Neuromuscular

Home, Search, Index, Links, Pathology, Molecules, Syndromes,
Muscle, NMJ, Nerve, Spinal, Ataxia, Antibody & Biopsy, Patient Info


Inflammatory and Immune Myopathies: Acquired

GENERAL FEATURES

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

Pathology
  Brachio-Cervical (BCIM)
  Calcinosis: X-rays
  Chondroitin sulfate C deficient
  Decorin antibody (BJ) myopathy
  Dermatomyositis
  Fasciitis
  Focal myositis
  Inclusion Body Myositis
  Inflammation: Muscle
  Inflammation: Cell patterns
  Myasthenia gravis
  Necrosis: Muscle fibers
  Neonatal perifascicular
  Nodular granulomatous myositis
  Perimysial pathology (IMPP)
  Signal recognition particle
  Vasculitis: Small & Large vessel

Animal Models
  Experimental autoimmune myositis
  MHC class I upregulation

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
  Malignancy-associated
  MAS antibody
  Perimysial pathology (IMPP)
    Jo-1
    OJ
    t-RNA synthetase antibodies
  PM + Mitochondrial (PM/COX-)
  Sarcoidosis
  Signal recognition particle antibody
  Systemic disorders & Myositis
  U1-snRNP antibodies

Dermatomyositis
  Childhood (Juvenile)
  Adult
    Drug-induced
    Malignancy-associated
    MDA-5 antibody
    PM-Scl antibody +
    Amyopathic
  Mi-2 antibody +
  Perimysial pathology (IMPP)
    EJ antibody
    Jo-1 antibody

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
    Quadriceps; Other
    TRAPS
  Granulomatous
  Hemophagocytic lymphohistiocytosis
  Hereditary
  IM + abundant Macrophages
  Infection
    Influenza
    Lyme myositis
  Macrophagic myofasciitis
  Masticator myopathy
  Mitochondrial antibody
  Multinodular polymyositis
  Myasthenia gravis
    Brachio-Cervial Inflammatory (BCIM)
    Myositis
    Lymphorrhages
  Necrotizing
    with Encephalopathy
    Paraneoplastic
    with Pipestem capillaries
    SRP antibodies
  Neonatal perifascicular myopathy
  Orbital
  Perimyositis
  Polymyalgia Rheumatica
  Pyomyositis
  Sarcocystis
  Sarcoidosis
  Trichinellosis (Trichinosis)
PATHOLOGIC CLASSIFICATION 68

Perimysial pathology (IMPP)
Feature: Connective tissue pathology
  Aldolase high
  Dermatomyositis with IMPP
    Amyopathic (CADM-140)
  Fasciitis
  Focal myositis
  Graft-vs-host disease
  t-RNA synthetase antibodies: Jo-1
  IMPP + Necrosis
    HMGCR (200/100) antibody

Myovasculopathies
Feature: Damage to large or small vessels
  Dermatomyositis with Vascular Pathology
    (DM-VP; Childhood Dermatomyositis)
  Regional Immune (Paraneoplastic)
  Pipestem capillaries

Immune Polymyopathies
Features: Necrosis; Little inflammation
  SRP antibody: Scattered necrosis

IM + Endomysial Pathology (IM-EP)
Features: C5b9 deposits; Glycoprotein Δ
  Brachio-Cervical Inflammatory (BCIM)
  Chondroitin sulfate C deficient
  Decorin antibody (BJ) myopathy

Histiocytic Myopathies
Feature: Foci of histiocytic cells
  Granulomatous
    Mitochondrial antibody
    Sarcoid
  Hemophagocytic lymphohistiocytosis
  IM + Abundant Macrophages (IMAM)
  Macrophagic myofasciitis

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

From: M Al-Lozi


Skin lesions


Erythema


Gottron's papules


Nailfold lesions

From: Chinju, South Korea

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 Weakness Pain Other systemic features Prognosis worse
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

DERMATOMYOSITIS

MYOSITIS + SKIN CHANGES

Dermatomyositis
  Clinical features
  Laboratory
  Pathology
    Description
    Histology
  Treatment

Classification
  Dermatomyositis with Vasular Pathology
    Childhood
    Adult
    Childhood & Adult
      Mi-2 antibody positive
  Dermatomyositis: Other Adult
    Amyopathic
    Drug-related
    Mi-2 antibody negative
    Malignancy-associated
    Jo-1 antibody positive
    EJ antibody positive
    PM-Scl

Tim Miller


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

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

Subtypes
  Inclusion body myositis (IBM)
    Muscle Pathology
  Inflammatory myopathy with 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 myopathies with Anti-mitochondrial antibodies 72


Necrotizing myopathy with pipestem capillaries1


Myopathy with deficient chondroitin sulfate C in skeletal muscle connective tissue3


Benign acute childhood myositis8


Influenza-Associated Myositis


Idiopathic orbital myositis


Masticator Myopathy37


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

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

References
1. Neurology 1996;46:1448-51
2. Curr Opin Rheum 1997;9:513-519
3. Neurology 1998;50:526-529, J Neurol Neurosurg Psychiatry 2002;73:89–90
4. Lancet 1998;352:347-351, Neurology 2000;54 S3:A439, Brain 2001;124:974-983
5. NEJM 1998;338:1592-1600
6. J Neuroimmunol 1999;98:130-135
7. J Neurol Sci 1999;168:68-70
8. Neurology 1999;53:2127-2131
9. Lupus 2000;9:42-46
10. J Neurol Sci 2000;175:140-144, Muscle Nerve 2011;44:994-999
11. PNAS 2000;97:9209-9214
12. PNAS 2000;97:9174-9179
13. Eur J Hum Genet 2000;8:717-720
14. J Neurol Sci 2000;176:92-102
15. Lancet 2001;357:96-100,, Ann Int Med 2001;134:1087-1095, Arthritis Res Ther 2010;12:R70 Online April
16. Brain 2001;124:341-351; Neurology 2000;54:65
17. Muscle Nerve 2001;24:433-437, Neuromusc Disord 1996;6:255-260
18. Arch Neurol 2000;57:527-531
19. Muscle Nerve 2000;23:1133-1136
20. Arch Int Med 2001;161:1381-1389
21. Ann Int Med 2001;135:27-31
22. Medicine 2001;80:320-327, Brain 2011 Sep 29, Brain 2011 Oct 12, Curr Opin Neurol 2014;27:591-598
23. Curr Opin Rheumatol 2001;13:428-433
24. Arch Dermatol 2002;138:23-27
25. Muscle Nerve 2002;25:461-465
26. Neurology 2002;58:1779–1785
27. Arthritis Rheum 2002;46:1885-1893
28. Rheumatology 2002;41:1040–1046
29. J Exp Med 2002;196:781-791
30. Rheumatology 2003;42:34–39, Eur J Haematol 2005;75:522-526
31. Acta Neurol Scand 2003;107:134–141
32. Arthritis Rheumatism 2002;47:614–622, Respir Med 2007 Mar 10, Clin Rheumatol 2009;28:639–646, Curr Rheumatol Rep 2012; Feb 25
33. Rheumatology 2003;42:575–582
34. Muscle Nerve 2003;27:407–425
35. Muscle Nerve 2003;27:442–448
36. J Am Acad Dermatol 2003;48:530-534
37. Muscle Nerve 2003;28:123-127, Muscle Nerve 2004;29:782–789
38. Arthritis Rheumatism 2003;48:2285–2293
39. Am J Hum Genet 2003; October
40. J Neuropath Exp Neurol 2003;62:464-474
41. J Rheumatol 2003;30:2513-2516
42. Brain 1997;120:39-45; Muscle Nerve 1998;12:1724-1728
43. Muscle Nerve 2002;25:293–296
44. Am J Trop Med Hyg 1999;61:548–553
45. Curr Opin Rheumatol 2005;17:725—730
46. Arthritis Research & Therapy 2006;8:R13
47. Arthritis & Rheumatism 2006;54:1687-1696, J Neurol Sci 2010 Mar 15, Neurol Sci 2010 Oct 2
48. Brain 2002;125:264-275
49. Rheumatology (Oxford) 2007 Jan 25
50. Ann Rheum Dis 2007 Mar 28, J Rheumatol 2009;36:2704-2710
51. Semin Respir Crit Care Med 2007;28:15-21
52. Arthritis Rheum 2007;56:2719-2728
53. Rheum Dis Clin North Am 2007;33:525-553, Lancet 2008;371:2201-2212, Curr Dir Autoimmun. 2008;10:313-332
54. J Am Acad Dermatol 2008; Jul 12
55. J Rheumatol 2008 Jul 15
56. J Eur Acad Dermatol Venereol 2008 Aug 22
57. Medicine 2008;87:70-86
58. J Neurol Sci 2009;278:25-29
59. J Neurol Neurosurg Psychiatry 2009 Feb 23
60. Clinical and Experimental Rheumatology 2009; 27: 519-526
61. Arthritis & Rheumatism 2009;60:2499–2504
62. J Rheumatol 2009;36:1943–1946
63. Muscle Nerve 2010; Online Jan
64. Arthritis & Rheumatism 2010; Online May, Arthritis & Rheumatism 2011;63:713-721, Arthritis Care & Research 2012;64:269-272, Arthritis & Rheumatism 2012; Online August
65. Clin Exp Rheumatol 2009;27:1031-1038
66. Pediatric Neurology 2011;45:114-116
67. J Rheumatol 2011 Aug 15, Medicine (Baltimore) 2009;88:91-97
68. Curr Opin Rheumatol 2011;23:595-604
69. Semin Arthritis Rheum 2011 Nov 9
70. PLoS One 2011;6:e20266, Neuromuscular Disorders 2012;22:875
71. Clin Exp Rheum 2012; Online Jan, Medicine (Baltimore) 2013;92:223-243
72. Brain 2012; Online May
73. Journal of Neuroimmunology 2012; Online May
74. Curr Opin Rheumatol 2012 Aug 17
75. Am J Trop Med Hyg 2012;87:297-302
76. Muscle Nerve 2013; Online August
77. Support Care Cancer 2014; Online March
78. Neurology 2014;83:98-99
79. Rheumatology 2014; Online July
80. Rheumatology 2014; Online July

9/5/2014