Charts of Rheum 2 Flashcards
Drug induced Lupus (DIL) type of immune injury?
Type 2 and 3
DIL manifestation?
Immune complex, disease of vessels, glomeruli
Loss of blood cells
Renal and CNS involvement UNCOMMON
Pathogenesis of Drug induced lupus?
Drug reaction to hydrazine, procainamid, isoniazid, D-penicillamine
ANA/HLA associations of DIL?
Antihistones
HLA-DR4
Discoid lupus (DL) pathogenesis?
Dysregulation of immune response/UV light
Immune mechanism of DL?
Immune comples and complement deposits along skin
Type of immune injury is DL?
Type 3
Manifestation of DL?
Limited to skin
Rare systemic symptoms
Diffuse scleroderma (DS) pathogenesis?
Excessive deposition of collagen due to immune activation
Immune mechanism of DS?
CD4 cells stimulate cytokine production and fibrosis in skin ad other organs.
There is also blood vessel fibrosis
HLA/ANA associations for DS?
DNA topoisomerase 1 (Scl-70)
Type of immune injury is DS?
Type 4 due to Cytokine stimulation of fibrosis, endothelial activation
Manifestations of DS?
Fibrosis of dermis, esophagus, lungs
Vascular injury to kidneys
Pathogenesis of Limited Scleroderma (CREST)?
Similar to DS but limited to skin of fingers, forearms, and face
Also calcification of subcutaneous tissues
Immune mechanism of CREST?
CD4 cells stimulate cytokine production and fibrosis in skin, organs, and blood vessels
HLA/ANA associations of CREST?
Anti-centromere
Type of immune injury is CREST?
Cytokine stimulation of fibrosis, type 4
Manifestations of CREST?
Calcinosis Raynaud's Esophageal dysfunction Sclerodactyl Telangiectasia
Sjogrens syndrome pathogenesis?
Tissue specific response triggered by retrovirus HTLV or EBV infection
Immune mechanism of Sjogren?
CD4 response to auto antigen such as cytoskeletal protein alpha fodrin
HLA/ANA associations of Sjogren?
Anti-RNP (Ribonucleoprotein)
Multiple HLA-DR and DQ
Type of immune injury for Sjogren?
Type 4
Manifestation of Sjogren?
Cell mediated destruction of lacrimal and salivary glands with fibrosis
Pathogenesis of Dermatomyositis?
Activation of CD4 cells leading to antibody formation
Immune mechanism of Dermatomyositis?
Antibody and complement mediated vasculitis with secondary loss of muscle fibers
Type of immune injury for Dermatomyositis?
Type 3
Manifestation of Dermatomyositis?
Loss of small vessels in muscle with ischemic necrosis of muscle fibers
Pathogenesis of Polymyositis?
Activation of CD8 cells
Immune mechanism of Polymyositis?
Cell mediated destruction of myofibers
Type of immune injury for Polymyositis?
Type 4
Manifestation of Polymyositis?
Necrosis of muscle fibers
Pathogenesis of Inclusion body myositis (IBM)?
Activation of CD8 cells
Immune mechanism of IBM?
Unknown and immunosuppressive therapy isn’t helpful
Type of immune injury for IBM?
Also unknown
Manifestation of IBM?
Necrosis of muscle fibers
Secondary immune response