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POLYMYOSITIS, DERMATOMYOSITIS, AND
INCLUSION BODY MYOSITIS:
INTRODUCTION;

 The inflammatory myopathies represent the largest
 group of acquired and potentially treatable causes of
 skeletal muscle weakness.

 They are classified into three major groups:
   polymyositis (PM),
   dermatomyositis (DM), and
   inclusion body myositis (IBM).
POLYMYOSITIS
 The actual onset of PM is often not easily determined,
 and patients typically delay seeking medical advice for
 several weeks or even months.

 This is in contrast to DM, in which the rash facilitates
 early recognition.

 PM mimics many other myopathies and is a diagnosis
 of exclusion.
DERMATOMYOSITIS and
INCLUSION BODY MYOSITIS
 DERMATOMYOSITIS

 DM is a distinctive entity identified by a characteristic rash accompanying, or
 more often preceding, muscle weakness.

 DM usually occurs alone but may overlap with scleroderma and mixed
 connective tissue disease.

 INCLUSION BODY MYOSITIS

 In patients 50 years of age, IBM is the most common of the inflammatory
 myopathies. It is often misdiagnosed as PM and is suspected only later when a
 patient with presumed PM does not respond to therapy.
ASSOCIATION WITH
MALIGNANCIES
 The incidence of malignant conditions appears to be
 specifically increased only in patients with DM and not
 in those with PM or IBM.

 The most common tumors associated with DM are :
    ovarian cancer, breast cancer, melanoma, colon
   cancer, and non-Hodgkin lymphoma.
OVERLAP SYNDROMES
 sclerotic thickening of the
 dermis, contractures, esophageal
 hypomotility, microangiopathy, and calcium deposits
ASSOCIATION WITH VIRAL INFECTIONS

 Several viruses, including coxsackieviruses, influenza,
 paramyxoviruses, mumps, cytomegalovirus, and
 Epstein-Barr virus, have been indirectly associated with
 myositis.
Differential Diagnosis
 SUBACUTE OR CHRONIC PROGRESSIVE MUSCLE
 WEAKNESS : This may be due to denervating conditions
 such as the spinal muscular atrophies or amyotrophic
 lateral sclerosis
 ACUTE MUSCLE WEAKNESS : This may be caused by an
 acute neuropathy such as Guillain-Barr
 syndrome, transverse myelitis, a neurotoxin, or a
 neurotropic viral infection such as poliomyelitis or West
 Nile virus.
MYOFASCIITIS

NECROTIZING MYOSITIS

HYPERACUTE NECROTIZING FASCIITIS/MYOSITIS
(FLESH-EATING DISEASE)

DRUG-INDUCED MYOPATHIES
Treatment: Therapy of Inflammatory
Myopathies
1. Glucocorticoids. Oral prednisone is the initial treatment of choice;

2. Other immunosuppressive drugs. Eg Azathioprine, Methotrexate

3. Immunomodulation.

o Calcinosis, a manifestation of DM, is difficult to treat;

o IBM is generally resistant to immunosuppressive therapies.
  Prednisone together with azathioprine or methotrexate is often
  tried
THANK YOU

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Polymyositis dermatomyositis and inclusion body myositis

  • 1.
  • 2. POLYMYOSITIS, DERMATOMYOSITIS, AND INCLUSION BODY MYOSITIS: INTRODUCTION; The inflammatory myopathies represent the largest group of acquired and potentially treatable causes of skeletal muscle weakness. They are classified into three major groups: polymyositis (PM), dermatomyositis (DM), and inclusion body myositis (IBM).
  • 3. POLYMYOSITIS The actual onset of PM is often not easily determined, and patients typically delay seeking medical advice for several weeks or even months. This is in contrast to DM, in which the rash facilitates early recognition. PM mimics many other myopathies and is a diagnosis of exclusion.
  • 4. DERMATOMYOSITIS and INCLUSION BODY MYOSITIS DERMATOMYOSITIS DM is a distinctive entity identified by a characteristic rash accompanying, or more often preceding, muscle weakness. DM usually occurs alone but may overlap with scleroderma and mixed connective tissue disease. INCLUSION BODY MYOSITIS In patients 50 years of age, IBM is the most common of the inflammatory myopathies. It is often misdiagnosed as PM and is suspected only later when a patient with presumed PM does not respond to therapy.
  • 5. ASSOCIATION WITH MALIGNANCIES The incidence of malignant conditions appears to be specifically increased only in patients with DM and not in those with PM or IBM. The most common tumors associated with DM are : ovarian cancer, breast cancer, melanoma, colon cancer, and non-Hodgkin lymphoma.
  • 6. OVERLAP SYNDROMES sclerotic thickening of the dermis, contractures, esophageal hypomotility, microangiopathy, and calcium deposits
  • 7. ASSOCIATION WITH VIRAL INFECTIONS Several viruses, including coxsackieviruses, influenza, paramyxoviruses, mumps, cytomegalovirus, and Epstein-Barr virus, have been indirectly associated with myositis.
  • 8. Differential Diagnosis SUBACUTE OR CHRONIC PROGRESSIVE MUSCLE WEAKNESS : This may be due to denervating conditions such as the spinal muscular atrophies or amyotrophic lateral sclerosis ACUTE MUSCLE WEAKNESS : This may be caused by an acute neuropathy such as Guillain-Barr syndrome, transverse myelitis, a neurotoxin, or a neurotropic viral infection such as poliomyelitis or West Nile virus.
  • 9. MYOFASCIITIS NECROTIZING MYOSITIS HYPERACUTE NECROTIZING FASCIITIS/MYOSITIS (FLESH-EATING DISEASE) DRUG-INDUCED MYOPATHIES
  • 10. Treatment: Therapy of Inflammatory Myopathies 1. Glucocorticoids. Oral prednisone is the initial treatment of choice; 2. Other immunosuppressive drugs. Eg Azathioprine, Methotrexate 3. Immunomodulation. o Calcinosis, a manifestation of DM, is difficult to treat; o IBM is generally resistant to immunosuppressive therapies. Prednisone together with azathioprine or methotrexate is often tried