Rheumotology

antihistone ab w/o other autoabs. Associated with hydralazine and procaniamide

antibody to ribonucleoprotein (RNP)

• HLA-DR4
• Get rhemutaoid arthritis in your 40s

• DR3/DR4
• I had to wake up Nat Granger 3 to 4 times

• DR2/DR3
• Get SLE when you are a 23 y/o F.

• DQ2, or DQB
• QUIT eating bread!!

• HLA-B8
• Bummer to have this Grave disease.

• DR2
• It causes two diseases, MS and Goodpasture

• DR2
• It causes two diseases MS and Goodpasture syndrome

• 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
• 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.

• 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

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

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
• 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.

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!

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.
• Photosensitivity.

• 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)

Edrophonium

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

• #1 DMARD, freq. used with biologic
• Inhibits dihydrofolate reductase
• immunosuppressant, also antineoplastic.

• DMARD
• Salicylic derivitive, metabolized to 5-ASA and sulfapyridine, used in IBD.

Anti-inflammatory, immunomodulatory.

• DMARD
• reduce macrophade and lysosomal functions.

• DMARD,
• Purine metabolism and nucleic acid synthesis reduced, immunosuppressant.

• DMARD
• 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.

Hepatotoxic.

Monoclonal Ab to TNF-A

• also used in Inflmmatory Bowel Disease.
• Injected.

• costimulatory blocker, anti CD40 L on T-Cells!
• T-cells = CD40 L
• APCs= CD40!

SLE, nonspecific

SLE

Antihistone

Anti-IgG (Rheumatoid Factor), Anti-CCP

Anticentromere

Anti Scl-70 (anti-DNA topoisomerase I)

Antimitochondrial

Antigliadin, antiendomysial

Anti-Basement Membrane

Anti-desmoglein

Antimicrosomal, antithyroglobulin

Anti-Jo-1

• 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)

Anti-smooth muscle

Anti-glutamate decarboxylase

c-ANCA

p-ANCA

• Mixed Connective Tissue Disorder
• Features of SLE, polymyositis, and scleroderma.
• No Renal Disease.
• Great response to corticosteroids.

• Popliteal Cyst
• AN enlargment of semimebranous bursa.

• Associated with Rheumatoid Arthritis.
• Eosinophilic on H and E. Dead cells. Associated with immune complex disease.