Anda di halaman 1dari 3

Systemic Lupus Erythematosus (SLE)

SLE is a complex disorder of multifactorial origin resulting from interactions among genetic, immunological, and environmental factors that act in concert to cause activation of helper Tcells and B cells and result in the production of several species of pathogenic autoantibodies Pathogenesis related to genetic predisposition and exogenous factors (drugs exposure, UV, estrogens), but the cause is unknown involved some basic defect in the maintenance of B-cell peripheral self tolerance defect in regulation B cell proliferation Helper T cell hyperactivity self antigen specific B cells to produce antibody Morphology Typical in all tissue is a type III hypersensitivity response with an acute necrotizing vasculitis and fibrinoid deposits, involving small arteries and arterioles. Ig dsDNA and C3 may be found within vessel walls - Kidney : lupus nephritis - Skin : butterfly rash (malar erythema) - Joints : non erosive synovitis - CNS : neuropsychiatric manifestation - pericarditis and other serosal cavity involvement - non bacterial verrucous (Libman-Sack) endocarditis - Spleen : onion skin appearance - Lung : pleural effusion, interstitial pneumonitis, diffuse fibrosing alveolitis Tissue damage occurs by formation of immune complexes (type III hypersensitivity) or by antibody-mediated injury (type II hypersensitivity)

Immunologic Deficiency syndromes


Subdivided into primary and secondary Primary immunodeficiency disorders are usually hereditary, typically manifesting between 6 months and 2 years of life as maternal antibody protection is lost Example : X-linked agamaglobulinemia of Bruton Common variable immunodeficiency Isolated IgA deficiency Hyper-IgM syndrome DiGeorge Syndrome (thymic hypoplasia)

Severe combined immunodeficiency disease Immunodeficiency with thrombocytopenia and eczema (Wiskot Aldrich syndrome) Genetic deficiencies of the complement system

Secondary immunodeficiencies result from altered immune function due to infections, malnutrition, aging, immunosupression, irradiation, chemotherapy or autoimmunity Example : AIDS (Acquired Immunodeficiency Syndrome ) AIDS is an infectious secondary form of immunodeficiency cause by HIV-1. It is characterized by profound suppression of T-cell-mediated immunity, opportunistic infections, secondary neoplasms and neurologic disease. Epidemiology : - transmission of HIV : fetus) sexual contact, parenteral inoculation, vertical transmission (mom to

- high risk groups : Homosexual/bisexual Blood/components men, intravenous drug abusers, hemophiliacs,

recipiens, other (heterosexual contact of high- risk groups) Natural history of HIV infection 1. early, acute phase - transient viremia, seeding of lymphoid tissue, CD4+ - clinically; a self limited acute illness with sore throat, myalgias and asepti Meningitis may develop - Clinical recovery and near normal CD4+ occur in 6-12 weeks 2. Middle, chronic phase - characterized by clinical latency with continued viral replication in lymphoid tissue, with gradual decline of CD4+ - no constitutional symptoms, patient may develop persistent generalized lymph node enlargement - this phase may last for 7-10 years - toward this end of this phase fever, rash, fatigue and viremia appear

3. Final progression to AIDS - characterized by rapid decline in host defenses manifested by low CD4+counts, weigt loss, diarrhea, opportunistic infections and secondary neoplasms. - Opportunistic infection : pneumocytitis carinii, candida, CMV, mycobacteria, HSV, cryptococcus, Toxoplasma - Malignant neoplasms : kaposi sarcoma, lymphoma (NHML) Morphology the tissue change are neither specific nor diagnostic

Anda mungkin juga menyukai