Survival and associated comorbidities in inclusion body myositis

Elie Naddaf1, Shahar Shelly1, Jay Mandrekar2

  • 1Department of Neurology.

Abstract

Insights

Inclusion body myositis (IBM) patients face significantly lower survival rates and a higher incidence of peripheral neuropathy, Sjögren's syndrome, and hematologic malignancies compared to the general population. Careful monitoring for IBM complications is crucial.

Area of Science:

  • Neurology
  • Immunology
  • Epidemiology

Background:

  • Inclusion body myositis (IBM) is a rare, progressive idiopathic inflammatory myopathy.
  • Understanding the long-term outcomes and associated conditions in IBM is critical for patient management.

Purpose of the Study:

  • To evaluate survival rates and the prevalence of comorbidities in patients with inclusion body myositis (IBM).
  • To compare these outcomes against other inflammatory myopathies (IIM) and population controls.

Main Methods:

  • A population-based, case-control study utilizing the Rochester Epidemiology Project medical records-linkage system.
  • Identification of 50 IBM patients, 65 IIM controls, and 294 population controls across 27 counties in Minnesota and Wisconsin.
  • Comparison of comorbidity frequencies and survival rates among the identified groups.

Main Results:

  • IBM patients exhibited a 10-year survival rate of 36%, significantly lower than IIM (67%) and population controls (59%).
  • Higher frequencies of peripheral neuropathy (2.7x), Sjögren's syndrome (6.2x), and hematologic malignancies (3.9x) were observed in IBM patients compared to population controls.
  • Dysphagia was common in IBM (64%), and respiratory failure/pneumonia was the leading cause of death (44%).

Conclusions:

  • Inclusion body myositis is associated with reduced survival and an increased risk of specific comorbidities, including peripheral neuropathy, Sjögren's syndrome, and hematologic malignancies.
  • These findings underscore the need for vigilant monitoring of IBM patients for potential complications to improve management and outcomes.

Related Concept Videos

Disorders of the Skeletal Muscle01:28

Disorders of the Skeletal Muscle

The clinical conditions affecting the skeletal muscle tissue are broadly categorized as musculoskeletal and neuromuscular disorders.
Musculoskeletal disorders
Musculoskeletal disorders involve injuries and conditions affecting the skeletal muscles and associated connective tissues. These disorders can arise from acute biomechanical stresses or chronic overuse and can occur across different age groups. Common injuries include sprains, fractures, and muscular strains, often resulting from...
1.3K
Myocarditis I: Introduction01:21

Myocarditis I: Introduction

Myocarditis is inflammation of the myocardium, which is the muscular layer of the heart.EtiologyMyocarditis has a diverse etiology, including a wide range of infectious and non-infectious causes:Infectious CausesViral: Common viruses include Coxsackie A and B, adenovirus, parvovirus B19, enteroviruses, and influenza A.Bacterial: Examples include infections caused by Streptococcus, Staphylococcus, and Mycoplasma species.Rickettsial: Infections like Rocky Mountain spotted fever can result in...
71
Chronic Bowel Disorders: Introduction01:17

Chronic Bowel Disorders: Introduction

Chronic bowel diseases are a group of long-term conditions affecting the digestive tract, characterized by inflammation and damage to the gut lining. These conditions primarily include irritable bowel syndrome and inflammatory bowel disease.
Irritable Bowel Syndrome (IBS) is a common disorder affecting the gastrointestinal tract. The distinctive feature is recurrent abdominal pain associated with altered bowel movements, manifesting as constipation, diarrhea, or fluctuating between both. The...
569
Inflammatory Bowel Disease II: Crohn's Disease01:30

Inflammatory Bowel Disease II: Crohn's Disease

Introduction
Inflammatory bowel disease, commonly known as IBD, refers to a collection of disorders that lead to persistent inflammation of the gastrointestinal tract. The two types of IBD are ulcerative colitis, which impacts the colon, and Crohn's disease, which can involve any part of the gastrointestinal segment.
Crohn's disease
Crohn's disease is a chronic, systemic inflammatory bowel disease (IBD) that predominantly affects the gastrointestinal tract. It is marked by...
467
Myocarditis IV: Nursing Management01:22

Myocarditis IV: Nursing Management

Myocarditis is an inflammatory condition of the myocardium requiring meticulous nursing management for optimal patient outcomes. Effective management begins with a thorough assessment of the patient's medical history, paying close attention to past infections, autoimmune disorders, travel history, and exposure to toxins or drugs. Recent viral infections and systemic diseases are particularly relevant due to their potential role in triggering myocarditis.Physical Examination and MonitoringThe...
58
Myocarditis II: Clinical Features and Diagnostic Tests01:27

Myocarditis II: Clinical Features and Diagnostic Tests

Myocarditis is an inflammation of the heart muscle. The symptoms vary widely, encompassing asymptomatic presentations to severe, acute manifestations.Clinical PresentationAsymptomatic cases: In some instances, myocarditis may be asymptomatic, with the infection resolving without intervention. These cases often go undetected unless discovered incidentally through diagnostic imaging or tests conducted for other reasons.General Early Symptoms: Early symptoms of myocarditis are non-specific and can...
47