Department of Internal Medicine - Division of Immunology

Educational Goals: Rheumatology
Rotating Residents and Medical Students


  1. Fibromyalgia
    1. Know historical features
      1. chronic, diffuse musculoskeletal pain
      2. nonrefreshing sleep
      3. may be associated with other conditions (i.e. RA, etc)
    2. Know features of musculoskeletal exam
      1. no synovitis
      2. normal ROM and strength
      3. characteristic tender points
    3. Describe laboratory features
      1. lab tests are normal
      2. check TSH; thyroid dysfunction can imitate
    4. Describe therapeutic options
      1. Education
      2. exercise and stretching
      3. antidepressants to improve quality of sleep
  2. Low Back Pain
    List at least 9 causes and know their distinguishing features
      1. Inflammatory back disease
        1. AM stiffness, better with activity
        2. ABNL SI films
        3. elevated ESR in young man
      2. Osseous Malignancy
        1. Primary
          • multiple myeloma
          • lymphoma
        2. Metastatic
          • prostate
          • breast
          • kidney
          • thyroid
          • ling
          • colon
      3. Mechanical/ Degenerative back (disc) disease
        1. better with rest, worse with activity
        2. little AM stiffness
      4. Visceral
        1. perforated ulcer
        2. pancreas
          • pancreatitis
          • malignancy
        3. pyelonephritis
        4. ovarian dysfunction
        5. uterine dysfunction
        6. others
      5. Abdominal aortic aneurysm
        1. diminished peripheral pulses
        2. pulsatile mass in abdomen
      6. Radiculopathy (disc)
        1. radicular radiation, focal neuro findings
        2. worse with valsalva
        3. cross over pain
      7. Spinal Stenosis
        1. "pseudo (neurogenic)claudication"
        2. better with back flexion
      8. Spondylolisthesis/Spondylolysis
        1. young males
        2. history of trauma
      9. Vertebral Compression Fracture
        1. sudden onset of localized pain
        2. risk factors (age, therapy with steroids, SLE)
      10. Infection
        1. TB
        2. IV drug abusers
    1. Know the features that suggest urgency in evaluation
      1. Unrelenting night pain
      2. Pain at rest
      3. Progressive symptoms
      4. Neurologic compromise
        1. incontinence
        2. sexual dysfunction
        3. saddle anesthesia
      5. known malignancy
    2. Know features on history and examination (as above)
  3. Bursitis (know anatomic location, presenting signs and symptoms, and provocative maneuvers)
    1. Subacromial
    2. Subdeltoid
    3. Prepatellar
    4. Olecranon
    5. Trochanteric
  4. Tendinitis/Tenosynovitis (know anatomic location, presenting signs and symptoms and provocative maneuvers)
    1. Bicipital
    2. Epicondylitis
    3. Posterior Tibial Tendinitis
    4. Rotator Cuff
    5. DeQuervain's
    6. Trigger Finger

II. Articular Disorders

  1. Osteoarthritis
    1. Primary
      1. 8 joints/areas typically affected
        1. DIP
        2. PIP
        3. 1st CMC
        4. Hip
        5. Knee
        6. 1st MTP
        7. cervical spine
        8. lumbar spine
      2. Recognize 2 noninflammatory symptoms
        1. AM stiffness absent or if present, < 30 minutes
        2. worse with activity, better with rest
      3. Recognize other historical/clinical findings
        1. crepitus
        2. decreased joint motion
        3. pain
        4. gelling
        5. "bony" swelling (Heberden's/Bouchard's nodes)
        6. warmth and swelling may be present; not typical
      4. Know 3 x-ray changes typical for osteoarthritis
        1. nonuniform joint space narrowing
        2. osteophytes (spurs)
        3. sclerosis (white areas in subchondral bone)
      5. Know 6 important treatment options
        1. analgesics (acetaminophen may be as effective as NSAIDs)
        2. exercise
        3. weight loss
        4. rest
        5. ambulatory aids
        6. joint replacement
      6. Know 2 indications for surgery
        1. severe pain unresponsive to conservative management
        2. limitation in function
    2. Secondary
      1. Know definition of secondary osteoarthritis
        1. same clinical features as osteoarthritis
        2. unexpected joint (i.e. shoulder, wrist)
        3. usually secondary to an identifiable cause
      2. Know 3 most common causes
        1. Hyperparathyroidism
        2. Hemochromatosis
        3. Prior trauma or developmental defects
    3. Erosive osteoarthritis
      1. Know definition
        1. osteoarthritis in typical distribution
        2. has an inflammatory component
          • swelling
          • redness
          • warmth
          • pain
        3. erosions ("gull-wing") can be seen on xray
      2. Know 2 typical locations
        1. DIP
        2. PIP
    4. Diffuse Idiopathic Skeletal Hyperostosis (DISH)
      1. Know definition
        1. bone-forming condition
        2. ossification occurs at skeletal sites subjected to stress
        3. has associated stiffness and pain
      2. Know 3 xray findings:
        1. flowing osteophytes connecting ³ 4 vertebrae
        2. normal disc heights
        3. normal SI joints
      3. Know most typical location: thoracic spine
    5. a
  2. Crystal disorders
    1. Gout
      1. List 3 morphologic features for urate crystals
        1. needle shaped
        2. strongly birefringent
        3. negatively birefringent (yellow when aligned parallel to the axis of red compensator on polarizing microscope)
      2. Name at least 3 drugs used to treat acute gout arthritis
        1. NSAIDs
        2. colchicine
        3. prednisone/ACTH
        4. NOT ALLOPURINOL OR PROBENECID!
      3. Know the natural history of untreated gout
        1. asymptomatic hyperuricemia
        2. acute gout with intercritical periods
        3. chronic tophaceous gout
      4. Know 4 commonly involved joints in acute gout
        1. 1st MTP (presenting joint in 50%)
        2. ankle
        3. mid-foot
        4. knee
      5. Describe the opposing effects of low-dose versus high-dose salicylates on uric acid excretion
        1. low: inhibit excretion (urate retaining)
        2. high: promote excretion (uricosuric)
      6. List 3 characteristics of cyclosporine-induced gout
        1. rapidly progressive
        2. polyarticular
        3. tophaceous
    2. CPPD
      1. List 3 most common sites of acute pseudogout
        1. knee
        2. wrist
        3. shoulder
      2. List 3 most common sites of chondrocalcinosis
        1. wrists
        2. knee
        3. pubic symphysis
      3. List 2 most common metabolic etiologies of CPPD
        1. hyperparathyroidism
        2. hemochromatosis
    3. Basic Calcium Phosphate Disease
      1. List 3 clinical presentations
        1. calcific periarthritis (rotator cuff)
        2. acute pseudopodagra (young woman with swellling 1st MTP)
        3. Milwaukee shoulder (elderly woman with destructive shoulder arthropathy
  3. Rheumatoid arthritis
    1. List 4 inflammatory features of RA
      1. joint swelling, redness and warmth
      2. AM stiffness greater than 30 minutes
      3. weight loss
      4. fatigue
    2. List 4 articular features of RA
      1. polyarticular
      2. symmetric
      3. chronic
      4. inflammatory
    3. List at least 8 typical joints/groups of joints involved in RA
      1. PIPs (hands and feet)
      2. MCPs
      3. MTPs
      4. wrists
      5. ankles
      6. shoulders
      7. hips
      8. knees
    4. List at least 10 extra-articular features
      1. Skin (3)
        1. periungual infarcts
        2. ulcerative vasculitis
        3. nodules
      2. Ocular (2)
        1. episcleritis
        2. erosive scleritis
      3. Cardiopulmonary (4)
        1. pericarditis/effusion
        2. interstitial pneumonitis
        3. pulmonary nodules
        4. pleural effusions
      4. Neurologic (2)
        1. Mononeuritis multiplex
        2. Cervical myelopathy (C1-C2 subluxation)
    5. Know 3 clinical and 5 serologic features of vasculitis in RA
      1. periungual infarcts
      2. skin ulcerations
      3. mononeuritis multiplex
      4. RF
      5. ESR
      6. low complements
      7. cryoglobulins
    6. List 5 factors that correlate with poor prognosis in RA
      1. rheumatoid factor
      2. HLA-DR4
      3. female sex
      4. erosive disease
      5. lower level of formal education
    7. List 3 medications used for treatment of early RA
      1. NSAIDS
      2. Hydroxychloroquine
      3. Sulfasalazine
    8. Describe 2 indications for addition of "second line" meds
      1. development of erosions
      2. progressive/persistent synovitis
    9. Know what percentage of RA patients are seropositive
      1. 80%
    10. Felty's syndrome
      1. Know diagnostic triad
        1. Leukopenia
        2. Splenomegaly
        3. RA
      2. Know 3 clinical features of patients with Felty's
        1. seropositive (usually high titer), nodular
        2. erosive RA (may be quiet at time of presentation)
        3. usually have extra-articular features
      3. Know major complication
        1. recurrent infections
      4. Know 4 therapies
        1. gold
        2. MTX
        3. splenectomy
        4. Lithium
    11. Adult-Onset Still's Disease
      1. Know 4 clinical features
        1. fever (quotidian)
        2. "salmon-colored" rash that occurs with fever
        3. polyarticular arthritis
        4. lymphadenopathy/hepatosplenomegaly
      2. Know 4 laboratory features
        1. leukocytosis
        2. elevated ESR
        3. seronegative
        4. elevated ferritin level
  4. Spondyloarthropathies
    1. Common features of spondyloarthropathies
      1. Know 4 characteristics of inflammatory back pain
        1. insidious onset
        2. pain greater than 3 months duration
        3. morning stiffness > 30 minutes
        4. symptoms better with exercise, worsened with rest
      2. Know 2 common pathologic features(different than RA)
        1. enthesitis
        2. abnormal bone production
      3. Know 2 extraspinal, musculoskeletal features
        1. oligoarticular, asymmetric, arthritis of lower extrem
        2. dactylitis (sausage digits)
      4. Know 5 extra-articular features
        1. Uveitis (Iritis)
        2. Cardiac
          • Aortic insufficiency
          • Conduction abnormalities
        3. Pulmonary fibrosis
        4. Peripheral neuropathy
        5. Oral ulcers
      5. Know therapy of inflammatory back pain
        1. No medications alter underlying process
        2. NSAIDs may improve symptoms
        3. Posture and flexibility exercises very important
      6. HLA-B27
        1. Know when obtaining HLA-B27 can be useful:
          • patient whose pre-test probability of spondyloarthropathy is moderate (approx 50%)
        2. Know the prevalence of HLA-B27 in AS, Reiter's, Psoriasis and in the general population
          • Ankylosing Spondylitis
            White males: 90%
            Black males: 45%
          • Reiter's
            • 80%
          • Psoriasis
            with sacroiliitis: 50%
            without sacroiliitis: 20%
          • General population
            • White males: 6-9%
          Black males: 3-5%a
    2. Ankylosing Spondylitis
      1. Know
        1. back involvement predominates
        2. 25% may get peripheral arthritis
      2. Know 5 common radiologic findings
        1. symmetric sacroiliitis
        2. vertebral squaring
        3. "shiny" or "ivory" corners on vertebrae
        4. syndesmophytes
        5. joint ankylosis :("bamboo" spine in some)
    3. Reiter's Syndrome/Reactive Arthritis
      1. Know classic triad (usually not seen)
        1. arthritis
        2. conjunctivitis
        3. urethritis, cervicitis
      2. Know 2 initiating events
        1. dysentery
        2. sexually transmitted diseases
      3. Know 2 cutaneous manifestations
        1. keratodermia blenorrhagicum
          • psoriasis-like skin lesions
          • seen on palms, soles
        2. balanitis
    4. Psoriatic Arthritis
      1. Know that 5% of patients with psoriasis have arthritis
      2. Know 5 patterns of arthritis
        1. Oligoarticular, asymmetric involvement: > 50%
        2. Symmetric Polyarthritis (like RA): 25%
        3. Predominant DIP involvement: 5-10%
        4. Exclusive back involvement: 5%
        5. Arthritis Mutilans: 5%
      3. Know that nail changes are associated with arthritis
        1. 80% with arthritis have nail changes
      4. Know one characteristic radiologic finding
        1. "pencil-in-cup" erosions
    5. Enteropathic arthritis (Crohn's or Ulcerative Colitis)
      1. Know peripheral arthritis correlates with the activity of the bowel disease; the back disease does not
      2. Know 2 cutaneous manifestations
        1. pyoderma gangrenosum
        2. erythema nodosum
  5. Infectious Arthritis
    1. Gonococcal
      1. Know what demographic features suggest GC
        1. young, sexually active person
        2. women especially:
          • during pregnancy
          • just after menses
      2. Know 4 articular features
        1. migratory polyarthralgias in most
        2. monoarticular arthritis in 40%
        3. polyarthritis in up to 50%
        4. tenosynovitis in 67%
      3. Describe the associated rash
        1. small papules
        2. few in number
        3. frequently missed
      4. Know the yield of cultures from synovial, urethra/cervix and blood cultures for offending organism; know the yield of gram stain of synovial fluid
        1. synovial cultures Å in 50%
        2. blood cultures Å in 20%
        3. Urethral cultures Å (men) in 60%
        4. Cervical cultures Å (women) in 85%
        5. gram stain Å in 25%
      5. Know a basic treatment regimen
        1. IV ceftriaxone until significant clinical improvement than switch to oral antibiotics
    2. Non-gonococcal (adult)
      1. Know 3 common clinical features
        1. pain, swelling and severely affected ROM in joint
        2. usually monoarticular involvement
        3. fever in most
      2. Know the 2 joints most commonly affected
        1. knee (55%)
        2. hip (11%)
      3. Know the 2 most common organisms cultured
        1. Staph aureus (60%)
        2. Strep (group A) (16%)
      4. Know 3 unusual organisms and associated conditions
        1. Pseudomonas - IV drug abusers
        2. Staph epi - prosthetic joint infections
        3. Salmonella - patients with sickle cell
      5. Know at least 3 joints that can be infected in drug abusers that are not typically infected in other patients
        1. sternoclavicular
        2. sacroiliac
        3. pubic symphysis
      6. Know 3 qualities of joint fluid from an infected joint
        1. low viscosity
        2. opaque or purulent
        3. high WBC (usually > 50,000)
      7. Know the yield of synovial and blood cultures for offending organism; know the yield of gram stain of synovial fluid
        1. synovial cultures - nearly 100%
        2. blood cultures - 50%
        3. synovial fluid (centrifuged) gram stain
          • 75% in staph
          • 50% in gram negatives
      8. Know that serial closed-needle drainage of infected joint fluid is an accepted part of therapy in easily aspirated joints
      9. Know indications for arthrotomy (or arthroscopic drainage) of septic joint
        1. hip involved
        2. long duration (> 3 days since onset)
        3. prosthetic joint involvement
        4. difficult to access joint
        5. loculated fluid
        6. synovial WBC/volume don't Ø with repeat arthrocentesis
      10. Have a strategy in mind for empiric antibiotic therapy
        1. IV nafcillin for suspected community-acquired gram positive infection
        2. IV vancomycin for suspected MRSA infection
        3. IV 1st or 3rd generation cephalosporin + nafcillin or vancomycin for suspected gram negative infection
    3. Viral
      1. Know 5 viral infections that can present with a symmetric, polyarticular inflammatory arthritis
        1. Parvovirus B12
        2. Hepatitis B
        3. Rubella
        4. Mumps
        5. Enteroviruses: Coxsackie and Echoviruses
    4. Lyme
      1. Know the etiologic agent and vector of Lyme disease
        1. agent: Borrelia burgdorferi
        2. vector: ixodid ticks
      2. Know the 3 endemic areas in the U.S.
        1. Northwest: Northern MD to MA
        2. MN and WI
        3. Northern CA and OR
      3. Know the features of Erythema migrans
        1. erythematous annular lesion with central clearing
        2. appears 3-30 days after tick bite
        3. may see satellite lesions
      4. Know 4 features of early Lyme disease
        1. Malaise, fatigue
        2. Lymphadenopathy
        3. Polyarthralgias/myalgias
        4. Fever
      5. Know 3 manifestations of Lyme neurologic disease
        1. meningoencephalitis
        2. cranial neuropathies
        3. bilateral Bell's palsy
      6. Know the most common manifestation of Lyme carditis
        1. AV block
      7. Know the classic presentation of Lyme arthritis
        1. Intermittent large joint monoarthritis (typically the knee)
        2. Can be oligoarticular
      8. Know the therapy for Lyme disease:
        1. Early and EM: Tetracyclines > Amoxacillin
        2. Neuro:
          • Isolated Bell's: TCN for > 20 days
          • Others: Ceftriaxone or IV PCN
        3. Cardiac: Ceftriaxone or IV PCN
        4. Arthritis:
          • TCN for > 20 days
          • IV Ceftriaxone for failures
    5. Mycobacterium tuberculosis
      1. Know 3 most common musculoskeletal locations
        1. back (Pott's disease) in 50%
        2. Knee in 15%
        3. hip in 15%
      2. Know what % with musculoskeletal TB have evidence of coexistent pulmonary infection
        1. 10-50%
      3. Know clinical features
        1. insidious onset
        2. monoarticular in 85%
      4. Know synovial fluid findings
        1. cloudy
        2. high WBC (10,000 with PMN predominance)
        3. low viscosity
      5. Know yield of synovial fluid smear and culture
        1. smear Å in 20%
        2. culture Å in 80%
    6. Fungal
      1. Know that fungal arthritis has insidious onset and found frequently in patients with underlying debilitating illnesses
    7. HIV-associated arthritis
      1. Know 4 musculoskeletal conditions seen in HIV
        1. Reiter's syndrome
        2. Psoriatic arthritis
        3. Fibromyalgia
        4. Polyarthralgias
      2. Know that RA is rarely seen in HIV infection

III. Systemic Diseases

  1. Systemic Lupus Erythematosus
    1. Know the following clinical manifestations of SLE
      1. Mucocutaneous (5)
        1. malar rash
        2. subacute cutaneous lupus
        3. discoid lupus
        4. oral ulcers
        5. alopecia
      2. Lymphoreticular (2)
        1. lymphadenopathy
        2. splenomegaly
      3. Cardiovascular-pulmonary (7)
        1. Plerisy (+/- effusions)
        2. Pericarditis (+/- effusions)
        3. Pneumonitis
        4. Pulmonary hemorrhage
        5. Pulmonary hypertension
        6. Endocarditis (noninfectious)
        7. Raynaud's
      4. Gastrointestinal (3)
        1. Peritonitis (serositis)
        2. Pancreatitis
        3. Mesenteric vasculitis
      5. Genitourinary (1)
        Glomerulonephritis
          • Mesangial
          • Membranous
          • Membranoproliferative
          • Focal Proliferative
          • Diffuse proliferative
      6. Neurologic (4)
        1. Organic Brain Syndrome
        2. Seizures
        3. Psychosis
        4. CVA/ focal defects
      7. Hematologic (4)
        1. leukopenia
        2. lymphopenia
        3. hemolytic anemia
        4. thrombocytopenia
        ? anemia of chronic disease
      Serologic (5)
        1. ANA (Å in 98%)
        2. Sm
        3. DsDNA
        4. False-positive VDRL
        5. LE cell
        6. ? SSA/SSB
        ? hypocomplementemia
    2. Know 4 most common causes of drug-induced lupus
      1. Procainamide
      2. Isoniazide
      3. Hydralazine
      4. a methyl dopa (aldomet)
    3. Describe the 2 dominant features of drug-induced lupus
      1. arthritis
      2. serositis
    4. Describe 5 clinical and serologic features that distinguish drug-induced lupus from native SLE
      1. males = females
      2. no renal or CNS disease
      3. disease resolves after DC offending medication
      4. no: DsDNA, low complements, Sm
      5. Diffuse ANA with histone antibodies only
    5. Know the ACR criteria for SLE
    6. List 2 manifestations of active SLE that can be treated with the following doses of prednisone
      1. low (10-20 mg)
        1. arthritis
        2. rash
      2. moderate (20-40mg)
        1. serositis
        2. low-grade hematologic abnormalities
      3. high (> 40 mg)
        1. active nephritis
        2. CNS disease
        3. vasculitis
        4. severe hematologic abnormalities
  2. Sjogren's Syndrome
    1. Know 2 demographic features
      1. 90% patients are women
      2. usually after age 50
    2. Know clinical features
      1. inflammatory autoimmune process of exocrine glands
        1. dry eyes with Ø tear production and corneal erosions
        2. dry mouth (increased risk for dental caries)
        3. parotid swelling
      Extra-glandular features
        1. arthralgias/arthritis in 60%
        2. Raynaud's phenomenon in 40%
        3. lymphadenopathy
        4. purpura
        5. increased risk for development of lymphomas
    3. Know laboratory features
      1. ANA Å in 90%
      2. RF Å in 60%
      3. Hypergammaglobulinemia
      4. SSA/Ro in 90%
      5. SSB/La in 80%
      6. Abnormal lip (minor salivary gland) biopsy
  3. Antiphospholipid Antibody syndrome (APLS)
    1. List 5 clinical features of APLS
      1. recurrent fetal loss
      2. recurrent venous/arterial thrombosis
      3. livedo reticularis
      4. thrombocytopenia
      5. valvular vegetations
    2. List 3 laboratory features of APLS
      1. "lupus anticoagulant"
        1. prolonged APTT (despite mix with normal plasma)
        2. prolonged Russell Viper Venom Time
        3. prolonged Kaolin Clotting time
      2. False positive VDRL/RPR
      3. Anticardiolipin antibodies (IgG or IgM) in a moderate or high titer
  4. Inflammatory myositis
    1. List the diagnostic criteria for inflammatory myositis
      1. symmetrical, proximal muscle weakness
      2. elevation of skeletal-muscle enzymes
        1. CK
        2. aldolase
        3. AST
        4. ALT
        5. LDH
      3. EMG evidence of inflammatory myositis
      4. muscle biopsy evidence of inflammatory myositis
      5. need 3 of 4 to make diagnosis of polymyositis and typical skin features (plus 3 of 4 criteria) to make diagnosis of dermatomyositis
        1. Heliotrope: violaceous discoloration/swelling of eyelids
        2. Gottron's sign: scaly, erythematous dermatitis over dorsal joint surfaces
        3. Gottron's rash: above rash elsewhere on body
        4. Shawl rash: erythema/scaling in V of neck
        5. Mechanic's hands
    2. Give a differential diagnosis for inflammatory myositis
      1. thyroid disease
      2. neuropathy
      3. inherited myopathy (i.e. muscular dystrophy)
      4. electrolyte disorders
      5. metabolic or mitochondrial myopathy
      6. Parasitic infiltration (trichosomiasis)
      7. medication side effect
      8. toxin exposure
    3. Give features of inflammatory myositis that differentiate it from neuropathy
      1. proximal weakness
      2. sparing of sensation and reflexes
      3. sparing of facial/pharyngeal muscles
    4. List 3 most common inflammatory myopathies a) Polymyositis b) Dermatomyositis c) Inclusion Body Myositis
    5. Know that dermatomyositis is associated with malignancies and what needs to be done to evaluate this
      1. Complete H & P
      2. CBC, General Screen, UA
      3. CXR
      4. Prostate exam in men
      5. Breast, mammogram, pelvic and PAP in women
      6. ? Flex sigmoidoscopy
    6. Know the clinical/ lab features of the anti-synthetase syndrome
      1. Laboratory features: antibodies directed against amino acid tRNA synthetases (Jo-1 or histidyl tRNA synthetase is most well known
      2. Clinical features
        1. Inflammatory myositis
        2. Interstitial lung disease
        3. Raynaud's
        4. Arthritis
        5. Mechanic's hands
    7. Describe approach to suspected steroid myopathy
      1. no specific tests helpful
        1. CK NL
        2. muscle biopsy nonspecific
        3. EMG usually nonspecific
      2. provocative challenge with higher dose vs. rapid taper
  5. Scleroderma
    1. CREST (limited systemic sclerosis)
      1. Describe the clinical manifestations of CREST
        1. Calcinosis
        2. Raynaud's
        3. Esophageal Dysmotility
        4. Sclerodactyly
        5. Telangiectasias
      2. Know 1 life-threatening (late) complication of CREST
        1. pulmonary hypertension
      3. Describe the extent and time course of skin thickening
        1. usually limited to hands and forearms
        2. can be seen on face and neck
        3. progression very slow
        4. usually seen after years of Raynaud's
      4. Describe the natural history of CREST
        1. NL life-span unless develop pulmonary hypertension
        2. Centromere antibody predicts good prognosis
    2. Diffuse Scleroderma
      1. Describe 6 clinical manifestations
        1. rapid progression of diffuse skin thickening
        2. interstitial pulmonary disease
        3. myocardial fibrosis, pericarditis
        4. 'scleroderma renal crisis'
          • rapid rise in blood pressure
          • rapid decline in renal function
        5. myositis
        6. arthritis
      2. Describe the natural history of diffuse scleroderma
        1. 77% survival at 5 years
        2. renal, cardiopulmonary disease most common causes of mortality
        3. SCL-70 (anti-topisomerase) and nucleolar pattern of ANA predict poor prognosis
      3. Describe the therapeutic goals in 'renal crisis'
        1. ACE inhibitors dramatically decreased mortality
        2. rigid control of blood pressure
  6. Vasculitis
    1. General
      1. List 5 common but nonspecific laboratory features:
        1. ­ ESR/CRP
        2. Anemia
        3. Thrombocytosis
        4. leukocytosis
        5. Ø albumin
      2. List 5 causes of pulmonary-renal syndrome
        1. SLE
        2. Wegener's Granulomatosis
        3. Goodpasture's syndrome
        4. Churg-Strauss
        5. Microscopic PAN
    2. Polymyalgia Rheumatica
      1. List 2 demographic features of PMR
        1. age > 50 (usually > 60)
        2. European-American (>95%)
      2. List 3 clinical or laboratory features
        1. pain/ache in shoulder/hip girdle
        2. ­ ESR (usually > 50)
        3. rapid and dramatic response (within 12-36 hours) to low dose prednisone (10-20mg)
    3. Giant Cell Arteritis (Temporal Arteritis)
      1. List 2 demographic features
        1. age > 50 (usually > 60)
        2. European-American (> 95%)
      2. List 6 clinical/laboratory features
        1. new onset temporal headache
        2. jaw/tongue claudication
        3. scalp tenderness
        4. vision changes (amaurosis fugax or diplopia)
        5. enlarged, tender temporal artery
        6. ­ ESR (frequently > 80)
      3. Describe the diagnostic and therapeutic approach
        1. ESR, CRP, CBC
        2. start high-dose corticosteroids (60 mg/day)
        3. obtain temporal artery biopsy (after starting steroids)
    4. Polyarteritis Nodosa
      1. List the following clinical features
        1. skin (4)
          • palpable purpura
          • palmar lesions
          • livedo reticularis
          • digital ischemia
        2. musculoskeletal (1)
          • arthralgias/arthritis
        3. renal (3)
          • glomerulonephritis
          • hypertension
          • hematuria/proteinuria
        4. neurologic (2)
          • mononeuritis multiplex
          • sensorimotor polyneuropathy
        5. miscellaneous (1)
          • testicular pain
      Describe the diagnostic approach
        1. tissue biopsy showing vasculitis
          • vasculitis rarely seen on renal bx
        2. angiogram showing aneurysms and narrowing
        3. P-ANCA may be seen in some
        4. Hepatitis B surface antigen (in ª 15%
    5. Wegener's Granulomatosis
      1. List the clinical features
        1. Upper respiratory tract (4)
          • sinusitis
          • epistaxis
          • serous otitis media
          • nasal ulcerations
        2. Lower respiratory tract (4)
          • cough
          • hemoptysis
          • pulmonary infiltrates
          • pulmonary nodules
        3. Renal (1)
          • glomerulonephritis
        4. Musculoskeletal (1)
          • arthralgia/arthritis
        5. Skin (2)
          • nodules
          • palpable purpura
        6. Systemic (3)
          • fever
          • malaise
          • weight loss
      2. Describe the diagnosis
        1. C-ANCA Å in 85-90% with active, systemic disease
        2. biopsy of tissue showing necrotizing, granulomatous vasculitis
          • vasculitis rarely seen on renal biopsy
    6. Henoch/Schonlein Purpura
      1. List the classic triad in HSP
        1. hematuria
        2. abdominal pain
        3. palpable purpura (biopsy showing leukocytoclastic vasculitis with IgA deposits)
    7. Cryoglobulinemia
      1. Know what cryoglobulins are
        1. proteins that precipitate at temperatures below body temperature
        2. associated with a occlusive vasculopathy
      2. Know the disease most often associated with cryoglobulinemia
        1. Chronic hepatitis C infection
    8. Cholesterol Emboli Syndrome
      1. Know clinical features of cholesterol emboli syndrome
        1. Purpuric lesions, typically on lower extremities
        2. Usually follows instrumentation of large, atherosclerotic vessels (cardiac catheterization is prototype)
        3. May have associated:
          • elevated ESR
          • low complement levels
          • eosinophilia

IV. Bone Disorders

  1. Osteoporosis (Know risk factors)
    1. age
    2. heredity
    3. gender (female)
    4. hormonal (estrogen and testosterone)
    5. tobacco use
    6. alcohol use
    7. low activity levels
    8. low body weight
    9. drugs
      1. steroids
      2. thyroid (if TSH suppressed)
      3. heparin
    10. Diseases
      1. renal failure
      2. hepatic failure
      3. myeloma
      4. hypercalciuria
    11. falls risk
  2. Avascular Necrosis
    1. know typical locations
      1. femoral neck
      2. humeral head
      3. talus
      4. scaphoid
    2. Risk factors
      1. steroids
      2. trauma
      3. SLE
      4. RA
      5. hemoglobinopathies
      6. osteoporosis
    3. Diagnosis:
      1. MRI: most sensitive
      2. Plain films: staging system
      3. CT
      4. Bone scan
    4. Most progress and if symptomatic will require surgery
  3. Insufficiency Fractures
    1. know definition:
      1. fracture
      2. normal or abnormal loading
      3. structurally weak bone
    2. know typical clinical features
      1. usually in elderly females
      2. usually unaccompanied by significant trauma
    3. know typical locations
      1. femoral neck
      2. vertebral bodies
      3. sacrum/pelvis
      4. ribs


V. Neurovascular Disorders

  1. Reflex Sympathetic Dystrophy
    1. Know historical and exam features
      1. hyperpathia (persistent pain after DC stimuli)
      2. allodynia (pain from nonpainful stimuli)
      3. vasomotor instability
      4. pain
      5. atrophic skin changes
    2. Know 3 diagnostic modalities that could confirm suspicions
      1. osteopenia on plain films
      2. asymmetric uptake on 3-phase bone scan
      3. chemical sympathectomy & symptom improvement
    3. Know 2 therapeutic modalities
      1. physical therapy
      2. corticosteroids
  2. Cervical and Lumbar Radiculopathies
    1. Know 6 common sensory distributions
      1. C6
      2. C7
      3. C8
      4. L4
      5. L5
      6. S1
    2. Know distributions of deep tendon reflexes
      1. biceps C5
      2. triceps C6
      3. brachioradialis C7
      4. patellar L4
      5. Achilles S1
  3. Entrapment Neuropathies
    1. Carpal Tunnel Syndrome
      1. Know 3 presenting features
        1. pain, paresthesias in 1st-3rd fingers
        2. can involve wrist and forearm
        3. typically nocturnal
      2. Know 5 exam features
        1. Ø 2-point discrimination
        2. Ø pinch strength (late)
        3. thenar atrophy (late)
        4. Tinnel's sign - tapping over nerve
        5. Phalen's sign - pressing flexed wrists together
      3. Know 3 therapeutic options
        1. splint
        2. inject
        3. operate
      4. Know indications for referral for surgery
        1. motor involvement
        2. intractable pain unresponsive to conservative therapy
    2. Ulnar Nerve Entrapment
      1. pain, paresthesias in 4th, 5th fingers
      2. know locations of entrapment
        1. elbow (cubital tunnel)
        2. wrist (Guyon's canal)
  4. Neuroarthropathies
    1. know clinical features
      chronic, painless (in 50%) with deformity
    2. know typical disease associations
      1. diabetes ... foot
      2. syphilis ... lower extremity
      3. syringomyelia ... upper extremity
    3. know typical radiologic presentation
      1. destruction
      2. debris
      3. dislocation
      4. osteophytes
  5. Raynaud's
    1. Know typical symptoms: color changes associated with cold
    2. Know 2 'types' of Raynaud's
      1. primary (idiopathic)
      2. secondary (part of systemic autoimmune disease)
    3. Know 3 features associated with systemic autoimmune disease
      1. nailfold capillary abnormalities
      2. ANAÅ
      3. older age of onset
    4. Know 2 conservative therapeutic maneuvers
      1. avoidance of stimuli (cold, etc)
      2. vasodilators
    5. Have at least 7 point differential
      1. Idiopathic
      2. SLE
      3. Systemic Sclerosis
        1. CREST (limited systemic sclerosis)
        2. Scleroderma (diffuse systemic sclerosis)
      4. Sjogren's syndrome
      5. Vasculitis
      6. Malignancies
        1. myeloma
        2. macroglobulinemia
      7. Previous cold injury


VI. Medications

  1. Know
    1. major indications
    2. drug interactions
    3. major or unique side effects
  2. NSAIDS
    1. Inflammatory arthropathies
    2. antihypertensives, warfarin
    3. Ulcers/gastritis, renal insufficiency, bleeding, bruising
      1. aseptic meningitis with propionic acids
      2. altered CNS with indocin
      3. nonacetylated salicylates with no PG inhibition and no platelet aggregation
  3. Corticosteroids
    1. Many
    2. -
    3. Major Side Effects
      1. osteopenia
      2. HPA axis suppression
      3. immune suppression
      4. hypertension
      5. petechiae/bruising
      6. acne
      7. hirsuitism
      8. hyperglycemia
      9. centripetal fat deposition
      10. hypokalemia
      11. striae
      12. glaucoma
      13. accelerated cataract formation
      14. accelerated atherosclerosis
      15. psychosis/mood disorder
      16. insomnia
  4. Hydroxychloroquine
    1. SLE, RA, cutaneous lesions
    2. -
    3. Retinal toxicity, GI
  5. Sulfasalazine
    1. RA, Spondyloarthropathies
    2. -
    3. GI, cytopenias, LFTs, oligospermia, hemolytic anemia in G6PD deficient patients
  6. Gold
    1. RA, Psoriasis
    2. ACE inhibitors (nitritoid reaction)
    3. proteinuria, stomatitis, nitritoid reaction, rash, cytopenias
  7. Methotrexate
    1. RA, Myositis, SLE, Vasculitis, Sarcoidosis
    2. sulfa medications, probenecid
    3. cytopenias, cirrhosis/fibrosis, GI, stomatitis, pneumonitis
    4. List 4 laboratory tests that need to be monitored periodically
      1. CBC
      2. AST
      3. ALT
      4. Albumin
    5. List 2 things to caution patients about when using MTX
      1. avoidance of alcohol
      2. effective birth control
  8. Azathioprine
    1. RA, SLE
    2. Allopurinol
    3. cytopenias, hepatitis, aseptic meningitis, risk for lymphomas
  9. D-Penicillamine
    1. RA, Systemic sclerosis
    2. -
    3. cytopenias, proteinuria, autoimmune diseases, dysgeusia
  10. Cyclophosphamide
    1. SLE, Vasculitis
    2. ? allopurinol (­ marrow toxicity)
    3. cytopenias, bladder inflammation/malignancy, ovarian failure
  11. Colchicine
    1. Gout, CPPD
    2. -
    3. GI, cytopenias
  12. Allopurinol
    1. indications
      1. > 1100 mg/24hour urine uric acid
      2. tophi
      3. renal insufficiency
      4. renal stones
      5. anticipated tumor lysis
      6. serum uric acid > 11.0 mg/dl (controversial)
    2. azathioprine
    3. Allopurinol hypersensitivity syndrome: Know the clinical features:
      1. occurs in 2-3 %
      2. 30% who get it have fatal reaction
      3. occurs 2-3 weeks most commonly after starting it
      4. fever, eosinophilia, rash
      5. renal insufficiency may predispose

II. Laboratory Tests

  1. ANA
    Patterns
      1. Know
        1. specific or not
        2. antigens that antibodies are directed against
      2. Speckled (Most common)
        1. Nonspecific
        2. antibody directed against extractable nuclear antigens
      3. Homogeneous
        1. Nonspecific
        2. directed against histone
      4. Centromere
        1. Specific
        2. associated with CREST
      5. Nucleolar
        1. Somewhat specific
        2. associated with Scleroderma
  2. ESR
    1. Know that ESR is nonspecific marker of inflammation
    2. Know Age correction
      1. Men: Age/2
      2. Women: (Age + 10)/2
    3. Know differential of ESR >100
      1. 33% due to infections
      2. 33% autoimmnune disease
      3. 25% malignancy
  3. RF
    1. Found in 80% RA patients
    2. Associated with more agressive disease
  4. Extractable Nuclear Antigens (know disease association)
    1. Sm- associated with SLE
    2. RNP - associated with MCTD
    3. SSA/SSB - associated with:
      1. Sjogren's syndrome
      2. SLE
      3. Neonatal lupus syndrome
  5. Miscellaneous (Know disease associations)
    1. DsDNA
      1. associated with SLE
    2. ANCA
      1. cANCA
        1. associated with Wegener's Granulomatosis
      2. pANCA
        1. associated with microscopic PAN, other vasculitides, limited Wegener's, others
    3. Jo-1 (anti-histidyl tRNA synthetase)
      1. associated with polymyositis and interstitial lung disease
    4. Anti histone antibody
      1. associated with drug-induced lupus
    5. HLA
      1. B27-spondyloarthropathies
      2. DR4-RA
      3. DR3-SLE,Sjogren's syndrome
    6. SCL-70 (anti-topoisomerase)
      1. diffuse scleroder