The Clinical and Histological Spectrum of Idiopathic Inflammatory Myopathies

Ilaria Cavazzana, Micaela Fredi, Carlo Selmi, Angela Tincani, Franco Franceschini

Research output: Contribution to journalArticle

9 Citations (Scopus)

Abstract

Idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of myositis, characterised by chronic muscle weakness, cutaneous features, different extra-muscular manifestations and circulating autoantibodies. IIMs included classical polymyositis (PM), dermatomyositis (DM) and other different types of myositis with a wide range of muscle involvement. A complete autoantibody profile and a muscle biopsy are mandatory to correctly diagnose different clinical entities and to define their different prognosis. Bohan and Peter’s criteria included five items to diagnose adult onset PM and DM. The sensitivity was 74–100 %, while the specificity is low, due to a poor ability to differentiate PM from neuromuscular diseases. Other criteria included a more accurate histological definition of PM, DM or amyopathic DM, obtaining a higher specificity. Autoantibodies’ association, interstitial lung disease and clinical cardiac involvement represent the main items that could define the prognosis of these patients. On the other hand, inclusion body myositis is a different myopathy characterised by a peculiar muscle mass involvement, muscle atrophy and progressive loss of function, due to complete failure to all immunosuppressive drugs used. Treatment of IIMs is based on corticosteroids (CS), which show rapid clinical response and functional improvement. Different immunosuppressant drugs are given to obtain a better control of the disease during CS tapering dose. No controlled double blind trials demonstrated the superiority of one immunesuppressant on another. The occurrence of interstitial lung involvement requires the immediate introduction of immunosuppressants in addiction to CS. Severe dysphagia seems to improve with intravenous immunoglobulins (Ig). Physical therapy could be started after the acute phase of diseases and seems to have a beneficial role in muscle strength recovery.

Original languageEnglish (US)
JournalClinical Reviews in Allergy and Immunology
DOIs
StateAccepted/In press - Oct 29 2015
Externally publishedYes

Fingerprint

Myositis
Dermatomyositis
Immunosuppressive Agents
Autoantibodies
Adrenal Cortex Hormones
Muscles
Inclusion Body Myositis
Polymyositis
Neuromuscular Diseases
Muscular Atrophy
Intravenous Immunoglobulins
Interstitial Lung Diseases
Muscle Weakness
Muscle Strength
Acute Disease
Muscular Diseases
Deglutition Disorders
Pharmaceutical Preparations
Biopsy
Lung

Keywords

  • Dermatomyositis
  • Muscle biopsy
  • Muscle enzyme
  • Myositis
  • Myositis treatment

ASJC Scopus subject areas

  • Immunology and Allergy

Cite this

The Clinical and Histological Spectrum of Idiopathic Inflammatory Myopathies. / Cavazzana, Ilaria; Fredi, Micaela; Selmi, Carlo; Tincani, Angela; Franceschini, Franco.

In: Clinical Reviews in Allergy and Immunology, 29.10.2015.

Research output: Contribution to journalArticle

Cavazzana, Ilaria ; Fredi, Micaela ; Selmi, Carlo ; Tincani, Angela ; Franceschini, Franco. / The Clinical and Histological Spectrum of Idiopathic Inflammatory Myopathies. In: Clinical Reviews in Allergy and Immunology. 2015.
@article{891ffe30ad7d44fe9f330a375f64cd7d,
title = "The Clinical and Histological Spectrum of Idiopathic Inflammatory Myopathies",
abstract = "Idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of myositis, characterised by chronic muscle weakness, cutaneous features, different extra-muscular manifestations and circulating autoantibodies. IIMs included classical polymyositis (PM), dermatomyositis (DM) and other different types of myositis with a wide range of muscle involvement. A complete autoantibody profile and a muscle biopsy are mandatory to correctly diagnose different clinical entities and to define their different prognosis. Bohan and Peter’s criteria included five items to diagnose adult onset PM and DM. The sensitivity was 74–100 {\%}, while the specificity is low, due to a poor ability to differentiate PM from neuromuscular diseases. Other criteria included a more accurate histological definition of PM, DM or amyopathic DM, obtaining a higher specificity. Autoantibodies’ association, interstitial lung disease and clinical cardiac involvement represent the main items that could define the prognosis of these patients. On the other hand, inclusion body myositis is a different myopathy characterised by a peculiar muscle mass involvement, muscle atrophy and progressive loss of function, due to complete failure to all immunosuppressive drugs used. Treatment of IIMs is based on corticosteroids (CS), which show rapid clinical response and functional improvement. Different immunosuppressant drugs are given to obtain a better control of the disease during CS tapering dose. No controlled double blind trials demonstrated the superiority of one immunesuppressant on another. The occurrence of interstitial lung involvement requires the immediate introduction of immunosuppressants in addiction to CS. Severe dysphagia seems to improve with intravenous immunoglobulins (Ig). Physical therapy could be started after the acute phase of diseases and seems to have a beneficial role in muscle strength recovery.",
keywords = "Dermatomyositis, Muscle biopsy, Muscle enzyme, Myositis, Myositis treatment",
author = "Ilaria Cavazzana and Micaela Fredi and Carlo Selmi and Angela Tincani and Franco Franceschini",
year = "2015",
month = "10",
day = "29",
doi = "10.1007/s12016-015-8517-4",
language = "English (US)",
journal = "Clinical Reviews in Allergy and Immunology",
issn = "1080-0549",
publisher = "Humana Press",

}

TY - JOUR

T1 - The Clinical and Histological Spectrum of Idiopathic Inflammatory Myopathies

AU - Cavazzana, Ilaria

AU - Fredi, Micaela

AU - Selmi, Carlo

AU - Tincani, Angela

AU - Franceschini, Franco

PY - 2015/10/29

Y1 - 2015/10/29

N2 - Idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of myositis, characterised by chronic muscle weakness, cutaneous features, different extra-muscular manifestations and circulating autoantibodies. IIMs included classical polymyositis (PM), dermatomyositis (DM) and other different types of myositis with a wide range of muscle involvement. A complete autoantibody profile and a muscle biopsy are mandatory to correctly diagnose different clinical entities and to define their different prognosis. Bohan and Peter’s criteria included five items to diagnose adult onset PM and DM. The sensitivity was 74–100 %, while the specificity is low, due to a poor ability to differentiate PM from neuromuscular diseases. Other criteria included a more accurate histological definition of PM, DM or amyopathic DM, obtaining a higher specificity. Autoantibodies’ association, interstitial lung disease and clinical cardiac involvement represent the main items that could define the prognosis of these patients. On the other hand, inclusion body myositis is a different myopathy characterised by a peculiar muscle mass involvement, muscle atrophy and progressive loss of function, due to complete failure to all immunosuppressive drugs used. Treatment of IIMs is based on corticosteroids (CS), which show rapid clinical response and functional improvement. Different immunosuppressant drugs are given to obtain a better control of the disease during CS tapering dose. No controlled double blind trials demonstrated the superiority of one immunesuppressant on another. The occurrence of interstitial lung involvement requires the immediate introduction of immunosuppressants in addiction to CS. Severe dysphagia seems to improve with intravenous immunoglobulins (Ig). Physical therapy could be started after the acute phase of diseases and seems to have a beneficial role in muscle strength recovery.

AB - Idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of myositis, characterised by chronic muscle weakness, cutaneous features, different extra-muscular manifestations and circulating autoantibodies. IIMs included classical polymyositis (PM), dermatomyositis (DM) and other different types of myositis with a wide range of muscle involvement. A complete autoantibody profile and a muscle biopsy are mandatory to correctly diagnose different clinical entities and to define their different prognosis. Bohan and Peter’s criteria included five items to diagnose adult onset PM and DM. The sensitivity was 74–100 %, while the specificity is low, due to a poor ability to differentiate PM from neuromuscular diseases. Other criteria included a more accurate histological definition of PM, DM or amyopathic DM, obtaining a higher specificity. Autoantibodies’ association, interstitial lung disease and clinical cardiac involvement represent the main items that could define the prognosis of these patients. On the other hand, inclusion body myositis is a different myopathy characterised by a peculiar muscle mass involvement, muscle atrophy and progressive loss of function, due to complete failure to all immunosuppressive drugs used. Treatment of IIMs is based on corticosteroids (CS), which show rapid clinical response and functional improvement. Different immunosuppressant drugs are given to obtain a better control of the disease during CS tapering dose. No controlled double blind trials demonstrated the superiority of one immunesuppressant on another. The occurrence of interstitial lung involvement requires the immediate introduction of immunosuppressants in addiction to CS. Severe dysphagia seems to improve with intravenous immunoglobulins (Ig). Physical therapy could be started after the acute phase of diseases and seems to have a beneficial role in muscle strength recovery.

KW - Dermatomyositis

KW - Muscle biopsy

KW - Muscle enzyme

KW - Myositis

KW - Myositis treatment

UR - http://www.scopus.com/inward/record.url?scp=84945579159&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84945579159&partnerID=8YFLogxK

U2 - 10.1007/s12016-015-8517-4

DO - 10.1007/s12016-015-8517-4

M3 - Article

JO - Clinical Reviews in Allergy and Immunology

JF - Clinical Reviews in Allergy and Immunology

SN - 1080-0549

ER -