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Mixed Connective Tissue Disease
by
Farshid Mokhberi
Shahid beheshti University Of Medical Sciences & health services
Definition
• Mixed connective-tissue disease (MCTD) was first
recognized by Sharp and colleagues (1972) in a group
of pati...
Pathophysiology
• B-lymphocyte hyperactivity
• T-lymphocyte activationin
• Apoptotic modification of the U1-70 kd antigen
...
Etiology
• The fundamental cause of MCTD remains unknown.
• Autoimmunity to components of the U1-70 kd snRNP
are a hallmar...
Differential Diagnoses
• Bacterial Sepsis
• Dermatomyositis
• Polymyositis
• Primary Pulmonary Hypertension
• Raynaud Phen...
Diagnosis
• High titers of anti-U1-RNP antibody, of SLE,
scleroderma, and inflammatory myositis.
• High-titer speckled pat...
Diagnostic Criteria
Diagnostic Criteria
Imaging Studies
• Chest radiography : infiltrates, effusion, or
cardiomegaly
• Echocardiography: effusion ,pulmonary
hyper...
Treatment
• MCTD:
extremely responsive to Glucocorticoids
Prognosis
 Mortality:
• Progressive pulmonary
hypertension and its cardiac
complications.
• Pulmonary hypertension due to...
•Thanks for your attention
Mixed Connective Tissue Disease By Farshid Mokhberi
Mixed Connective Tissue Disease By Farshid Mokhberi
Mixed Connective Tissue Disease By Farshid Mokhberi
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Mixed Connective Tissue Disease By Farshid Mokhberi

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Mixed Connective Tissue Disease By Farshid Mokhberi

  1. 1. Mixed Connective Tissue Disease by Farshid Mokhberi Shahid beheshti University Of Medical Sciences & health services
  2. 2. Definition • Mixed connective-tissue disease (MCTD) was first recognized by Sharp and colleagues (1972) in a group of patients with overlapping clinical features: Systemic lupus erythematosus Scleroderma Myositis
  3. 3. Pathophysiology • B-lymphocyte hyperactivity • T-lymphocyte activationin • Apoptotic modification of the U1-70 kd antigen • Immune response against apoptotically modified self-antigens • Genetic association with major histocompatibility genes human leukocyte antigen (HLA)–DRB1*04/*15 • Vascular endothelial proliferation with widespread lymphocytic and plasmacytic infiltration of tissues
  4. 4. Etiology • The fundamental cause of MCTD remains unknown. • Autoimmunity to components of the U1-70 kd snRNP are a hallmark of disease. Anti-RNP antibodies can precede overt clinical manifestations of MCTD but after 1 year. • The loss of T-lymphocyte and B-lymphocyte tolerance, abnormalities of apoptosis, or molecular mimicry by infectious agents, and driven by U1-RNA-induced innate immune responses, are proposed current theories of pathogenesis.
  5. 5. Differential Diagnoses • Bacterial Sepsis • Dermatomyositis • Polymyositis • Primary Pulmonary Hypertension • Raynaud Phenomenon • Rheumatoid Arthritis • Scleroderma • Systemic Lupus Erythematosus (SLE)
  6. 6. Diagnosis • High titers of anti-U1-RNP antibody, of SLE, scleroderma, and inflammatory myositis. • High-titer speckled pattern fluorescent antinuclear antibody (FANA) is typical of MCTD • Anti-RNP antibodies are required for diagnosis of MCTD.The presence of anti–U1-70 kd is characteristic of MCTD. • MCTD can enter sustained remission later in the clinical course. Anti-RNP autoantibodies typically become undetectable in patients in remission.
  7. 7. Diagnostic Criteria
  8. 8. Diagnostic Criteria
  9. 9. Imaging Studies • Chest radiography : infiltrates, effusion, or cardiomegaly • Echocardiography: effusion ,pulmonary hypertension • Ultrasonography/CT scanning :abdominal pain (indicated for evidence of serositis, pancreatitis, or visceral perforation related to vasculitis) • MRI - Used to assess neuropsychiatric signs or symptoms
  10. 10. Treatment • MCTD: extremely responsive to Glucocorticoids
  11. 11. Prognosis  Mortality: • Progressive pulmonary hypertension and its cardiac complications. • Pulmonary hypertension due to scleroderma-like vasculopathy can lead to death in a few weeks. • Myocarditis • Renovascular hypertension and cerebral hemorrhage  Morbidity: • recurrent musculoskeletal pain • low energy levels • Flares of polymyositis • glucocorticoid use: aseptic necrosis, vertebral compression fractures, cataracts, weight gain, accelerated atherosclerosis, nosocomial infections, and proximal myopathy.
  12. 12. •Thanks for your attention

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