Rheumatology

Question 1

Osteoarthritis

Answer 1

• Definition:
  
  • chronic degenerative disease due to loss of articular cartilage & joint degermation. Minimal inflammation. Non-inflammatory!
• Pathophys:
  
  • thinning cartilage → joint space narrowing → lose ability to absorb shock → subchondral sclerosis (stress shielding) → osteophytes (excess bone growth)
• Risks:
  
  • females >45 yo, family hx, obesity
• Joints:
  
  • Joints (weight-bearing): knee, hips, hands (distally-IP joints vs MCP in RA), neck, lumbar spine (asymmetrical)
• S/sxs:
  
  • Joint pain & stiffness: worse in the evening & with activity, weather influenced
  • -Morning stiffness: <30 minutes (vs RA > 30 minutes)
  • -Decreased ROM, -Effusion, Crepitance,-Muscle weakness( hyperlaxity of joints)
  • -Bouchard’s nodes: PIP enlargement
  • -Heberden’s Nodes: DIP enlargement
• Dx:
  
  • Need to r/o other causes
  • Labs = normal
  • XRAY:
    
    • joint space narrowing, osteophytes, subchondral sclerosis/cysts, bone cysts; want bilateral standing films
    • (disease of cartilage NOT bone, so it’s difficult to dx via x ray so MRI is best but its not commonly done)
• Tx:
  
  • First line = education, exercise, & weight control
  • Second-line: pharmacological pain relief (tylenol, ASA, NSAIDs), PT
  • Third-line: surgery (joint replacement)
  • Avoid Opiates! Consider injections: hyaluronic acid, unclear on efficacy

Question 2

Systemic Lupus Erythematosus (SLE)

Answer 2

• Definition:
  
  • Chronic systemic, multi-organ autoimmune disorder of the connective tissue
• Risks:
  
  • young females (~31 yo), African Americans, Hispanics
• Drug-induced Lupus:
  
  • autoimmune disorder similar to SLE caused by use of certain drugs (hydralazine, procainamide, methyldopa, quinidine, isoniazid); need to r/o so ask pt for med history!!
• S/sxs:
  
  • Malar Rash (Pathognomonic)
  • -Discoid rash: annular patches
  • -Arthritis (non-erosive): joint pain
  • -photosensitivity
  • -oral ulcers, -fever, chills, fatigue, -serositis (pleuritis, pericarditis), -seizures, psychosis, -proteinuria, -leukopenia, lymphopenia, thrombocytopenia
• PE:
  
  • Triad: joint pain, fever, malar “butterfly” rash
  • Classification:
    
    • -ANA > 1:80
    • >10 points on the EULAR/ACR Criteria
• Dx:
  
  • -Anti-nuclear antibodies: sensitive, but not specific for SLE, initial test then refer
  • -Anti-DsDNA: specific for SLE, less sensitive, will have lupus nephritis
  • -Anti-Smith: indicator for development of severe SLE
  • -Antiphospholipid antibodies: blood clotting disorders (increased risk of thrombosis), fetal loss
  • -Complement levels: decreased C3, C4, CH50
  • -ESR/CRP: increased in active state only
  • -Renal Biopsy
  • -Pancytopenia: anemia
• Tx:
  
  • -lifestyle: sun protection, smoking cessation, diet (HLP), bone health (calcium, vitamin D)
  • -Medications: hydroxychloroquine, corticosteroids, belimumab, mycophenolate, methotrexate
  • -Mild: hydroxychloroquine +/- NSAIDs
  • -Moderate: hydroxychloroquine +/- short term steroids
  • -Severe: high-dose steroid + immunosuppressive agent
• Cardiovascular Complications:
  
  • -Myocardial infarction risk x 50 in females
  • -Need careful screening for HTN, HLP, DM, Smoking
• SLE Prognosis:
  
  • has improved greatly. Mortality d/t infections in early disease & CV disease in late disease.
    
    • Poor prognosis factors: myocarditis, nephritis, males, low SES, age > 50 at dx.

Question 3

Gout

Answer 3

• Definition:
  
  • uric acid deposition in the soft tissues, joints, & bones. Precipitation of uric acid is very painful.
• Etiology:
  
  • -Underexcretion: 85% = most common
  • • Overproduction: 15%
  • *Categorization falling out of favor d/t need for 24 hour urine collection
• Pathophys:
  
  • elevated purines → xanthine → increased monosodium urate (hyperuricemia) → fecal excretion or renal clearance (decreased in gout)
• Triggers:
  
  • -Elevated Purine Source (overproduction): meat, seafood, beer, drinks with fructose, red wine, aged cheese
  • -Decreased Renal Clearance (underexcretion): thiazides, loop diuretics, aspirin, ACEI
• Risks:
  
  • males (estrogen may be protective), older age, pacific islanders, metabolic syndrome, kidney disease, genetics
• S/sxs:
  
  • *Abrupt onset
  • Severe joint pain, erythema, warmth, swelling, & exquisite tenderness (usually monoarticular, but can be oligoarticular; Fever
  • Most commonly first MTP joint of the great toe (podagra)
• Dx:
  
  • Clinical eval is the most important for diagnosis. Eval for secondary causes (i.e. renal disease)
  • Arthrocentesis:
    
    • Gold standard:++ crystals, needle shape, negative birefringent
    • -Increased WBC count (but <50K)
  • Uric Acid:
    
    • can order, but may lag, and you won’t treat hyperuricemia till after gout flare
  • Xray:
    
    • Do NOT need Xray to make dx
    • -Mouse or Rat bite lesions: punched out erosions with sclerotic & overhanging margins
    • -Tophi in long-standing disease
• Tx:
  
  • Acute:
    
    • -NSAIDS = first line
    • -Prednisone: 10-20 mg PO x 5-7 days
    • -Colchicine: IV/PO
    • **not allopurinol b/c that is chronic only
  • Chronic:
    
    • -lifestyle: low-purine diet (less seafood, meats), decreased alcohol consumption
    • -D/c diuretics
    • -Allopurinol = first line, xanthine oxidase inhibitor → decreases uric acid production; use febuxostat in renal disease
    • -Alternative: probenecid → increases renal excretion of uric acid
    • -Refractory: pegloticase → converts uric acid to soluble form increasing excretion
    • -Lesinurad → inhibitor of uric acid transporters increases renal excretion

*Medication indicated if: tophi on PE, frequent gout attacks, CKD, hx of uric acid urolithiasis

Question 4

OA vs RA comic

Answer 4

Question 5

Calcium Pyrophosphate Deposition Disease (Pseudogout)

Answer 5

• Definition:
  
  • calcium pyrophosphate dihydrate deposition in the joints & soft tissue -→ inflammation & bone destruction
• Epidemiology:
  
  • most > 70 yo, female
• S/sxs:
  
  • *Usually asymptomatic-incidental finding of chondrocalcinosis on Xray
  • -Severe joint pain, erythema, warmth, swelling & exquisite tenderness (clinically indistinguishable from gout)
  • Most commonly in the knee
• Dx:
  
  • Arthrocentesis = gold standard: **3Ps
    
    • -prism shaped crystals
    • -Pyrophosphate
    • -Positive birefringence
  • XR
    
    • chondrocalcinosis of cartilage
• Tx:
  
  • If symptomatic:
  • -NSAIDs = first line
  • -Corticosteroids: PO/injected
  • -Joint replacement (common in knee)

Question 6

Rheumatoid Arthritis: def, pathophys, risks, s/sxs, PE

Answer 6

• Definition:
  
  • chronic systemic autoimmune inflammatory disease with symmetric polyarthritis, bone erosion, cartilage destruction, & joint structure loss
• Pathophys:
  
  • hyperplastic synovial tissue (pannus) leads to joint destruction. Bony erosion is not d/t primary bony changes, but underlying attack on the joint capsule which creates a state of hyperlaxity
• Risks:
  
  • Women, 30-50yo, HLA-DR4, all ethnic groups, smoking (may inhibit TNF tx)
• S/sxs:
  
  • *insidious onset
  • polyarticular, SYMMETRICAL joint pain & swelling (small joints, MCP, PIP, Wrist, MTP)
  • -Morning stiffness > 30 min; BETTER with activity
  • -Fatigue, fever, myalgias, weight loss, joint laxity
• PE:
  
  • Late stage:
    
    • -Boutonniere deformity of thumb
    • -ulnar deviation of metacarpophalangeal joints
    • -Swan-neck deformity of fingers
  • Organ Involvement_:_
    
    • -Skin: nodules
    • -Eyes: keratoconjunctivitis sicca, scleritis
    • -Lungs: ILD
    • -Heart: pericardial effusion
    • -Neuro: cervical spine instability

Question 7

Rheumatoid Arthritis: Dx & Tx

Answer 7

• ACR/EULAR Classification Criteria:
  
  • -Joint involvement: 2 (1 small)
  • -SErology: RF, ACPa
  • -Acute-phase reactants (CRP, ESR)
  • -Duration (> 6weeks)
  • *score > 6 classified as RA, if <6 pt might meet criteria eventually; r/o other causes→ psoriatic arthritis, gout, SLE
• Labs:
  
  • -RF: worse prognosis if positive, 85% positive in RA
  • -ANTI-CCP: = test of choice (more sensitive & specific)
  • -ESR/CRP: elevated during active inflammation → useful for monitoring disease progress/tx
  • -ANA: worse prognosis if positive
• XR:
  
  • symmetric joint narrowing
  • -Bone & joint erosions (only seen after 3 months)
  • -Subluxation (if severe)
• Tx:
  
  • Chronic & progressive. 50% of patients have irreversible joint destruction at 2 years → treat EARLY!!
  • non-biologic DMARDs: Methotrexate, sulfasalazine, leflunomide, hydroxychloroquine
  • Biologic DMARDs: Adalimumab, Etanercept, Infliximab, Abatacept, Rituximab
• -Glucocorticoids for controlling sxs
• *Be aggressive → remission in 10-15%

Question 8

Polymyalgia Rheumatica (PMR)

Answer 8

• Definition:
  
  • idiopathic inflammation of joints, bursae, & tendons.
• Epidemiology:
  
  • age > 50yo with a peak at 70-80 yo, women, European descent, 2nd most common cause of joint pain (1st = RA)
  • Closely associated with giant cell arteritis
• S/sxs:
  
  • Morning joint stiffness > 30 minutes
  • -Shoulder pain
  • -Hip pain
  • -Fatigue
  • -weight loss
  • -Low-grade fever
  • -Myalgias
  • -Decreased ROM with normal muscle strength
• Required Criteria:
  
  • -Age > 50 yo
  • -Bilateral shoulder pain
  • -Abnormal ESR &/or CRP
  • *More points for other criteria with >/= 4 having sensitivity of 68%
• Dx:
  
  • -Elevated ESR/CRP
  • -Normocytic anemia
  • -Other markers negative (RF, anti-CCP)
  • *Must r/o RA
• Tx:
  
  • Corticosteroids: Prednisone 10-20 mg/day x 7-10 days, then taper → 50% of pts will feel better in 3 days
  • *maintain low dose steroids PRN
• *Favorable prognosis: rapid response to steroids (~3 days) → most will go into remission, but some may need to be maintained with low-dose steroids

Question 9

Psoriatic Arthritis

Answer 9

• Definition:
  
  • inflammatory arthritis in patients with psoriasis. Psoriasis precedes arthritis by ~10 years so need to monitor joint sxs in these pts
• Epidemiology:
  
  • 30-50 yo, ⅓ of patients with psoriasis will develop PsA, Male = female, 40% with family hx of PsA
• S/sxs:
  
  • Arthritis: asymmetric pattern, SI joint = common
  • -Heel & foot pain: soft tissue involvement of achilles & plantar fascia
  • Psoriasis: erythematous plaques with thick silvery-white scales
  • -Dactylitis: sausage fingers
  • -Eye inflammation
  • -Nail pitting & crumbling
• PE:
  
  • Unique clinical findings:
  • -Skin changes (psoriasis)
  • -Nail changes
  • -Finger Swelling
  • -”Pencil in cup” sign: photos demonstrate progression from normal to pencil in cup
• Dx:
  
  • Clinical Dx
  • Xray: erosive changes & new bone formation in distal joints
  • -”pencil in a cup”deformities: thin end of one bone appears to be inserted into a thicker bone
  • Labs:
  • -RF & ESRP/CRP elevated
  • -Anti-CCP: used to r/o RA
• Tx:
  
  • -Mild: NSAIDs
  • -Severe: DMARDs (methotrexate =1st line, TNF inhibitors, Interleukins
  • -Joint replacement

Question 10

Sjogren’s Syndrome

Answer 10

• Definition:
  
  • autoimmune disease affecting the exocrine glands (salivary & lacrimal glands)
• Pathophys:
  
  • lymphocytic invasion of lacrimal & salivary glands
• Epidemiology:
  
  • females (90%), median age of 50yo, usually associated with other connective tissue disorders (RA/SLE)
• S/sxs:
  
  • -Dry Mouth, Dry Eyes,Vaginal dryness, dyspareunia
  • -Parotid gland enlargement; dental caries & dysphagia d/t lack of saliva
  • -Arthriti, Neuro problems, Peripheral neuropathy
• PE:
  
  • Schirmer test: decreased tear production (wetting of <5mm of filter paper placed in lower eyelid for 5 min)
  • -Rose Bengal Stain: abnormal corneal epithelium
• Dx:
  
  • -RF/ANA may be positive (d/t associated RA or SLE)
  • -AntiSS-A (Ro), AntiSS-B (la)
  • -Tissue biopsy: mouth mucosa shows inflammation
• Diagnostic criteria:
  
  • -Ocular symptoms (dry eye, FB sensation, artificial tear use)
  • -Oral symptoms (dry mouth, swollen salivary glands)
  • -Ocular signs: abnormal Schirmer’s or positive Rose Bengal
  • -Oral signs: abnormal parotid sialography, salivary scintigraphy, or sialometry
  • -Histopathology: lymphocytic foci
  • -Autoantibodies: Anti-Ro, anti-La
• Tx:
  
  • *No cure only supportive care
  • -Dry mouth: saliva substitutes (sprays, rinses), saliva stimulation (hard candy), cholinergic agents (cevimeline, pilocarpine), active dental care > q 6 months)
  • Dry eyes: lubricants (artificial tears, ointments), punctal plugs (block the tear ducts)
  • Dry Nasal Mucosa: saline nasal spray, lavage
  • -Immunosuppressives

Question 11

Reiter’s Syndrome (Reactive Arthritis)

Answer 11

• Definition:
  
  • inflammatory arthritis in response to an infection (usually STD or foodborne illness) or inflammation in another part of the body
• Etiology:
  
  • may be seen in 1-4 weeks after GI or GU infection -chlamydia, salmonella, shigella, campylobacter, HIV
• Risks:
  
  • HLA-B27 gene (<20% will develop it)
• Epidemiology:
  
  • young male 20-50 yo
• S/sxs:
  
  • Triad:
    
    • -Conjunctivitis
    • -Urethritis
    • -Arthritis
    • “Mr Reiter Can’t see, can’t pee, can’t climb a tree”
  • Other:
    
    • Keratoderma blennorrhagicum: hyperkeratotic lesions on palms & soles
• Dx:
  
  • arthrocentesis → need to r/o septic arthritis
• Tx:
  
  • *usually improves on its own within a few weeks or months
  • NSAIDs = first line
  • -Abx to tx the underlying cause
  • -Corticosteroids
  • -Immunosuppressants: if no response to NSAIDs

Question 12

Juvenile Idiopathic Arthritis

Answer 12

• Definition:
  
  • autoimmune mono- or polyarthritis in children < 16 yo for > 6 weeks. Umbrella term. “ Kids don’t get arthritis, but if they do it goes under this category”
• S/sxs:
  
  • **Needs 2+ of the following:
  • -Joint swelling, joint effusion, joint warmth (calor), decreased ROM of the joint, painful ROM of the joint, Tenderness
• PE:
  
  • S-JIA ILAR Classification:
    
    • -<16 yo
    • -Fever > 2 weeks (remittent but need > 3 days in a row)
    • -Arthritis >1 joint
    • -At least 1 of the following: evanescent erythematous rash, generalized LND enlargement, hepatomegaly, splenomegaly, serositis
• Dx:
  
  • dx of exclusion
  • ESR/CRP = very high
  • -CBC: higher WBC, neutrophilia
  • -Thrombocytosis
  • -RF, ANA, CCPig may be helpful
  • -High ferritin levels
  • -Anemia is common (need to r/o iron deficiency)
• Tx:
  
  • **Refer out → Multidisciplinary
  • -NSAIDs = first line, corticosteroids, DMARDs, biologic agents
  • *Similar to adults

Question 13

Oligoarthritis (Pauci) JIA

Answer 13

• Definition:
  
  • 2-4 joints affected within 6 months of disease onset. Most common type of JIA. F>M. onset usually by 6 yo.
• S/sxs:
  
  • Anterior Uveitis
  • -arthritis: knee = most common
• Dx:
  
  • positive ANA (lupus variant??)
• Tx:
  
  • Best prognosis
• Complications:
  
  • chronic uveitis (needs routine eye exam)
  • -may progress to polyarthritis

Question 14

Polyarthritis JIA

Answer 14

• Definition:
  
  • 5+ joints affected within 6 months of disease onset. Onset = 8-16yo. Closely related to RA in adults. 2nd most common type
• S/sxs:
  
  • -Extra-articular involvement
  • -Joint erosions
  • -Skin nodules
• Other:
  
  • often misdiagnosed as acute lymphoblastic leukemia (ALL)
• Dx:
  
  • -RF may be positive (2-7%) or negative (11-28%)
  • -HLA+
  • -ANA may be positive
• Tx:
  
  • Poor prognosis: +RF, hip or cervical spine arthritis, erosions/joint space narrowing on XR, early hand involvement

Question 15

Systemic (Still’s Disease) JIA

Answer 15

• F>M. 1-6yo. Closely related to systemic sclerosis.
• S/sxs:
  
  • Fever
  • -migratory salmon-pink rash
  • -Arthritis
• PE:
  
  • HSM, lymphadenopathy
  • -growth delays
  • -pericarditis, pleuritis
• Complications:
  
  • -tamponade, pulmonary HTN
  • -Macrophage activation syndrome

*

Question 16

Enthesitis-Related Arthritis JIA

Answer 16

• M>F. Most >9yo, Associated with HLA-B27. Closely related to ankylosing spondylitis.
• S/sxs:
  
  • -soft tissue involvement: tendon, ligament, fascia
  • -Arthritis: peripheral pattern
• PE:
  
  • Extra-articular involvement: IBD, uveitis, valvular heart disease

Question 17

Psoriatic JIA

Answer 17

• F>M. <16yo at diagnosis. Similar to adult type (except rash may not proceed arthritis)
• S/sxs:
  
  • -Psoriasis
  • -Arthritis: peripheral & symmetric, >4 joints
• PE:
  
  • -SI joint involvement
  • -chronic uveitis
• Tx:
  
  • -Needs optho consult

Question 18

Undifferentiated JIA

Answer 18

• Definition:
  
  • arthritis that doesn’t meet criteria for other JIA subtypes
• *Variable clinical presentation

Question 19

Systemic Sclerosis (Scleroderma): Def, types, epidemiology, S/sxs, & PE

Answer 19

• Definition:
  
  • systemic autoimmune connective tissue disorder where collagen deposition → fibrosis of skin & internal organs. Multi-system disease. > 6 months before diagnosis
• Types:
  
  • Limited (CREST syndrome)
  • -Diffuse: Females > males
• Epidemiology:
  
  • Women 30-50, HLA-B27
• S/sxs:
  
  • CREST syndrome:
  • -tight, shiny, thickened skin involving the face, neck & distal knees/elbows
    
    • -Swollen puffy fingers
    • -C: calcinosis of soft tissue
    • -R; Raynaud’s phenomenon
    • -E: esophageal (dysmotility) motility disorder
    • -S: Sclerodactyly
    • -T: Telangiectasias
  • -Pulmonary HTN
• PE:
  
  • -MSK: Arthralgias, joint tenderness, joint contractures
  • -Pulmonary: DOE, rales (get PFTs), ILD, pulmonary fibrosis
  • -GI: oral dryness, periodontal disease, dysphagia, reflux, dysmotility, diverticulosis
  • -Cardiac: DOE, palpitations, CP, pericardial effusions
  • -Renal: HTN (accelerated renal failure in Diffuse scleroderma), renal sparing in crest

Question 20

Systemic Sclerosis (Scleroderma): Dx & Tx

Answer 20

• Diagnostic Criteria (ACR):
  
  • *One major + two minor for dx
  • -Major: proximal scleroderma (symmetrical thickening, tightening of the skin of fingers & MCP/MTP)
  • -Minor: sclerodactyly, digital pitting scars/loss of substances from finger pad, bibasilar pulmonary fibrosis
• Classification Criteria:
  
  • *Score > 9 has high sensitivity & specificity
• Labs:
  
  • ANA: positive, nonspecific, centromere pattern in CREST & speckled in Diffuse
  • -Anti-centromere antibodies: CREST
  • -Anti-SCL-70 antibodies: Diffuse
  • -Mild anemia often present
  • -ORDER PFTs
• Tx:
  
  • primary cause of mortality: pulmonary involvement
  • *Supportive care -→ No cure!
• NSAIDs, corticosteroids, analgesics, & PT
• -Good hygiene, skin care
• -Raynaud’s: vasodilators
• -Hypertensive renal disease: ACEI
• -GERD: PPIs, H2 Blockers
• -Severe: DMARDs (Methotrexate)

Question 21

Diffuse vs Limited Scleroderma

Answer 21

Question 22

Giant Cell Arteritis

Answer 22

• Definition:
  
  • large vessel granulomatous systemic vasculitis commonly affecting the extracranial branches of the carotid artery (usually temporal)
• Risks:
  
  • women > 50 yo, genetics, post-viral or bacterial infection, post-abx use
• Associated with polymyalgia rheumatica
• S/sxs:
  
  • *Acute onset
  • -Headache: new onset,localized, unilateral, temporal
  • -Jaw claudication with mastication
  • -Visual changes: transient monocular visual loss (amaurosis fugax)
  • -Constitutional symptoms: fever, fatigue, weight loss, night sweats, malaise
• PE:
  
  • -Focal tenderness over temporal artery
  • -Neuropathy: UE paresthesia
  • -Aortic arch involvement: UE claudication, decreased pulses
• ACR Classification:
  
  • -Age > 50yo
  • -New onset headache
  • -Temporal artery abnormalities
  • -ESR elevated
  • -Histologic evidence of arteritis on temporal artery biopsy
• Dx:
  
  • -ESR/CRP = Very High
  • -Anemia: microcytic hypochromic
  • -Thrombocytopenia
  • -Elevated Alk Phos
  • Temporal Biopsy = definitive (tx 1st then refer to ophtho)
  • Aortography: to r/o aortic involvement
• Tx:
  
  • -High dose oral 60 mg prednisone (ASAP to prevent blindness) → refer to ophtho)
  • -May need repeated steroid courses over lifetime
  • -Aspirin 81-325mg/day (to prevent stroke)
  • -Methotrexate
• Complications:
  
  • -Aortic aneurysm
  • -Irreversible blindness = most common
  • -Stroke

Question 23

Polyarteritis Nodosa

Answer 23

• Definition:
  
  • systemic vasculitis of small-medium vessels (bifurcations common). Commonly affects renal (most common), CNS, & GI vessels. Pulmonary vessels not involved.
• Pathophys:
  
  • Type III hypersensitivity → ischemia & microaneurysms of the affected vessels.
• Risks:
  
  • males 45-65yo , associated with chronic hepatitis B
• S/sxs:
  
  • -Arthralgias
  • -Myalgias
  • -Constitutional: fever, weight loss, malaise -Palpable purpuric rash -Neuropathy -HTN
• PE:
  
  • Systems:
  • -Renal: HTN (renal artery stenosis) renal insufficiency
  • -Skin: livedo reticularis, nodules, ulceration, palpable purpura
  • -CNS: peripheral neuropathy
  • -GI: postprandial pain, N/V/D, bleeding
  • -GU: testicular pain
• Dx:
  
  • ESR/CRP elevated
  • -Creatinine is elevated
  • -LFTs are abnormal
  • -Anemia
  • -CPK: elevated
  • Angiography:
  • -renal or mesenteric
  • -Microaneurysms
  • Biopsy = definitive
• Criteria: * need 3/10
  
  • -Weight loss > 4kg
  • -Livedo reticularis
  • -Testicular pain
  • -myalgias
  • -Neuropathy
  • -Diastolic BP > 90
  • -Increased BUN/Cr
  • -Hep B virus
  • -Arteriographic abnormality
  • -Artery biopsy containing PAN
• Tx:

Glucocorticoids: 50/50 cure

-Refractory: cyclophosphamide

Question 24

Behcet’s Disease

Answer 24

• Definition:
  
  • multisystemic autoimmune disorder that causes variable blood vessel inflammation
• Risks:
  
  • 20-40 yo, Asian/Middle Easter
• S/sxs:
  
  • Painful oral & genital ulcers
  • -Erythema nodosum
  • -Uveitis & conjunctivitis
  • -Arthritis
• PE:
  
  • Pathergy: sterile skin papules or pustules from minor trauma
• Dx:
  
  • clinical dx
    
    • Increased ESR, CRP, Leukocytes
• Tx:
  
  • -Corticosteroids during flares
  • -Refractory to tx: colchicine, cyclophosphamide, azathioprine

Question 25

Kawasaki’s Disease

Answer 25

• Definition:
  
  • medium blood vessel necrotizing vasculitis where the immune system attacks arteries, damaging endothelial cells (especially of the coronary arteries)
• Pathophys:
  
  • unidentified respiratory agent or viral pathogen with a propensity towards vascular tissue
• Risks:
  
  • males < 5yo, asians
• S/sxs:
  
  • Warm & CREAM:
  • -Fever > 5 days
  • -Conjunctivitis
  • -Rash
  • -Extremity changes: edema, erythema, or desquamation
  • -Adenopathy
  • -Mucositis: strawberry tongue, lip swelling, fissures
• Dx:
  
  • -Labs: elevated WBCs & platelets, anemia, increased ESR/CRP
  • -Echo
  • -ECG
• Tx:
  
  • IV immunoglobulin & aspirin (for fever & coronary complications)
• Complications:
  
  • -coronary vessel arteritis: coronary artery aneurysms, MI, pericarditis, myocarditis

Question 26

Answer 26

Ankylosis of SI joints & Hips: late stage Ankylosing spondylosis

Question 27

Fibromyalgia

Answer 27

• Definition:
  
  • abnormal pain perception of unknown etiology. Most common cause of widespread pain in young women.
• Etiology:
  
  • unclear
  • -Disorder of CNS pain regulation: heightened response to stimuli, loss of gray matter, altered neurotransmitter function, abnormal resting state
  • -Sleep/mood/cognitive abnormalities
  • -Stress/autonomic dysfunction
  • -Peripheral pain mechanisms: myofascial, ligamentous, OA
• Risks:
  
  • women 22-50yo, genetics
• S/sxs:
  
  • Chronic, widespread musculoskeletal pain (>3 months)
  • -Extreme fatigue
  • -Sleep & cognitive disturbances
  • -HA
  • -Bowel irritability
• PE:
  
  • Widespread tenderness( > 11/18 trigger points)
  • -Absence of joint swelling or inflammation
• Dx:
  
  • Clinical Dx
  • Widespread pain Index (WPI):
    
    • -Score: 0-19 (based on number of areas pain is felt)
• Symptom Severity (SS):
  
  • -Score: 0-12 (based on fatigue, trouble thinking, waking up tired, abdominal pain, HA, depression)
  • *FM: WPI score > 7 & SS Score > 5 or WPI >3 & SSS > 9
• ESR/CRP & CBC = normal
• Tx:
  
  • -Low impact aerobic exercise
  • -Mood: TCA (amitriptyline), SSRI, SNRI (Duloxetine)
  • -Body pain: APAP, NSAIDs, anticonvulsants (gabapentin, pregabalin)
  • *First line tx: TCA, SNRI, or gabapentin/pregabalin with aerobic exercise
  • *Second line tx: tramadol, fluoxetine, citalopram