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Autoimmune disease

Clinical manifestations of scleroderma (systemic sclerosis)
  1. Skin
  1. The distinctive feature of scleroderma are striking cutaneous changes, notably skin thickening.
  2. A majority of the patients have diffuse sclerotic atrophy of the skin which usually begins in the fingers and distal regions of upper extremities and extends proximally to involve upper arm, shoulders, neck and face.
  3. In advanced stage, the fingers take on a tappered, clawlike appearance with limitation of motion in the joints and sometimes the tips of the fingers undergo autoamputation.
  4. Face becomes a drawn mask.
  1. Alimentary tract
  1. Most severe involvement is in esophagus.
  2. Lower third esophageal involvement develops a rubber hose inflexibility that causes gastroesophageal reflex.
  1. Raynaud's phenomenon
    1. It is episodic digital ischemia due to vasoconstriction of arteries and arterioles of the extremities, precipitated by cold.
    2. It is usually the first manifestation of disease.
  2. Musculoskeletal symptoms
  1. Inflammation of synovium (synovitis) with hypertrophy and hyperplasia of synovial tissues.
  2. Joint destruction is not common.
  3. Inflammatory myositis may develop in 10% of patients.
  1. Kidney
  1. The most prominent changes are in vessel walls and cause appearance similar to those of malignant hypertension, i.e. onion skin concentric proliferation of smooth muscles with thickening of intima, and fibrinoid necrosis.
  2. Hypertension may occur due to kidney involvement.
  3. Most common cause of death is development of malignant hypertension with subsequent renal failure.
  1. Lungs
  1. Pulmonary hypertension.
  2. Interstitial fibrosis.
  1. Heart
  1. Pericardial effusion / Pericarditis
  2. Myocardial fibrosis
  1. CREST syndrome (with limited systemic sclerosis).
SLE: Multisystem disease of autoimmune origin characterized principally by injury to skin, joints, kidney and serosal membranes.
Fundamental defect is a failure of the regulatory mechanism that sustain self tolerance
  1. Past history of spontaneous abortion, shrinking lung syndrome and coomb's positive hemolytic anemia are complication of SLE.
  2. Autoantibodies against red cells, white cells and platelets mediate their effects via type II hypersensitivity and visceral lesions are mediated by "type III.
  3. In tissues, nuclei of damaged cells react with ANAs which lose their chromatin pattern and become homogenous, to reduce so called LE bodies or Hematoxylin bodies.
  4. LE cell-it is a phagocytic leukocyte (macrophage or neutrophil) which engulfs the denatured nucleus of an injured cell.
Membranous glomerulonephritis almost always have severe proteinuria or the overt nephrotic syndrome.
Clinical features of SLE are:
  1. Hematologic                                                                                           
  2. Arthritis
  3. Butterfly malar rash                                                                               
  4. Fever, fatigue, weight loss
  5. Renal (various GN)                                                                                 
  6. Pleurisy and diffuse fibrosing alveolitis
  7. Myalgia, pericarditis, nonbacterial verrucous endocarditis           
  8. Raynaud phenomenon
  9. Peripheral neuropathy

Wire loop lesions are characteristic of type IV GN (Membranous GN)

MC cause of death are renal failure and intercurrent infections followed by diffuse CNS disease.

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