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Rheumatology
Internal Med
75
Science
Graduate
09/29/2015

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Term
chronic systemic, multi organ autoimmune d/o of connective tissues. Type III HSN
Definition
systemic lupus erythematosus (SLE)
Term
Systemic lupus erythematosus (SLE) usually happens in...
Definition

young females

onset in 20s-40s

increased in AA, hispanic, native americans

Term
Causes of lupus
Definition

genetic

environmental sun exposure

infections

hormonal (estrogen)

Term
Drugs that cause drug induced SLE
Definition

procainamide

hydralazine

INH

quinidine

+ anti-histone bodies

drug induced lupus usually resolves w/ drug d/c

Term
Clinical manifestations of SLE
Definition
  1. triad of joint pain (90%), fever, & malar "butterfly" rash (cheeks & bridge of nose - sparing nasolabial folds)  Serositis: pericarditis, pleuritis
  2. discoid lupus: annular, erythematous patches on face & scalp heals w/ scarring
  3. systemic: CNS, cardio, glomerulonephritis, retinitis, oral ulcers, alopecia
Term
How do you diagnose systemic lupus erythematosus (SLE)
Definition
  1. + anti-nuclear ab (ANA) - best initial test, not specific + RF
  2. + anti-double stranded DNA & Anti-smith Ab 100% spp for SLE (not sensitive)
  3. antiphospholipid ab syndrome (APLS): increased risk of arterial & venos thrombosis
  • + anticardiolipid Ab assoc w/ false + VDRL/RPR 
  • lupus anticoagulant
  • B-2 glycoprotein I ab. women w/ APLS may have frequent miscarriages, livedo reticularis

4. CBC: +- hemolytic anemia, leukopenia, lymphopenia, thrombocytopenia

Term
treatment of SLE
Definition

sun protection

hydroxychloroquine (for skin lesions)

NSAIDs or acetaminophen for arthritis

+- pulse dose steroids

cytotoxic drugs (methotrexate, cyclophosphamide)

Term
systemic connective tissue d/o : thickened skin (sclerodactyl), lung, heart, kidney, and GI tract
Definition
scleroderma (systemic sclerosis)
Term
tight, shiny, thickened skin (localized or generalized) d/t fibrous collagen buildup
Definition
scleroderma (systemic sclerosis)
Term
Clinical manifestations of scleroderma (systemic sclerosis)
Definition
  1. Limited cutaneous systemic sclerosis "crest syndrome" 
  • Calcinosis cutis
  • Raynaud's phenomenon
  • Esophageal motility d/o
  • Sclerodactyl (claw hand)
  • Telangectasia
  • MC type
  • affects the face, neck as well as distal to elbows & knees
  • Raynauds phenomenon : worse w/ cold, smoking, or emotional stress.  CCB tx of choice 

2. Diffuse cutaneous systemic sclerosis: skin thickening - trunk & proximal extremities

Term
How do you diagnose scleroderma (systemic sclerosis)
Definition
  1.  + anti-centromere Ab: assoc limited/CREST dz - more specific. Better prognosis
  2. + anti-scl-70 Ab: assoc w/ diffuse dz & multiple organ involvement. + ANA (nonspp)
Term
Treatment of scleroderma (systemic sclerosis)
Definition
DMARDS, steroids
Term
treatment of raynauds dz
Definition
vasodilators (CCBs, prostacyclin)
Term
autoimmune d/o attacking exocrine glands.

salivary glands --> xerostoma (dry mouth); lacrimal glands --> dry eyes (keratoconjunctivitis sicca) & parotid enlargement. Thyroid gland dysfxn
Definition
sjogren's syndrome
Term
Primary sjogren's syndrome
Definition
occurs alone
Term
secondary sjrogens syndrome
Definition
assoc w/ other autoimmune disorders - SLE, RA, ect
Term
Activation of the immune system possibly triggered by viruses aggregation of lymphocytes (ex CD4 cells) in exocrine glands (lymphocytic invasion).

HLA-DR52 seen in 85% of patients
Definition
sjrogen's syndrome
Term
increased incidence of lymphoma, interstitial nephritis, and pneumonia
Definition
sjrogen's syndrome
Term
How do you diagnose sjrogen's syndrome?
Definition

ANA: esp antiSS-A (Ro) & antiSS-B (la)

+RF

Shirmer test (decreased tear production)

Term
How do you treat sjrogen's syndrome?
Definition
  1. artificial tears, pilocarpine (cholinergic) for xerostoma
  2. Pilocarpine: cholinergic drug that increases lacrimation & salivation (SLUDD-C) SE: diaphoresis, flushing, sweating, bradycardia, diarhea, N/V, incontinence, blurred vision. Cyclosporine may help w ocular symptoms
  3. Cevimeline (Evoxac): stimulates muscarinic cholinergic receptors
Term
widespread muscular pain (us chronic), fatigue, muscle tenderness, HA's, poor sleep/memory problems (may be d/t increase in pain perception, increased substance P)
Definition
fibromyalgia
Term
MC in middle aged women. increased incidence w/ RA, SLE, ankylosing spondylitis
Definition
fibromyalgia
Term
Clinical manifestations of fibromyalgia
Definition

diffuse pain (esp in the morning)

extreme fatigue

stiffness

painful, tender, joints

sleep disturbances

sx often worsened w/ physical/psych stress

Term
How do you diagnose fibromyalgia?
Definition
  1. diffuse pain in 11 out of 18 trigger points > 3 mo + widespread pain
  2. Biopsy: "moth-eaten" appearance type 1 muscle fibers, injury to muscle.  
Term
how do you treat fibromyalgia?
Definition

TCA's, cymbalta, SSRI's, neurontin, exercise

Pregabalin only drug FDA approved 

swimming preferred exercise (relaxing effect of water on muscles)

Term
idiopathic inflammatory condition causing synovitis, bursitis, & tenosynovitis --> aching/stiffness of proximal joints (shoulder, hick, neck) in pts > 50
Definition
polymyalgia rheumatica
Term
________ is closely related to giant cell arteritis
Definition
polymyalgia rheumatica
Term
Clinical manifestations of polymyalgia rheumatica
Definition
  1. bilateral prox jt pain/STIFFNESS morning stiffness > 30 mins of the pelvic and shoulder girdle
  2. difficulty w/ combing hair, putting on coat, getting out of chair
  3. no severe muscle weakness 
Term
How do you diagnose polymyalgia rheumatica
Definition

clinical dz

increased ESR, anemia (normochromic normocytic)

+- plts (acute phase reactant)

Term
how do you treat polymyalgia rheumatica
Definition
corticosteroids (low dose - 10-20 mg/d); methotrexate
Term
idiopathic inflammatory muscle disease of proximal limbs, neck, pharynx
Definition
polymyositis & dermatomyositis
Term
Clinical manifestations of polymyositis & dermatomyositis
Definition
  1. progressive symmetrical proximal m. weakness (usually painless)
  2. dysphagia
  3. skin rash
  4. polyarthralgias
  5. muscle atrophy (difficulty rising from chair/climbing stairs, combing hair, problems w/ overhead movement)
Term
Diagnosing polymyositis & dermatomyositis
Definition
  1. increased muscle enzymes: increased aldolase, creatine kinase ); increased ESR, + muscle bx, abnormal EKG
  2. + anti-Jo 1 Ab: myositis specific antibody. assoc w/ interstitial lung fibrosis, "mechanic hands" (hyperkeratotic "cracked hands" with dirty appearance
  3. + anti-SRP Ab; (signal recognition particle ab).  almost exclusively seen w/ PM. Assoc w/ more prominent muscle weakness/atrophy & cardiac manifestations
  4. + anti-Mi-2 Ab: specific for dermatomyositis
  5. muscle bx: endomysial invovlement w/ PM; perifascicular/perivascular involvement w/ DM
Term
Dermatomyositis: pathognomonic
Definition
  1. heliotrope (blue-purple) upper eyelid discoloration
  2. gottron's papules: raised violaceous scaly eruptions on the knuckles. malar rash w/ erythema (including nasolabial folds) photosensitive poikiloderma "shawl or V sign," diffuse alopecia
Term
How do you diagnose polymyositis & dermatomyositis?
Definition

muscle enzymes: increased aldolase, creatine kinase

increased ESR

increased incidence of malifnancy 

Term
how do you treat polymyositis & dermatomyositis?
Definition

corticosteroids 1st line tx

+- methotrexate, azathioprine, IV immune globulin

Term
Gout mostly happens in....
Definition

mostly adult men (& postmenopausal women)

middle aged

Term
monosodium urate
Definition
gout
Term
What does gout show in the synovial fluid?
Definition
negatively birefringent: needle-shaped
Term
Most frequently affected joints for gout
Definition
  1. first MTP joint MC (podagra) 50% initially, 90% eventually
  2. 80% monoarticular typically lower extremities (ankle, knees, foot)
Term
x-ray findings of gout
Definition
"mouse bite" (punched out) erosions
Term
Treatment for gout
Definition
  • acute attacks - NSAIDs first line, colchicine, steroids
 
  • chronic - urate lower agents (allopurinol, febuxostate, probenacid) colchicine can also be used .
Term
Pseudo gout usually happens in...
Definition

female predominance, elderly

less common than gout

Term
calcium pyrophosphate dihydrate
Definition
pseudogout
Term
What does synovial fluid show in pts with pseudo gout?
Definition
calcium pyrophosphate dihydrate
Term
What joints are most frequently affected in pseudogout
Definition
knees, wrists, MCP joints, elbows, MTP
Term
x-ray findings in pseudogout
Definition
chondrocalcinosis- calcification of the cartilage
Term
Treatment for pseudogout
Definition
  1. acute attacks: steroids 1st line (intraarticular), NSAIDs, colchicine
  2. Chronic managmement: NSAIDS, +- colchicine 
Term
Important to remember during gout and pseudogout
Definition

Note that chronic management NOT initiated during acute attack (may precipitate an attack) 

- any acute increase or decrease in uric acid levels may precipitate an attack moreso than the actual uric acid levels

Term
systemic vasculitis of medium/small arteries --> necrotizing inflammatory lesions
Definition
polyarteritis nodosa (PAN)
Term
polyarteritis nodosa (PAN) has increased association with...
Definition
hepatitis B & C
Term
increased micro aneurysms w/ aneurysmal rupture --> hemorrhage, thrombosis--> organ ischemia or infarction

muscular arteries involved

Type III HSN
Definition
polyarteritis nodosa (PAN)
Term
Clinical manifestations of polyarteritis nodosa (PAN)
Definition
  1. renal: HTN (d/t increased renin), renal failure.  usually no glomerulonephritis. No capillaries involved
  2. constitutional: fevers, myalgias, arthritis. lungs usually spared
  3. CNS: neuropathy, amaurosis fugax, peripheral neuropathy, mononeuritis complex
  4. Dermatologic: livedo reticularis, purpura, ulcers, gangrene, nodules, Raynauds phenomenon
Term
How do you diagnose polyarteritis nodosa (PAN)
Definition
  1. increased ESR; angiography
  2. MC seen in men ~ 45 yo
  3. classic PAN is ANCA negative 
  4. BX: necrotizing inflammatory lesions. no granulomas
  5. Renal or mesenteric angiography: microaneurysms c/ abrupt cut-off of small arteries
Term
how do you treat polyarteritis nodosa (PAN)
Definition

steroids (+- cyclophosphomaide if refractory)

+- plasmapharesis in pts w/ HBV

Term
Primary joints affected with RA
Definition
wrists, MCP, PIP
Term
Heberden's nodes
Definition

OA

ABSENT in RA

Term
soft, warm, BOGGY, tender joints
Definition
RA
Term
Worse after resting. morning stiffness that lasts @ least or > 30 minutes
Definition
RA
Term
X-ray findings on RA
Definition

osteopenia

symmetric joint narrowing 

Term
laboratory findings positive for RA
Definition

Positive RF

anti CCP ab

ESR, CRP

Term
chronic inflammatory dz w/ persistent symmetric polyarthritis w. bone erosions, cartilage destruction, and joint structure so (d/t destruction by pannus) T cell mediated
Definition
RA
Term
Felty syndrome
Definition

rare d/o triad

 RA+splenomegaly+decreased WBC/repeated infections

Term
caplan syndrome
Definition
pneumoconiosis + RA
Term
Which test is the best initial test for RA, sensitive but NOT specific
Definition
RF
Term
which test is the most specific for RA?
Definition
Anti-citrullinated peptide antibodies (anti-CCP)
Term
criteria for RA
Definition

arthritis >= 3 joints

morning stiffness

disease duration > 6 weeks

Term
Radiologic findings: narrowed joint space (osteopenia/erosions), subluxation, deformities
Definition
RA
Term
treatment of RA
Definition

PROMPT initiation of DMARDs (methotrexate, hydroxychloroquine, leflunamide, minocycline azathioprine) to reduce permanent damage to the joint, cartilage, bone

NSAID for pain control, low dose steroids

Term
autoimmune response to infection in another part of the body
Definition
reactive arthritis (Reiter's syndrome)
Term
Reactive Arthritis (Reiters syndrome)
Definition
  1. asymmetric inflammatory arthritis
  2. conjunctivitis/uveitis
  3. urethritis, cervicitis
Term
Reactive Arthritis (Reiters syndrome) is MC seen with...
Definition

chlamydia

+- seen with gonorrhea

GI (salmonella, shigella, campylobacter, yersinia)

Term
Clinical manifestations of Reactive Arthritis (Reiters syndrome)
Definition

conjunctivitis

urethritis

arthritis (esp. lower extremities)

sausage toes/fingers 

keratoderma blennorrhagica (hyperkeratotic lesions on palms/soles)

circinate balantitis 

Term
how do you diagnose reactive arthritis (reiters syndrome)
Definition

+ HLA-B27  (80%)

CBC: increased WBC (10,000-20,000)

increased ESR

normochromic anemia

increased IgG

synovial fluid: WBC 1,000-8,000 cells/mm3

synvoial fluid bacterial culture negative (aseptic)

Term
how do you treat reactive arthritis (reiters syndrome)
Definition

NSAIDS mainstay of therapy

abx to treat infx that triggered disease

if no repsonse to methotrexate --> sulfasalazine, steroids. 

anti-TNF agents (etanercept, infliximab)

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