MAN IN DISEASE

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• MAN IN DISEASE
• SYSTEMIC LUPUS ERYTHEMATOSUS
• PRESENTED BY
• DR SMITHA
  VELAYUDHAN

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• It is an inflammatory disease of autoimmune
  nature involving the connective tissue of several
  organ
• Two forms of SLE
• 1.Systemic or Disseminated form
• 2.Discoid form
• Male to female proportion is 118. More than 60
  of cases are between the ages of 30 and 60.

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• ETIOLOGY
• Exact etiology unknown.
• Genetic factors Predisposition to develop
  autoantibodies to nuclear and cytoplasmic
  antigens in SLE is due to immunoregularity
  functions of class II HLA. HLA B8 and DR3
  inherited deficiency of C1,C4,C2 complements.

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• Other factors are
• Certain drug-egPenicillamin D
• Viral infections-egEBV infections
• Hormones-egoestrogens

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• PATHOLOGY
• . Four types of histological pictures are seen.
  They are fibrinoid change, collagen sclerosis,
  formation of hematoxylin bodies and inflammatory
  changes in arterioles and capillaries. Tissue
  damage occurs in two different way
• Direct cytotoxicity(Type 2 reaction) caused by
  antibody and complement.
• Type 3 immune reaction caused by immune complexes
  and complement

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• PATHOGENESIS
• Type 2 reaction is characterized by formation of
  autoantibodies against blood cells(Rbc,Platelets
  leucocytes) and results in haematological
  derangement in SLE
• Type 3 reaction is characterized by an
  antigen-antibody complex which is deposited at
  sites such as renal glomeruli, walls of small
  blood vessels etc.

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• LE cell phenomenon
• This was the first diagnostic laboratory test
  described for SLE.
• LE cell is a phagocytic leucocyte, which engulfs
  the homogenous nuclear material of the injured
  cell.
• If this mass is engulfed by a neutrophil,
  displacing the nucleus of neutrophil to the rim
  of the cell, it is called LE cell.

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• CLINICAL FEATURES
• Presenting symptom is fever which may take
  several forms, from mild subacute illness to
  severe hectic fever.
• Arthritis and arthralgia-joint manifestations
  occur in 90 of subjects with SLE in the early
  stages. Large joints are mostly affected.
  Jaccouds arthropathy may affect the hand in up
  to 50 of cases. It is characterized by
  reducible, non-erosive joint deformities with
  preservation of hand function. Permanent
  deformities do not occur.

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• Skin lesions- occurs in 65 of cases. Classic
  lesion is the erythematous, photosensitive
  butterfly rash affecting the cheeks and nose.
  This is diagnostic. Discoid lupus, maculopapular
  rashes and purpura may occur in some. Frontal
  baldness may develop. Alopecia, painful ulcers in
  mouth and pharynx.

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• Cardiac- occurs in 25 to 40 of cases. Lesions
  include pericarditis, pericardial effusion,
  myocarditis with cardiac failure, and valvulitis
  involving the mitral valve. Libman-sacks
  endocaritis is a non-bacterial verrrucous
  endocarditis affecting the mitral valve. It
  results in mitral incompetence.

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• Respiratoty Occurs in 30 of cases. Lesions are
  dry pleurisy, pleural effusion, fibrosing
  alveolitis and lupus pneumonitis. A syndrome of
  shrinking lungs is uncommon, but virtually
  pathognomonic of SLE, characterized by
  progressive reduction in lung volume with
  evidence of diaphragmatic weakness.

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• Kidney lesion occurs in 60 of cases. Renal
  involvement used to be the most important lesion
  deciding the prognosis. Symptoms may vary.
  Sometimes asymptomatic with only proteinuria for
  long intervals or may manifest as nephritic
  syndrome, haematuria, acute nephritic syndrome or
  renal failure.

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• Nervous system Lesions in about 40 of cases, is
  generally a later manifestation even though
  psychiatric symptom may occur early. Parents with
  the aniphospholipid antibodies are more prone to
  develop Stroke. Other manifestations are
  Psychoses, convulsions, cranial nerve palsies,
  peripheral neuropathy, chorea, cerebellar
  disturbances.

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• Muscle involvement Myalgia,polymyositis and
  muscle wasting.
• Haematology Lesions involve normocytic
  normochromic anaemia, Coombs positive autoimmune
  haemolytic anaemia, leucopenia and
  thrombocytopenia.
• Eyes Lesions include dryness of the conjunctiva
  and retinal abnormalities such as haemorrhage and
  exudates known as cytoid bodies.

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• Diagnosis of SLE may be obvious in a young
  women with a butterfly rash over the face,
  fever, pain but no deformities, one or more
  peripheral joints, pleuritic chest pain and
  photosensitivity. But in some the presentation of
  SLE is subtle and puzzling, taking form such as
  febrile illness of an unknown origin, abnormal
  urinary findings or neuropsychiatric
  manifestations including psychosis.

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• A variety of clinical findings point towards
  renal involvement including haematuria, red cell
  casts, proteinuria, in some cases the classic
  nephritic syndrome. Renal failure occurs,
  especially in patients with diffuse proliferative
  or membranous glomerulonephritis or both. ANAs
  can be in 100 of patients but it can be present
  in patients with other autoimmune disorder.
  Antidouble stranded DNA antibody are considered
  highly diagnostic of SLE

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• The cause of SLE is extremely variable. But some
  unfortunate individuals follow a progressively
  down hill course to death within months. More
  often the disease is characterized by flare-ups
  and remissions. Acute attacks are usually
  controlled by adrenocortical steroids or
  immunosuppressive drugs. Overall the 10 year
  survival rate is approximately is 70. Renal
  failure, inter current infections and diffuse CNS
  involvement are major cause of death.

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• LABORATORY INVESTIGATIONS
• SLE gives rise to an array of abnormalities, some
  being specific and the other non specific. The
  non specific features include, markedly elevated
  ESR(above 100mm), moderate anaemia, leucopenia,
  thrombocytopenia and moderate to severe
  proteinuria.

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• DIAGNOSIS
• The diagnosis of SLE should be suspected if there
  are febrile episodes with multisystem involvement
  high ESR, not responding to general lines of
  treatment.
• The American Rheumatism assosciation has laid
  down criteria for diagnosis of SLE.

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• The 11 criteria used for diagnosing systemic
  lupus erythematosus are
• malar (over the cheeks of the face) "butterfly"
  rash
• discoid skin rash patchy redness that can cause
  scarring
• photosensitivity skin rash in reaction to
  sunlight exposure
• mucus membrane ulcers ulcers of the lining of
  the mouth, nose or throat

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• arthritis two or more swollen, tender joints of
  the extremities
• pleuritis/pericarditis inflammation of the
  lining tissue around the heart or lungs, usually
  associated with chest pain with breathing
• kidney abnormalities abnormal amounts of urine
  protein or clumps of cellular elements called
  casts

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• brain irritation manifested by seizures
  (convulsions) and/or psychosis
• blood count abnormalities low counts of white or
  red blood cells, or platelets
• immunologic disorder abnormal immune tests
  include anti-DNA or anti-Sm (Smith) antibodies,
  falsely positive blood test for syphilis,
  anticardiolipin antibodies, lupus anticoagulant,
  or positive LE prep test

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• antinuclear antibody positive ANA antibody
  testing
• In addition to the 11 criteria, other tests can
  be helpful in evaluating patients with SLE to
  determine the severity of organ involvement.
  These include routine testing of the blood to
  detect inflammation (for example, a test called
  the sedimentation rate), blood chemistry testing,
  direct analysis of internal body fluids, and
  tissue biopsies. Abnormalities in body fluids and
  tissue samples (kidney, skin, and nerve biopsies)
  can further support the diagnosis of SLE.

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• MANAGEMENT
• Since there is no curative measures, the main aim
  is to induce remission and prevent relapses.
  General management consists of avoidance of
  direct sunlight and offending drugs and prompt
  attention to complications.

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MIASMATIC CLEAVAGE

• Fundamental defect in SLE seems to be a failure
  to maintain self tolerance. As a result a wide
  array of autoantibodies that damage tissue is
  produced-SYPHILITIC
• .Pain in joints-SYPHILITIC
• Lupus-TRIMIASMATIC
• Baldness-PSORA

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• Painful ulcers in mouth and pharynx-SYPHILIS
• Valvular affections-PSORASYCOSIS
• Hematuria-TUBECULAR

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MEDICINES

• 3-Mark remedies
• ARS ALB,LYCO,NIT ACID,THUJA

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• THANK YOU