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What is marker for drug-induced lupus?
antihistone ab w/o other autoabs. Associated with hydralazine and procaniamide
Marker for Mixed connective tissude disease
antibody to ribonucleoprotein (RNP)
HLA group for RA
- Get rhemutaoid arthritis in your 40s
HLA group for DMI
- I had to wake up Nat Granger 3 to 4 times
SLE HLA link
- Get SLE when you are a 23 y/o F.
Celiac Disease Associated HLA
- DQ2, or DQB
- QUIT eating bread!!
HLA associated with Graves Disease
- Bummer to have this Grave disease.
What is HLA allele for MS?
- It causes two diseases, MS and Goodpasture
What is HLA for Goodpasture?
- It causes two diseases MS and Goodpasture syndrome
What diseases associated with DR2?
- MS and Goodpasture Syndrome
- It casues 2 diseases!
- Autoimmune, inflammatory disorder affecting:
- synovial joints with pannus formation in joints (MCP, PIP)
- subcutaneous rheumatoid nodules (fibrinoid necoris surrounding histiocytes)
- Ulnar deviation
- Baker's Cyst
- NO DIP involvement
- Females > Males
- Type III hypersensitivity (immune comlex)
- Most have positive RA factor (anti IgG antibody which is IgM) specific but not sensitive.
Presentation: morning stiffness lasting >30 minutes, improving with use, symmetric joints, systemic symptoms (fever, fatigue, pleuritis, pericarditis)
Boutonniere deformity, swan-neck deformity, and z-thumb deformity.
- Xerophthalmia (dry eyes, conjunctivitis, sand in my eyes)
- Xerostomia (dry mouth, dysphagia)
- Parotid enlargment, Increased risk of B-cell lymphoma
- SS-A (ro) SS-B (La)
- Females 40-60 years old
- Assoc. with RA
dry eyes, dry mouth, nasal and vaginal dryness, chronic brnchitis, reflux esophagitis, but no arthritis.
Deposition of calcium pyrophosphate crystals in joint space. Basophilicm rhomboid crystals. Weakly positively birefringent
usually effects large joints (classically knee)
- Greater than 50 years of age, both
- sexes. No Tx!
four h's: hyperPTH, hemochromatosis, hypophosphatemia, hypomagnesia
Contrasting Gout and Pseudogout crystals
Gout= Needle shaped. Yellow w/ parallel, blue with perpendicular, negatively birefringment
Pseudogout = Rhomboid crystals, blue w/parallel light, and yellow with perpendicular light.
- IM DAMN SHARP
- ANA = Sensitive, not specific
- anti-dsDNA = specific, poor prognosis.
- Anti-Sm (smith) = specific, no prognostic value
- Antihistone = Drug induced
- Malar Rash
- Discoid Rash
- Mucositis (oropharyngeal ulcers)
- Neurologic disorders
- Serositis (pleuritis, pericarditis)
- Heamtologic Disorders (thrombocytopenia (short life span), hypercoaguability (need warfarin), but can show prolonged PTT due to rxn to phospolipis used to determine PTT)
- Renal (Immune complex deposition, nephritic)
- Libman-Sacks endocarditis
False RPR/VDRL syphilis test due to antiphospholipid antibodies that react with cardiolipin.
90% females between 14-45, worse in African Americans
Characterized by immune-mediated, widespread noncaseating granulomas, elevate ACE. Common in Black females
- RA factor
- ACE increase
- Interstitial firbosis
- Non-caseating granulomas.
Assocations: restrictive lung disease, bilateral hilar lymphadenopathy, erythema nodosum, Bell's palsy, epithelila granulomas containing microscopic Schaumann and asteroid bodies, uveoparotitis, hypercalcemia due to Vit. D activation from epitheloid macrophages.
Treated with steroids!
Pain and stiffness in shouldres and hips, often with fever, malaise, and weight loss. Does not cause muscular weakness. Elderly patients.
- Assoc. with temporal arteritis
- Normal CK but elevated ESR.
- Tx: Prednisone
Progressive symmetric proximal muscle caused by CD8 damage of myofibers. Often involves shoulders. Dx with muscle biopsy with evidence of perifascicular inflammation.
- Similar to polymyositis, but involves malar rash helitropic. "Mechanic's hands" and increased risk of internal malignancy only in adults.
- Most common neuromuscular junction disorder. Autoantibodies to postsynaptic AChR causes ptosis, diplopia, and general weakness. Assciated with thymoma. Symtoms worsen with muscle use.
- Revs. of symptoms occur with AChE inhibitors (edrophonium test)
What is the AChE inhibitor used to treat myasthenia gravis?
Autoantibodies to presynaptic Ca+2 channel results in decreased in ACh release leading to proximal muscle weakness. Associated with paraneoplastic disorders such as small cell lung cancer.
Symptoms improve with use (contrast with myasthenia gravis) and do not get better with AChE inhibitors alone.
- excessive fibrosis and collagen deposition throughout the body. Commonly sclerosis of skin, manifesting as puffy and taught skin with no wrinkles. Sclerosis of renal pulmonary, CV and GI symptoms. 2 major types:
- Diffuse = Associated with anti-Scl-70 ab
- CREST = Calcinosis, Raynaud's, Esophageal dysmotility, Sclerodactyly, tealngiectasia. Limited skin involvement. Often confined to fingers and face. Anticentromere Ab.
DMARD. Stabilizes lysosomes, reduces chemotaxis, also an antimiral.
S/E: Ophthalmic abnormalities, derm rxns, hematoxicity, GI
Methotrexate as DMARD
- #1 DMARD, freq. used with biologic
- Inhibits dihydrofolate reductase
- immunosuppressant, also antineoplastic.
- Salicylic derivitive, metabolized to 5-ASA and sulfapyridine, used in IBD.
- reduce macrophade and lysosomal functions.
- Purine metabolism and nucleic acid synthesis reduced, immunosuppressant.
- think of it as "the opposite of penicillin"
- reduces T-cell activity and Rheumatoid factor,
- also a chelator.
Binds TNF, soluble receptor. Requires injection.
Inhibits cell proliferation and antiinflamatory. Works on pyrimdiine byiosyntehsis.
Monoclonal Ab to TNF-A
- also used in Inflmmatory Bowel Disease.
- costimulatory blocker, anti CD40 L on T-Cells!
- T-cells = CD40 L
- APCs= CD40!
Antinuclear Antibodies (ANA)?
Drug-induced lupus Auto-Ab?
Anti-IgG (Rheumatoid Factor), Anti-CCP
Anti Scl-70 (anti-DNA topoisomerase I)
Primary biliary cirrhosis Auto-Ab?
Celiac Disease Auto-Ab?
Goodpasture's syndrome Auto-Ab?
Pemphigus vulgaris Auto-Ab?
Hashimoto's thyroiditis Auto-Ab?
- Anti-SS-A (Anti-Ro)
- Anti-SS-B (Anti-La)
"Row Row Row, La La La, can't stay because of Sjögren's!"
ANTI-U1 RNP (Riboncleuoprotein)
Autoimmune hepatitis Auto-Ab?
DM Type I Auto-Ab?
Wegener's granulomatosis Auto-Ab?
Other non Wegener's vasculitides auto ab?
- Mixed Connective Tissue Disorder
- Features of SLE, polymyositis, and scleroderma.
- No Renal Disease.Great response to corticosteroids.
Another name for Baker's Cyst?
- Popliteal Cyst
- AN enlargment of semimebranous bursa.
- Associated with Rheumatoid Arthritis.
- Eosinophilic on H and E. Dead cells. Associated with immune complex disease.