Autoimmunr Diaseases

AUTOIMMUNE DISEASES

May be humoral or cell-mediated.

4 theories as to why control mechanisms become deranged:

1. Sequestered antigen (body never had chance to develop tolerance)
2. Antigen modification—modified antigen no longer recognized as self, therefore attacked.
3. Cross-reactive antigen. An antibody made to a foreign invader (e.g., streptococcus)
4. Alteration in lymphoid system: balance between T helper and suppressor cells is impaired, leading to out of control system. 

 

SYSTEMIC TYPES OF AUTOIMMUNE DISEASE 

Systemic Lupus Erythematosus (SLE)

• appears to be strongly associated with antinuclear antibodies (especially to native) doublestranded) DNA.
• generally occurs in young females (age 20-40)

Features

articular pain, fever, skin rash (especially in butterfly facial distribution)

renal disease (glomerulonephritis: most common subtype is diffuse proliferative), pleuritis and pleural effusion, pericarditis neurological disease (seizures and/or psychosis), lymphadenopathy and splenomegaly, hemolytic anemia (autoimmune)

• there is a “variant” called chronic discoid lupus erythematosus which s usually FANA negative, but has skin disease resembling SLE. Only a few cases of discoid lupus progress to SLE.
• a SLE-like syndrome (including +FANA) can be induced by drugs (especially the antiarrhythmic procainamide and the antihypertensive drug hydralazine). Usually remits after the drug is withdrawn.

 

Scleroderma (Progressive Systemic Sclerosis)

• associated with antinucleolar antibodies, other antibodies
• usually middle-aged women
• see fibrosis of multiple organs, especially the dermis of the skin; may also see submucosal, fibrosis of esophagus, GI tract; thickening of renal injury and hypertension; and pulmonary fibrosis. May also be associated with joint pain.

Polyarteritis Nodosa 

 

• necrotizing vasculitis affecting medium or small arteries in any part of the body.
• involvement most common in kidneys (85%), where it may be associated with focal glomerulonephritis; heart (75%); liver (65%), GI tract (50%); but it can be seen almost anywhere (although pulmonary circulation is usually spared).
• most common clinical presentation is fever and renal manifestations due to renal vessel involvement (hypertension, renal failure). May cause infarcts elsewhere.
• Sarcoidosis (not necessarily autoimmune disease, but where else to put it?)
• numerous non-caseating granulomas of unknown etiology which can occur anywhere in the body.
• most common site of involvement is hilar lymph nodes (bilateral hilar adenopathy on CXR in young women is often sarcoid).
• generally disease of young women. More common in blacks (10x incidence). 

Goodpasture Syndrome

• antibodies to glomerular basement membrane
• damage to glomerular basement membrane leads to crescentic, rapidly progressive glomerulonephritis and damage to alveolar basement membrane leads to hemoptysis, pulmonary hemorrhage.

Wegeners Granulomatosis

• like polyarteritis nodosa, no specific antigen-antibody complex has been found.
• see acute necrotizing granulomas of upper and lower respiratory tracts (lung, nose, and sinuses, generally in young to middle aged individuals.
• also see focal necrotizing vasculitis, also most common in upper airways and lungs
• associated renal disease in the form of focal or diffuse necrotizing glomerulonephritis.

Sumber : Bpk. Dr. Iskandar Zulkarnain

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