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Inflammatory and Immune Myopathies (IIM): Acquired

GENERAL FEATURES

Signs & Lab findings
  Aldolase
  Antibodies
  Calcinosis
  CK: Serum
  Electrodiagnostic
  HLA & Genotypes
  Neoplasm associations
  Other systemic
  Pain
  Pathology
  Prognosis
  Skin lesions
  Syndromes
  Treatment
  Weakness

Pathology

Animal Models
  Experimental autoimmune myositis
  MHC class I upregulation

History
  Dermatomyopathies
  IIM: Possible 1st case, Hepp 1887
    Berliner Klinische Wochenschrift
SPECIFIC IMMUNE OR INFLAMMATORY MYOPATHIES

Immune myopathies (Polymyositis +...)
  General aspects
    Clinical
    Laboratory
    Subtypes & Comparative features
  Brachio-Cervical Inflammatory (BCIM)
  Collagen vascular disease
  Complement: C2 deficiency
  Drug-induced
  Graft-vs-host disease
  HMGCR (200/100) antibody
  Idiopathic
  IMPP
  MAS antibody
  Nup antibody
  Perimysial pathology (IMPP)
    Jo-1
    OJ
    t-RNA synthetase antibodies
  PM + Mitochondrial (PM/COX-)
  Regional ischemic (RIIM)
  Sarcoidosis
  Signal recognition particle Ab (SRP)
  Systemic disorders & Myositis
  U1-snRNP antibodies

Dermatomyopathies
  Childhood (Juvenile)
  Adult
    Drug-induced
    Malignancy-associated
      p155 (TIF-γ) Ab
      Regional ischemic (RIIM)
    MDA-5 antibody
    PM-Scl antibody +
    Amyopathic
  Mi-2 antibody +
  Perimysial pathology (IMPP)
    EJ antibody
    Jo-1 antibody

Histiocytic
  Granulomatous
    Mitochondrial antibody
    Sarcoidosis
  IM + Abundant Macrophages
  Macrophagic myofasciitis
  IM-VAMP
  Inclusion body myositis (IBM)
  IM-Mito (PM/COX-)

Other "Immune Myopathies"
  Benign acute childhood myositis
  Celiac disease
  Chondroitin sulfate C deficient
  Decorin antibody (BJ) myopathy
  Eosinophilia myalgia syndrome
  Fasciitis
  Focal myositis
    Orbital
    Quadriceps; Other
    TRAPS
  Hemophagocytic lymphohistiocytosis
  Hereditary
  Infections
    Influenza
    Lyme myositis
    Pyomyositis
    Sarcocystis
    Toxoplasmosis
    Trichinellosis (Trichinosis)
  Lupus
  Masticator myopathy
  Multinodular polymyositis
  Myasthenia gravis
    Brachio-Cervial Inflammatory (BCIM)
    Myositis
    Lymphorrhages
  Necrosis, Abundant
    Encephalopathy
    HMGCR (200/100) antibody
    Jo-1 antibody
    Pipestem capillaries
    Regional Ischemic Immune (RIIM)
    SRP antibodies
  Neonatal perifascicular myopathy
  Perimyositis
  Polymyalgia Rheumatica
IIM: PATHOLOGIC CLASSIFICATION 68

Myofasciopathy
Feature: Connective tissue pathology
  Perimysial Pathology(IMPP)
  Features
      Perimysial damage & Histiocytes
      Aldolase high
    Dermatomyopathy with IMPP
      Amyopathic (MDA5 Ab)
    Fasciitis
    t-RNA synthetase antibodies: Jo-1
    IMPP + Necrosis
      HMGCR (200/100) antibody
  Endomysial Pathology
  Features: C5b9 deposits; Glycoprotein Δ
    Brachio-Cervical Inflammatory (BCIM)
  Chondroitin sulfate C deficient
    Decorin antibody (BJ) myopathy
  Endomysial + Perimysial Pathology
  Feature: Connective tissue proliferation
    SRP antibody
    Focal myositis

Myovasculopathy
Feature: Damage to large or small vessels
  Dermatomyositis with Vascular Pathology
    (DM-VP; Childhood Dermatomyositis)
  Regional Ischemic (RIIM; Paraneoplastic)
  DM + TIF1γ antibodies
  Pipestem capillaries

Histiocytic Myopathy
Histiocytic cells: Foci or Predominant
  Granulomatous
    Mitochondrial antibody
    Sarcoid
  Hemophagocytic lymphohistiocytosis
  IM + Abundant Macrophages (IMAM)
  IRIS
  Macrophagic myofasciitis

Primary Myopathy
  IM-VAMP syndromes
  Features: Foci of T-cells but not B-cells
      Vacuoles, Aggregates or Mito Path
      No response to immunomodulation
    Inclusion body myositis
    IM + Mitochondrial Pathology

Varied Pathology
    Graft-vs-host disease


Immune or Inflammatory Myopathies

General Features 34

Antibodies
CK: Serum
Electrodiagnostic
Neoplasm associations
Other systemic
Pain
Pathology
Prognosis
Skin lesions
Treatment
Weakness

Skin lesions 74


DM-VP: Rash

From: M Al-Lozi

Rash: Erythema
From: Chinju, South Korea

Gottron's papules


Nailfold lesions
Weakness Pain Other systemic features
IIM: Prognosis
Serum: Creatine Kinase (CK) & Aldolase
Immune myopathies: HLA & Genetic associations 46
Inflammatory myopathies: Association with neoplasms 15
Serum Antibodies


Electrodiagnostic changes
MRI 33
Pathology

Classification

Inflammation
Muscle fiber pathology
Capillaries
Mitochondrial
Connective tissue
Matrix metalloproteinase
MHC-I
Endomysial inflammation

Oppenheim 1894

Treatment

IMMUNE MYOPATHIES ("Polymyositis" + Other syndromes)

Subtypes
Clinical features
Laboratory
Treatment


IMMUNE MYOPATHIES (Polymyositis): Clinical features IMMUNE MYOPATHIES (Polymyositis): Laboratory features IMMUNE MYOPATHIES (Polymyositis): Muscle Pathology IMMUNE & INFLAMMATORY MYOPATHIES: Clinical Syndromes

Polymyositis: Treatment options

DERMATOMYOPATHIES, IMMUNE

IMMUNE MYOPATHY + SKIN CHANGES
Dermatomyositis
  Clinical features
  Comparative features
  Laboratory
  Pathology
    DM-VP: Description
    IMPP
    RIIM
  Treatment

History: Firsts
  ? 1st Case: 1863 Wagner
    Archiv der Heilkunde
  Unverricht: Use of term
  Batten: Pathology
Classification
  Dermatomyositis with Vasular Pathology
    Childhood
    Adult
    Childhood & Adult
      Mi-2 antibody positive
    TIF1γ (p155) antibody
  Dermatomyopathy: Other Adult
    Amyopathic
    Drug-related
    MDA5 antibody
    Mi-2 antibody negative
    Malignancy-associated
    RIIM
  IMPP
    Jo-1 antibody positive
    EJ antibody positive
    PM-Scl

Tim Miller


Dermatomyopathies & Dermatomyositis: Clinical features, general Dermatomyositis syndromes: Specific features Dermatomyositis: Laboratory features Dermatomyositis treatment: Immunosuppressive & Other

First use of term "Dermatomyositis"

Full article 1891
Unverricht's first description of a case of Dermatomyositis: Polymyositis acuta progressive. Z Klin Med 1887;12:553

Inflammatory Myopathies with Vacuoles, Aggregates & Mitochondrial Pathology (IM-VAMP)

Subtypes
  Inclusion body myositis (IBM)
    Antibodies
    Clinical
    Epidemiology
    Laboratory
    Muscle Pathology
  Inflammatory myopathy + Mitochondrial pathology (IM-Mito)
    Muscle pathology


Inclusion Body Myositis (IBM) 22


Inflammatory Myopathy + Mitochondrial Pathology in muscle (IM-Mito), subtype of IM-VAMP 42



INFLAMMATORY MYOPATHIES: Other variants

Absent chondroitin sulfate C
Experimental autoimmune myositis
Fasciitis
Focal myositis
Granulomatous
Hemophagocytic lymphohistiocytosis
Hereditary
Infection
  BACM
  Influenza
  Lyme
Necrotizing with pipestem capillaries
Orbital
Perimyositis


Granulomatous myopathies


Granulomatous & Inflammatory Myopathies with Anti-mitochondrial antibodies 72


Myopathy with Muscle fiber necrosis & Pipestem capillaries 1


Myopathy with Chondroitin sulfate C loss in skeletal muscle endomysium 3


Benign acute childhood myositis 8


Influenza-Associated Myositis


Orbital (Ocular) Myositis, Idiopathic 98


Masticator Myopathy 37


Hemophagocytic Lymphohistiocytosis 41


Hereditary inflammatory myopathies


Myositis: Infectious agents


Inflammatory myopathy: Other


INFLAMMATORY MYOPATHIES: Animal Models


Sarcoidosis

General features
Neurological features
  Myopathy
    Acute
    Chronic
    Nodular
  Neuropathy
  Myelopathy
Laboratory
Pathology
Treatment

Hilar adenopathy

Trichinellosis (Trichinosis) 75


Sarcocystis (Sarcosporidiosis) 44


From USDA
Sarcocystis in muscle


From: CDC

Toxoplasmosis


H&E stain: From CDC
Toxoplasma gondii cyst
Contains bradyzoites

Eosinophilic fasciitis


Eosinophilic fasciitis
Chronic skin lesions

Perimyositis 25


Macrophagic myofasciitis 4


Inflammatory myopathy with abundant macrophages (IMAM) 40


Return to Myopathy Differential Diagnosis
Return to Neuromuscular Home Page

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