female patients. RA preceded sIBM in five of the six patients except for the presented case; the median age of RA onset was 41.0 years. Three patients had anti- tumor necrosis factor (TNF)α agent and MTX (cases 5–7), and four had PSL therapy (cases 2, 3, 5,

Discussion

We have described the clinicopathological features of an unusual sIBM patient who also had RA, for whom immunotherapy was partially effective. Our patient satisfied the European Neuromuscular centre IBM Research Diagnostic Criteria 2011 for clinico-pathologically defined sIBM, except for his younger age at onset (< 45 years). He had the distinct features of sIBM: selective involvement of finger flexors and quadriceps, myopathology showing CD8+ T-cell infiltration and surrounding of non-necrotic

Declaration of Competing Interest

The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

Acknowledgment

The authors would like to thank Yayoi Aoyama, Naoko Shimakura, Yoko Tateda, Maya Narisawa and Hinako Shigihara for their excellent technical assistance, and Dr Satoshi Yamashita, Dr Nozomu Tawara and Prof Yukio Ando for the anti-NT5C1A antibody assay. The authors would also like to thank Enago (www.enago.jp) for the English language review.

This work was supported by AMED under grant number 20dk0310086, an Intramural Research Grant (20FC1036) for Neurological and Psychiatric Disorders of NCNP,

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    • Myositis with prominent B cell aggregates may meet classification criteria for sporadic inclusion body myositis

      2023, Neuromuscular Disorders
      Citation Excerpt :

      In the present study, even though 5 patients had lymphoid aggregates with CD4+ T cell predominance over CD8+ T cell, 5 patients met at least one out of 5 sIBM classification criterion (range: 1-5 criteria; median: 3 criteria). Our literature search for myositis with prominent B cell aggregates mimicking sIBM was negative except for the single case of a 44-year-old man with finger flexor weakness, rimmed vacuoles and significant COX negative fibers on muscle pathology, and tubulofilamentous inclusions on EM yet had: 1) a myopathy responsive to treatment, 2) large, lymphoid-like aggregates at muscle pathology and 3) a diagnosis of RA on follow up (35). In fact, cases of sIBM with an associated CTD such as RA (46), SSc (47), SS (48) or SLE (49) were simply not tested for B cell aggregates.

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