Term
| What are the criteria used to diagnose Lupus in clinical studies |
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Definition
Malar rash
Discoid Rash
Oral Ulcers Painless
Photosensitivity
Serositis
Arthritis
Neurological
Hematologic
Renal
Immunologic-anti-Cardiolipin/SM/DNA, lupus Anti-coag, An
ANA |
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Term
| What type of hypersensitivities are seen in lupus |
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Definition
Type 2: hematologic
Type 3: Visceral
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Term
| What is a complication of lupus associated with pregnancy |
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Definition
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Term
| What are the signs of Sjogren's Syndrome and how is it diagnosed? |
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Definition
Keratoconjunctivitis sicca
Xerostomia
Dx: Lip biopsy with pervascular/ductal follicles
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Term
| What are the associations of sjogren's disease |
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Definition
SSA(Ro), SSB(La), LE Test, ANA, RF
HLA-DR3,DQA1,DQB1(Primary)
HLA-DR4 (Secondary)
Epstein Barr
B-cell marginal Zone Lymphoma |
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Term
| What is Mikulicz's syndrome and what autoimmune disease falls under this category |
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Definition
Lacrimal and Salivary enlargement
Sjogren's |
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Term
| How does Systemic Sclerosis present |
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Definition
Diffuse: fibrosis of skin, renal, cardiac, pulmonary, interstitial
Localized: Calcinosis, Raynaud's Esophogeal Dysmotility, Sclerodatyly,Telengiectasias |
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Term
| Pathogenic hypotheses of Systemic Scleroderma |
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Definition
Immunological: T-cell mediated
Vascular: Endothelial Injury-> Fibroblast activation->Ischemia |
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Term
| Antibodies associated with Systemic Scleroderma |
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Definition
Anti-Sc170"Anti-DNA Topoisomerase 1"(Pulm Fibrosis, PVD)
Anticentromeric Antibody (CREST Syndrome) |
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Term
| WHO classification of Lupus Nephritis |
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Definition
I: Normal light, With IG, deposits
II: Mesangial
III: Focal Proliferative GN
IV: Diffuse Proliferative GN (Wire Loops)
V: Membranous
VI: Tuberal/Interstitial |
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Term
| What are the multisystem manifestations of Systemic Sclerosis |
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Definition
Skin: Loss of rete pegs, lead to autoamputation
GI: Barrett's Esophogus, Rubber Hose-> Reflux
Synovitis
Kidney: Appears like malignant hypertension
Lung: Intersitial Fibrosis
Heart: Pericarditis |
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Term
| What are the main causes of morality in Lupus |
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Definition
Renal Failure
Infection
Coronary Artery Disease |
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Term
| Where are immune complexes found in Chronic Discoid Lupus Erythematosis |
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Definition
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Term
| What patients typical show the Subacute Cutaneous Lupus Erythematosus |
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Definition
| Patients with Anti-SSA and HLA-DR3 |
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Term
| What patients have a greater risk for hydralazine-induced lupus like syndrome |
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Definition
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Term
| What autoantibodies typically develop while taking medications that induce a lupus like syndrome? |
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Definition
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Term
| What does the pattern of the ANA results indicate |
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Definition
Homogeneous: Chromatin, Histone, dsDNA
Rim/Peripheral: dsDNA
Speckled: Non-DNA Nuclear components
Nucleolar: Nucleolar RNA (Systemic Sclerosis) |
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Term
| What is the cause and what are the signs of Dermatomyositis |
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Definition
Anti-capillary antibody-> Myocyte Necrosis
Lilac Skin rash (Heliotrope)
Periorbital Edema
Grotton's Lesions: Scaley knuckles
Proximal muscle weakness
Women: may get visceral cancer
Children: GI ulcers, soft tissue calcinosis |
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Term
| What is Polymyositis and who gets it |
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Definition
Adults
T-cell mediated CD8 myositis w/o vascular destruction: No skin lesions
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Term
| What is Inclusion body myositis and who typically gets it and how is it diagnosed |
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Definition
Older Adults
T-cell autoimmunity causing Myocyte inclusion bodies
Dx: Frozen Section to preserve inclusion bodies |
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Term
| What is Mixed connective tissue disease and how is it treated |
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Definition
Mixed clinical picture of SLE, Polymyositis, Systemic sclerosis with high Anti-RNP-particle containing U1 RNP Anti-bodies
Good response to steroids |
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Term
| What are the mechanisms of peripheral tolerance |
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Definition
Clonal deletion by Apotosis(Fas-Fas L)
Clonal Anergy
T-cell Peripheral Suppression(Treg) |
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Term
| What are the Immunologic disorders associated with SLE used for a research diagnosis |
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Definition
Anti-DNA
Anti-Sm
Anti-Cardiolipin
Lupus Anti-coagulant
False Positive Syphillis |
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Term
| What ANA pattern is most associated with Systemic Sclerosis |
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Definition
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Term
| What Anti-nuclear anti-body is associated with the incidence of renal disease in lupus? |
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Definition
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Term
| What antibody is typically shared by RA and sjogren's syndrome? |
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Definition
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Term
| What is the difference in presentation between inclusion body Myositis and Polymyositis? |
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Definition
Inclusion: Distal Muscle Weakness
Polymyositis: Proximal Muscle Weakness |
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