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Table of Contents    
Year : 2014  |  Volume : 62  |  Issue : 1  |  Page : 66-67

Inclusion body myositis involving the diaphragm: Report of a pathologically confirmed case

Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, IN, USA

Date of Submission23-Jan-2014
Date of Decision31-Jan-2014
Date of Acceptance24-Jan-2014
Date of Web Publication7-Mar-2014

Correspondence Address:
Eyas M Hattab
Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, IN
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.128313

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How to cite this article:
Martin SE, Gondim DD, Hattab EM. Inclusion body myositis involving the diaphragm: Report of a pathologically confirmed case. Neurol India 2014;62:66-7

How to cite this URL:
Martin SE, Gondim DD, Hattab EM. Inclusion body myositis involving the diaphragm: Report of a pathologically confirmed case. Neurol India [serial online] 2014 [cited 2022 Aug 7];62:66-7. Available from: https://www.neurologyindia.com/text.asp?2014/62/1/66/128313


Sporadic inclusion body myositis (IBM) is an acquired inflammatory myopathy most common in older men and presents with insidious slowly progressive muscle weakness. [1] Finger flexor muscles and quadriceps are characteristically involved, but patients may infrequently present with foot drop or dysphagia. [2] Although occasionally involved in other inflammatory myopathies, respiratory muscles, including the diaphragm, are not usually implicated in IBM and almost never biopsied. Herein, we describe a case with histopathologic evidence of diaphragm involvement in IBM.

A 42-year-old female patient with a history of IBM [Figure 1]a and b, presented 6 years later with persistent abdominal pain. Abdominal computed tomography (CT) showed diffuse bilateral thickening of the diaphragm [Figure 2], not present on comparison CT from 3 years prior and the etiology of which was not clinically clear. Due to the concern for peritoneal carcinomatosis, the patient underwent a diagnostic laparotomy. No significant abnormalities were found during surgery and a biopsy of the diaphragm was obtained. The biopsy [Figure 1]c-f showed many fibers containing one or more vacuoles, some of which displayed basophilic stippling at the periphery characteristic of rimmed vacuoles. In comparison to the patient's first biopsy, only a mild endomysial mononuclear inflammatory infiltrate was present, along with fibrosis. There was marked variation in muscle fiber size and a moderate increase in the number of fibers with centrally placed nuclei. Scattered basophilic, degenerating/regenerating and necrotic fibers as well as rare intact fibers infiltrated by macrophages were identified. A diagnosis of IBM was established.
Figure 1: Initial deltoid biopsy showing intense inflammatory infi ltrate, intact fi ber invaded by macrophages (arrow) (a) and rimmed vacuolelike structure (arrow) (b). A later diaphragm biopsy showing mild infl ammatory infiltrate and marked muscle fiber size variation (c). Necrotic fi bers (d) and fi bers with rimmed vacuoles (e-f)

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Figure 2: Coronal view of an abdominal computed tomography with contrast demonstrates diffuse, bilateral thickening of the diaphragm (arrow heads), measuring up to 1.7 cm in thickness

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Inflammatory myopathies are known to involve the muscles of respiration, but this pattern of involvement is much more common in polymyositis and dermatomyositis than in IBM. [3] The presumption is that most cases of respiratory failure in patients with IBM are secondary to aspiration pneumonia or coincidental pulmonary or cardiac disease. [3] However, there have been several reported cases of IBM with presumed diaphragmatic involvement based on clinical function tests or on the patient's clinical presentation of respiratory failure combined with a concurrent positive limb muscle biopsy. [1],[3],[4] In addition to these reports of circumstantial evidence for diaphragm involvement in IBM, there has been one reported postmortem examination demonstrating histologic features of IBM in several different skeletal muscles, including the diaphragm. [5]

The diaphragm is not a common muscle chosen for biopsy. In our case, it was biopsied only because the patient was undergoing an exploratory laparotomy to rule out neoplasia. Our patient did not have respiratory symptoms, supporting the conclusion by one that diaphragm abnormalities can be present in patients without respiratory symptoms; therefore, IBM may involve the diaphragm more often than is clinically recognized. [1] Although, there has been one described autopsy study, to the best of our knowledge, this is the first reported case of IBM with biopsy evidence of diaphragm involvement. Awareness of the clinicopathologic spectrum of IBM is paramount for optimal patient management and to avoid potentially hazardous procedures as in this case. Additional clinical and pathological studies are necessary to assess the true prevalence of such occurrences.

 » References Top

1.Teixeira A, Cherin P, Demoule A, Levy-Soussan M, Straus C, Verin E, et al. Diaphragmatic dysfunction in patients with idiopathic inflammatory myopathies. Neuromuscul Disord 2005;15:32-9.  Back to cited text no. 1
2.Greenberg SA. Pathogenesis and therapy of inclusion body myositis. Curr Opin Neurol 2012;25:630-9.  Back to cited text no. 2
3.Voermans NC, Vaneker M, Hengstman GJ, ter Laak HJ, Zimmerman C, Schelhaas HJ, et al. Primary respiratory failure in inclusion body myositis. Neurology 2004;63:2191-2.  Back to cited text no. 3
4.Littleton ET, Man WD, Holton JL, Landon DN, Hanna MG, Polkey MI, et al. Human T cell leukaemia virus type I associated neuromuscular disease causing respiratory failure. J Neurol Neurosurg Psychiatry 2002;72:650-2.  Back to cited text no. 4
5.Inamori Y, Higuchi I, Inoue T, Sakiyama Y, Hashiguchi A, Higashi K, et al. Inclusion body myositis coexisting with hypertrophic cardiomyopathy: An autopsy study. Neuromuscul Disord 2012;22:747-54.  Back to cited text no. 5


  [Figure 1], [Figure 2]

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