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AUTOIMMUNE DISEASES (PowerPoint)

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					AUTOIMMUNE
  DISEASES
 Ass. Sklyannaya E.V.
      SYSTEMIC LUPUS
      ERYTHEMATOSUS

   Systemic lupus erythematosus (SLE) is an
autoimmune disease in which organs, tissues,
and cells undergo damage mediated by tissue-
binding autoantibodies and immune
complexes.
                Epidemiology


   Ninety percent of patients are women of child-
    bearing years.



   Prevalence of SLE is 15 to 50 per 100,000.
                 Etiology

                              environmental
susceptibility                    factors
    genes                       (estrogen,
                             ultraviolet light)



                 abnormal
                  immune
                 responses
                Pathogenesis
   hypersensitivity of T and B lymphocytes
   production of pathogenic autoantibodies
   formation of immune complexes
   sequestration and destruction of Ig-coated
    circulating cells
   fixation and cleaving of complement proteins
   release of chemotaxins, vasoactive peptides,
    and destructive enzymes into tissues
                          Pathology
   In skin - deposition of Ig at the dermal-epidermal junction (DEJ),
    injury to basal keratinocytes, and inflammation dominated by T
    lymphocytes in the DEJ and around blood vessels and dermal
    appendages

   In renal biopsies - proliferative changes in mesangium and in
    glomeruli, predominantly membranous changes to scarred
    glomeruli

   In blood vessels - leukocytoclastic vasculitis

   In lymph node biopsies - nonspecific diffuse chronic
    inflammation.
         Clinical Manifestations
   Systemic Manifestations (fatigue, myalgias,
    arthralgias, fever, prostration, weight loss)
   Musculoskeletal Manifestations (polyarthritis,
    myositis)
   Renal Manifestations (Nephritis)
   Nervous System Manifestations (cognitive
    dysfunction, headaches, seizures, psychosis,
    myelopathy)
           Clinical Manifestations
   Cutaneous Manifestations
    (lupus dermatitis: discoid
    lupus erythematosus,
    systemic rash, subacute
    cutaneous lupus
    erythematosus)
                                    The classic malar rash, also known as
                                     a butterfly rash, with distribution over
                                     the cheeks and nasal bridge. Note that
                                     the fixed erythema, sometimes with
                                     mild induration as seen here,
                                     characteristically spares the nasolabial
                                     folds. Photosensitive SLE rashes
                                     typically occur on the face or
                                     extremities, which are sun-exposed
                                     regions.
         Clinical Manifestations
   Vascular Occlusions
   Pulmonary Manifestations
   Cardiac Manifestations
   Hematologic Manifestations
   Gastrointestinal Manifestations
   Ocular Manifestations
                      Workup
   Tests for Autoantibodies Diagnostically, the most
    important autoantibodies to detect are ANA since the
    test is positive in >95% of patients, usually at the
    onset of symptoms. High-titer IgG antibodies to
    double-stranded DNA (dsDNA) (but not to single-
    stranded DNA) are specific for SLE.
   Standard Tests for Diagnosis Screening tests for
    complete blood count, platelet count, and urinalysis
    may detect abnormalities that contribute to the
    diagnosis and influence management decisions.
            Diagnosis
   1. Malar rash.
   2. Discoid rash.
   3. Photosensitivity.
   4. Oral ulcers.
   5. Arthritis.
   6. Serositis.
   7. Renal disorder.
   8. Neurologic disorder.
   9. Hematologic disorder.
   10. Immunologic disorder.
   11. Antinuclear antibodies.
               Differentials
    Antiphospholipid Syndrome, Fibromyalgia,
Hepatitis C, Infectious Mononucleosis, Infective
Endocarditis, Lyme Disease, B-Cell Lymphoma,
Mixed Connective-Tissue Disease, Polyarteritis
Nodosa, Preeclampsia (Toxemia of Pregnancy),
Rheumatic Fever, Rheumatoid Arthritis,
Scleroderma, Serum Sickness, Thrombotic
Thrombocytopenic Purpura, Undifferentiated
Connective-Tissue Disease, Drug-induced lupus
erythematosus, Vasculitis, Leukemia, Neoplasia,
HIV, Multiple sclerosis, Parvovirus or other viral
infections.
     Treatment
   Analgesics

   antimalarials

   glucocorticoids

   cytotoxic drugs
               Prognosis

    Survival in patients with SLE is 90 to 95%
at 2 years, 82 to 90% at 5 years, 71 to 80% at
10 years, and 63 to 75% at 20 years.
   SYSTEMIC SCLEROSIS
    Systemic sclerosis (SSc) is a chronic
multisystem disorder of unknown etiology
characterized clinically by thickening of the
skin caused by accumulation of connective
tissue and by structural and functional
abnormalities of visceral organs, including the
gastrointestinal tract, lungs, heart, and kidneys.
            Epidemiology
    The estimated incidence of systemic
sclerosis is 19 cases per million population,
and the prevalence of systemic sclerosis has
been estimated at 240 cases per million
population, although reported prevalence has
ranged from 138 to 286 cases per million
population. The risk of systemic sclerosis is 3-
9 times higher in women than in men. The
peak onset occurs in individuals aged 30-50
years.
       Etiology
   1. Genetic abnormalities.
   2. Infectious agents.
   3. Environmental factors.
   4. Drugs.
Pathogenesis
                         Pathology
   Systemic sclerosis is
    characterized by excessive
    fibrosis in the skin and other
    affected organs. The skin
    and lungs also show
    prominent T-lymphocyte
    infiltration. A severe
    fibroproliferative
    vasculopathy that affects
    small arteries and arterioles
    is universally present in
    affected organs. Platelet
    microthrombi are often
    found in the lumen of the
    narrowed vessels.
                 Classification
1. Systemic sclerosis:
 Limited cutaneous disease

 Diffuse cutaneous disease

 Sine scleroderma

 Undifferentiated connective tissue disease

 Overlap syndromes

2. Localized scleroderma
 Morphea

 Linear scleroderma (en coup de sabre)
            Clinical Manifestations
                 Skin Features


   Tightening of the skin
    in the face, with a
    characteristic beaklike
    facies and paucity of
    wrinkles
            Clinical Manifestations
                 Skin Features


   Sclerodactyly with
    digital ulceration, loss
    of skin creases, joint
    contractures, and sparse
    hair
           Clinical Manifestations
                Skin Features

   Anterior chest
    demonstrating salt-and-
    pepper
    hypopigmentation and
    diffuse
    hyperpigmentation in a
    white woman
         Clinical Manifestations
   Raynaud’s Phenomenon
   Musculoskeletal Features
   Gastrointestinal Features
   Pulmonary Features
   Cardiac Features
   Renal Features
Workup
                      Workup
   CT scan
   Radiography
   Echocardiography
   Right-heart catheterization
   Esophagraphy
   Pulmonary function testing
   Serum N-terminal pro-brain natriuretic peptide
   Cardiac rhythm monitoring
   Esophagogastroduodenoscopy
   Biopsy of skin and lungs
                   Diagnosis
  Preliminary criteria of systemic sclerosis by
  the American Rheumatism Association:
major criterion sclerodermatous involvement
  proximal to the digits
minor criteria: 1)sclerodactyly,
2)digital pitting scars or tissue loss of the volar
  pads of the fingertips, and
3)bibasilar pulmonary fibrosis.
                   Differentials
   Eosinophilia, Eosinophilia-Myalgia Syndrome,
    Eosinophilic Fasciitis, Graft Versus Host Disease,
    Mycosis Fungoides, Primary Biliary Cirrhosis,
    Pulmonary Hypertension, Primary, Reflex
    Sympathetic Dystrophy, Scleroderma. Other
    Problems to be Considered: Toxic oil syndrome
    (adulterated rape seed oil), Porphyria cutanea tarda,
    Digital sclerosis of diabetes mellitus, Vibration
    disease, Radiation exposure, Intestinal obstruction,
    Infiltrative cardiomyopathy, Nephrogenic fibrosing
    dermopathy (nephrogenic systemic fibrosis),
    Amyloidosis, Scleromyxedema (generalized lichen
    myxedematosus), Scleredema diabeticorum
                 Treatment
   D-penicillamine,
   colchicine,
   IFN-γ,
   IFN-α,
   recombinant human relaxin,
   antiplatelet therapy,
   glucocorticoids,
   calcium channel blockers
                     Prognosis
   Pulmonary hypertension and scleroderma renal crisis
    are the most frequent causes of mortality. Survival
    averages 12 years from diagnosis and correlates best
    with the clinical disease subtype (diffuse cutaneous vs
    limited cutaneous) and extent of organ involvement.
   The limited cutaneous subset carries a 10-year
    survival rate of 71%. The diffuse cutaneous subset
    carries a 10-year survival rate of 21%. Pulmonary
    hypertension is a major prognostic factor for survival.

				
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posted:10/31/2011
language:English
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