Rheumotology

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Jastrzembski
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131268
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Rheumotology
Updated:
2012-02-20 15:50:45
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USMLE Rheumotology
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USMLE
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  1. What is marker for drug-induced lupus?
    antihistone ab w/o other autoabs. Associated with hydralazine and procaniamide
  2. Marker for Mixed connective tissude disease
    antibody to ribonucleoprotein (RNP)
  3. HLA group for RA
    • HLA-DR4
    • Get rhemutaoid arthritis in your 40s
  4. HLA group for DMI
    • DR3/DR4
    • I had to wake up Nat Granger 3 to 4 times
  5. SLE HLA link
    • DR2/DR3
    • Get SLE when you are a 23 y/o F.
  6. Celiac Disease Associated HLA
    • DQ2, or DQB
    • QUIT eating bread!!
  7. HLA associated with Graves Disease
    • HLA-B8
    • Bummer to have this Grave disease.
  8. What is HLA allele for MS?
    • DR2
    • It causes two diseases, MS and Goodpasture
  9. What is HLA for Goodpasture?
    • DR2
    • It causes two diseases MS and Goodpasture syndrome
  10. What diseases associated with DR2?
    • MS and Goodpasture Syndrome
    • It casues 2 diseases!
  11. Rheumatoid Arthritis
    • Autoimmune, inflammatory disorder affecting:
    • synovial joints with pannus formation in joints (MCP, PIP)
    • subcutaneous rheumatoid nodules (fibrinoid necoris surrounding histiocytes)
    • Ulnar deviation
    • Subluxation
    • 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.

    HLA-DR4 association

    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.
  12. Sjogren's
    • Triax:
    • Xerophthalmia (dry eyes, conjunctivitis, sand in my eyes)
    • Xerostomia (dry mouth, dysphagia)
    • Arthritis

    • Parotid enlargment, Increased risk of B-cell lymphoma
    • SS-A (ro) SS-B (La)

    • Females 40-60 years old
    • Assoc. with RA
  13. Sicca syndrome
    dry eyes, dry mouth, nasal and vaginal dryness, chronic brnchitis, reflux esophagitis, but no arthritis.
  14. Pseudogout
    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
  15. 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.
  16. SLE
    • IM DAMN SHARP
    • Immunoglobulins:
    • ANA = Sensitive, not specific
    • anti-dsDNA = specific, poor prognosis.
    • Anti-Sm (smith) = specific, no prognostic value
    • Antihistone = Drug induced

    • Malar Rash
    • Discoid Rash
    • ANA
    • 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)
    • Arthritis
    • Renal (Immune complex deposition, nephritic)
    • Photosensitivty
    • 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.
  17. Sarcoidosis
    Characterized by immune-mediated, widespread noncaseating granulomas, elevate ACE. Common in Black females

    • GRAIN
    • Gammaglobulinemia
    • 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!
  18. Polymyalgia Rheumatica
    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
  19. Polymyositis
    Progressive symmetric proximal muscle caused by CD8 damage of myofibers. Often involves shoulders. Dx with muscle biopsy with evidence of perifascicular inflammation.
  20. Dermatomyositis
    • Similar to polymyositis, but involves malar rash helitropic. "Mechanic's hands" and increased risk of internal malignancy only in adults.
    • Photosensitivity.
  21. Myasthenia gravis
    • 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)
  22. What is the AChE inhibitor used to treat myasthenia gravis?
    Edrophonium
  23. Lambert-Eaton syndrome
    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.
  24. Scleroderma
    • 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.
  25. Hydroxychloroquine
    DMARD. Stabilizes lysosomes, reduces chemotaxis, also an antimiral.

    S/E: Ophthalmic abnormalities, derm rxns, hematoxicity, GI
  26. Methotrexate as DMARD
    • #1 DMARD, freq. used with biologic
    • Inhibits dihydrofolate reductase
    • immunosuppressant, also antineoplastic.
  27. Sulfasalazine
    • DMARD
    • Salicylic derivitive, metabolized to 5-ASA and sulfapyridine, used in IBD.

    Anti-inflammatory, immunomodulatory.
  28. Gold compounds
    • DMARD
    • reduce macrophade and lysosomal functions.
  29. Azathioprine
    • DMARD,
    • Purine metabolism and nucleic acid synthesis reduced, immunosuppressant.
  30. Penicillamine
    • DMARD
    • think of it as "the opposite of penicillin"
    • reduces T-cell activity and Rheumatoid factor,
    • also a chelator.
  31. Etanercept
    Binds TNF, soluble receptor. Requires injection.
  32. Leflunomide
    Inhibits cell proliferation and antiinflamatory. Works on pyrimdiine byiosyntehsis.

    Hepatotoxic.
  33. Infliximab
    Monoclonal Ab to TNF-A

    • also used in Inflmmatory Bowel Disease.
    • Injected.
  34. Abatacept
    • costimulatory blocker, anti CD40 L on T-Cells!
    • T-cells = CD40 L
    • APCs= CD40!
  35. Antinuclear Antibodies (ANA)?
    SLE, nonspecific
  36. Anti-dsDNA?
    SLE
  37. Drug-induced lupus Auto-Ab?
    Antihistone
  38. RA Auto-Ab?
    Anti-IgG (Rheumatoid Factor), Anti-CCP
  39. CREST Auto-Ab?
    Anticentromere
  40. Scleroderma Auto-Ab?
    Anti Scl-70 (anti-DNA topoisomerase I)
  41. Primary biliary cirrhosis Auto-Ab?
    Antimitochondrial
  42. Celiac Disease Auto-Ab?
    Antigliadin, antiendomysial
  43. Goodpasture's syndrome Auto-Ab?
    Anti-Basement Membrane
  44. Pemphigus vulgaris Auto-Ab?
    Anti-desmoglein
  45. Hashimoto's thyroiditis Auto-Ab?
    Antimicrosomal, antithyroglobulin
  46. Poliomyositis/dermatomysoitis Auto-Ab?
    Anti-Jo-1
  47. Sjögren's Syndrome
    • Anti-SS-A (Anti-Ro)
    • Anti-SS-B (Anti-La)

    "Row Row Row, La La La, can't stay because of Sjögren's!"
  48. MCTD Auto-Ab?
    ANTI-U1 RNP (Riboncleuoprotein)
  49. Autoimmune hepatitis Auto-Ab?
    Anti-smooth muscle
  50. DM Type I Auto-Ab?
    Anti-glutamate decarboxylase
  51. Wegener's granulomatosis Auto-Ab?
    c-ANCA
  52. Other non Wegener's vasculitides auto ab?
    p-ANCA
  53. MCTD
    • Mixed Connective Tissue Disorder
    • Features of SLE, polymyositis, and scleroderma.
    • No Renal Disease.
    • Great response to corticosteroids.
  54. Another name for Baker's Cyst?
    • Popliteal Cyst
    • AN enlargment of semimebranous bursa.
  55. Fibrinoid necrosis
    • Associated with Rheumatoid Arthritis.
    • Eosinophilic on H and E. Dead cells. Associated with immune complex disease.

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