Title: MKSAP 12 Rheumatology Board Review Cases
1MKSAP-12 RHEUMATOLOGY BOARD REVIEW INSTRUCTIONS
- The MKSAP-12 Board Review for Rheumatology
includes - a printed copy of 70 case presentations and
questions, entitled MKSAP-12 Rheumatology page
57-75, available from the Olive View-UCLA Medical
Center Department of Medicine or Library AND - this Powerpoint presentation by Dr. Philip
Clements and Dr. Andrew Wong, which supplements
the MKSAP-12 Rheumatology question and answer
section, page 103-128. - Open the powerpoint file (double click). The
opening screen will show the menu bar on top.
Select SLIDE SHOW on the menu bar, then choose
the first command View Show (one click). - To advance, use your left mouse button, or your
DOWN arrow key. To go back, use your UP arrow
key. - To QUIT the presentation, hit ESC (escape).
You will return to the opening screen, then hit
Exit to quit Powerpoint. You will then return to
the website.
2Case 1
- Radiologic hallmarks of OA
- Joint space narrowing
- Osteophytes
- Subchondral bony sclerosis
- Subchondral cysts
- PE shows marked loss of motion (cannot fully
extend hip by 30o 10o total rotation) - Joint replacement is indicated when all other
measures no longer control pain. - Often it is pain at night that convinces the
patient to have surgery.
3Case 2
- Bimodal distribution of deaths in SLE
- Early SLE complications and infection
- Late Accelerated atherosclerotic CV disease
- Pertinent findings or lack of them
- Substernal chest pain in 39 y/o F with SLE
- Pain not positional nor pleuritic
- Lack of antiphospholipid antibodies
- T-wave changes
4Case 3
- Sjogrens is an autoimmune inflammatory disease
which destroys the exocrine glands (eyes, mouth,
bronchial, vaginal). - It is associated with multiple autoantibodies
- ANA 80
- Rheumatoid factor 90
- SSA / SSB 60-70
- Elevated Ig levels 80
- Elevated sed rate 80-90
5Parotid gland biopsy
Inflammatory lymphocytes
Damaged gland
6Case 4
- Sudden onset of low back pain after activity
pain radiating down right leg. Straight leg
raising positive (raising leg makes pain go down
the leg). Neuro intact. - Dx most likely acute low back (disc) or facet
joint strain with radicular pain. - Rx Pain meds, corset (maybe), encourage activity
as tolerated. - Imaging has high false positive rate.
- Image if suspect CA, osteoporotic fracture
7Case 5
- Suspect limited Wegeners (a chronic
granulomatous vasculitis), which can present as
an inflammatory process of larynx (producing
subglottic stenosis), ears and/or eyes. - Chances of finding Wegeners (granulomatous
vasculitis) by tissue bx - Upper airway bx may show inflammation, necrosis,
granulomasrarely vasculitis. - Renal bx focal GN, no Ig depositis
(pauci-immune) - Open lung granulomatous inflammation AND
vasculitis - ANCA (esp Proteinase 3) strongly supports
Wegeners - Other rheum serologies negative (ANA, RF, SSA,
SSB)
8Case 6
- L/S spine degenerative disease can affect the
disc and facet joints. - Painful syndromes can occur in soft tissues
downstream, in a Christmas-like affect
(paraspinal muscles, troch bursa, ant thighs)
9Case 7
- Hip pain often refers to groin, buttock and
anterior thigh ( L3, L4). - Acute or subacute hip pain in SLE patients on
steroids should suggest possibility of AVN. - MRI diagnostic procedure of choice for AVN.
- Disease process
- Ischemia infarcts medullary bone .
- Cartilage and thin layer of bone fed by synovial
fluid. - Dead bone and cartilage eventually collapse.
- Then OA takes over.
10AVN
Early
Late
11Case 8Enteropathic arthropathies
- Inflammatory bowel disease
- Enterogenic reactive arthritis
- Whipples
- Celiac disease
- Intestinal bypass surgery
12Case 8Celiac disease
- Arthralgias/arthritis occur in 28 of Celiac pts.
- Patterns of arthritis
- Polyarthritis
- Oligoarthritis
- Spondylitis
- Onset of arthritis may precede or follow GI Sxs
- A gluten-free diet reverses the arthritis.
13Case 9
- Hypertrophic pulmonary osteoarthropathy
- Diffuse bilateral upper and lower extremity pain
- COPD in older smoker
- Clubbing
- Tender, painful distal ulna and radius, tibia
- Non-inflammatory joint fluid
- High sed rate
- Look for lung CA with chest x-ray.
- Document HPO with radioactive bone scan or
periosteal elevation in long bone x-rays.
14HPO
15Case 10
- Inflammatory polyarthritis of short duration
think viral illness. - Characteristics of Parvovirus B19 infection
- Women who care for sick children
- Transient generalized rash can occur
- Usually small joint swelling, and pain is usually
symmetrical - Arthritis usually resolves in 2-4 weeks
- IgM parvovirus titers positive
- Occasionally RF and ANA may be positive
evanescently - Non-erosive arthritis
16Parvovirus hands
17Case 11
- CPPD secondary to hemochromatosis
- OA like changes in MCPs unusual in OAusually
means CPPD - OA changes in knee may be from CPPDsupported by
chondrocalcinosis (cartilage calcifications) of
menisci - Liver biopsy to assess iron load in
hemochromatosis. - Treating hemochromatosis changes patients
livesdoesnt help the joint disease.
18CPPD
OA-like changes of MCP joints
Chondrocalcinosis
19Case 12
- Polyarthritis, diarrhea, pleuritic chest pain and
weight loss (from malabsorption) in middle-aged
maleconsider Whipples in Diff Dx. - Small intestine biopsy looking for PCR sequence
of Tropheryma whippelii, which is essential to
making the diagnosis. - Lymph node biopsy looking for PAS-positive
macrophages supports Dx.
20Case 13
- Amyloid (?2-microglobulin) suggested by
- 13 years of ESRD on hemodialysis (50 p 13 yrs).
- Numbness tingling in thumb and index finger
suggests CTS (median nerve distribution). - Pain on motion and LOM of shoulders.
- Treatment of choicerenal transplant and get off
hemodialysis.
21Case 14
- Antiphospholipid syndrome suggested by
- Blood clots
- 2 or more miscarriages or abortions (esp 1st
trimester) - 2 or 3 of these lab abnormalities for APS
- Anticardiolipin Abs (IgG or IgM)
- Lupus anticoagulant (DRVVT)
- ?2-glycoprotein
- This patient had no lab abnormalities of APS and
only one miscarriage
22Case 15
- Young male with 2 DVTs (17 y/o and now at 24
y/o). - Family history of DVTs.
- Most frequent inherited cause of DVT in white pts
is Factor V Leiden deficiency. - Work up for APS and other inherited causes of
hypercoaguability (i.e., deficiencies of protein
C or S and antithrombin 3).
23Case 16
- Pt had some improvement, but is still weak and
has high CPKs while on high doses of steroidsnot
adequately controlled. As such at high-risk of
steroid AEs. - Add DMARD
- Methotrexate most widely use
- RCT showed benefit from Imuran (azathioprine)
24Case 17
- Amyotrophic dermatomyositis (skin rash without
muscle weakness). - Without muscle weakness there is no
myositisparaphase from Carl Pearson, MD - Dermatomyositis in older person (gt60 y/o) may
mean cancercheck the usual suspects (breast,
cervix, ovaries, chest)
25DM rashes
26Case 18
- Henoch-Schonlein purpura (HSP)
- Antecedent viral infection (esp URI)
- Arthralgias and/or arthritis
- Abdominal pain frequent
- Palpable purpura, esp lower extremities
(leukocytoclastic vasculitis and IgA deposits on
punch biopsy) - Some renal involvement (esp GN) commonIgA
positive immunofluroescence on biopsy - Usually treat conservatively.
- Severe GI or renal involvement may require
steroids.
27HSP purpura
28Case 19Polymyalgia rheumatic
- Shoulder and pelvic girdle pain/stiffness, esp AM
- Fatigue, anorexia, mild weight loss
- Occasionally mild synovitis
- Mild anemia
- Over 55 years old
- Sed rate usually elevatedoccasionally not by
much - Increased alkaline phosphatase in 1/3 of patients
- Dramatic response to therapeutic trial of low
dose prednisone (20 mg/d), usually within 24-72
hours.
29Case 20
- Transverse myelitis of spinal cord in SLE
- Sudden onset of symptoms
- Neurologic deficit of lower extremities with
urinary and/or fecal incontinence suggest cord
disease - Weakness of both legs (subjective and objective)
- Difficulty urinating, fecal incontinence
- Upper and lower motor neuron findings
- Rectal exam shows weak anal sphincter
- MRI most sensitive test for finding edema of the
spinal cord - Check for antiphospholipid antibodies
30Case 21
- This part of the clinical picture suggests
Reactive Arthritis (AKA Reiters) - 4 week hx diarrhea
- 3 week hx asymmetric polyarthritis, predominantly
affecting lower extremities, with enthesopathy
and skin lesions on bottom of feet. - The 3 month hx of temp to 101o F, fatigue, 22 lb
weight loss suggests something in addition. - Anyone with new onset Reactive Arthritis
(Reiters) deserves an HIV test.
31Reiters
32Anyone with new onset reactive arthritis deserves
and needs to be tested for HIV.
33Case 22
- 0 1 2 3 4 5 6 5 4 3 2 1 0
- Palindrome It leaves as it came.
- After one or two weeks of arthritic complaints,
the patient and her/his joints return to their
previous conditionwithout a trace. The episodes
recur and leave without a trace. - Madam, Im Adam (madam im adam)
34Case 23
35Hepatitis C associated syndromes
- Vasculitis (essential mixed cryoglobulinemia)
- Digit gangrene
- Nephritis
- Mononeuritis multiplex (foot drop, wrist drop)
- Arthritis
- Inflammatory polyarthritis (clinically can look
like RA but is usually nonerosive) - Rheumatoid factor positive
36Case 24Adult Stills
- High spiking fevers
- Arthralgias, arthritis
- Maculopapular rash
- Lymphadenopathy
- Serositis
- Sore throat (90), cultures negative
- Negative RF and ANA
- Leukocytosis
- Hepatosplenomegaly
37Case 25
- CPPD
- Short stubby crystal
- Weakly positively birefringent under polarized
compensated light - Verboten meds
- Colchicine Q2h is in-humane
- No NSAIDs with renal insuff
- Allopurinol doesnt work here
- Intra-articular steroid best Rx in renal or GI
disease
Regular light
Polarized
Compensated light
38Case 26
- Five factors differentiate inflammatory back
pain - Onset before 40 y/o
- Insidious onset
- Persistence for at least 3 months
- Associated morning stiffness
- Improvement with exercise
- Only 10-20 of male first-degree relatives who
inherit B-27 actually develop AS.
39Lumbar strain
- Mechanical back pain
- Sudden onset (only half remember an inciting
incident) - Minimal stiffness
- Hurts more with exercise
- Gets better lying down
- Pain may accentuate with cough or straining
- Motion may be limited but Schober usually OK
40Case 27
- Chemotherapeutic agents mask the inflammatory
aspects of hepatitis. Hepatitis may flare when
pt taken off MTX. Better in NOT to give MTX in
the face of Hep C. - HIV pts may convert to full-blown AIDS if given
MTX. AVOID MTX in HIV potential pts (new
Reactive Arthritis) until their HIV tests are
negative.
41Case 28
- Polymyalgia Rheumatica
- Profound limb girdle stiff
- Anemia
- Elevated sed rate
- Very responsive to 15-20 mg
- prednisone QDRx trial
- Vascular symptoms may indicate concomitant
temporal arteritis - Headache
- Jaw claudication
- Visual changes (amaurosis)
- Scalp tenderness
- Cough
42Case 29
- 1st CMC joint frequent site for OA.
- Tenderness on the joint.
- Crepitus, pain on motion of 1st CMC
- Extensor tendons of thumb not tenderand
Finkelsteins negative (for de Quervains).
43Case 30
- Trochanteric bursitis is often a sign of
something else (hip OA, sore feet, new shoes). - Patients say hip hurts but point to lateral
hip. - Tender lateral femur, about 4-6 inches below
tuberosity. - Injection best choicedirect Rx without systemic
toxicity.
Troch bursa
44Case 31
- Wait 2 wks after attack to start uricosuric
agent. - Benemid is first choice uricosuric agent.
- Allopurinol first choice if
- Creatinine gt2.0 mg/dl
- Tophi
- Urine uric acid gt800mg per 24 hours
- Chemotherapy
- Hx of renal stones
- Allergy to Benemid
45Case 32
46Case 32
- By history and exam, his RA has burned out.
Some patients may reactivate when they become
more active physically. - Probably doesnt need 7.5 mg prednisonereduce to
5 or less mg daily, a dose which preserves bone. - Pts on long term steroids need prophylactic
bisphosphonates or calcitonin started when
steroids are started.
47Case 33Lupus-like syndrome Drugs implicated
- Common
- Procainamide
- Hydralazine
- Uncommon
- Minocycline
- ?-blockers
- D-penicillamine
- Isoniazid
- Quinidine
- PTU
- Hydantoins
- Trimethodione
- Chlorpromazine
48Case 34
Peau dorange
- Rule of thumb
- No sclerodactyly
- No Raynauds/nailfold changes
- No ANA
- Equals NO SCLERODERMA!
- Think of some other entity, like eosinophilic
fasciitis (EF). - EF usually has high percent eosinophils on WBC
diff. - Bx for EF requires open, deep fascial biopsynot
punch.
49Case 35
- Diabetic neuroarthropathy
- Affecting intertarsal tarsometatarsall joints
here. - Marked destruction and erratic calcifications.
Normal
- In spite of marked erosions, may have little
pain, unless infected. - Sensation and proprioception usually impaired.
Diabetic
50Case 36
- Five factors differentiate inflammatory back pain
(spondyloarthropathies or SAs) - Onset before 40 y/o
- Insidious onset
- Persistence for at least 3 months
- Associated morning stiffness
- Improvement with exercise
- NSAIDs are for pain in SAs.
- Patient probably has a spondyloarthropathy
(undifferentiated) and needs to learn stretching
exercises and posture training.
51Case 37
- ELISA testing for Borrelia burgdorferi
notoriously gives false positive results (3-7
normals). - Needs confirmation by Western blot.
- Absence of IgG Ab at 3 years (in pt not Rxd with
Abx) is against active or ongoing Lyme. - Pts knee problem probably OARx with
acetaminophen.
E. Chronicum migrans
52Case 38
- RCTs have shown Omega-3 fatty acids and high
salmon/halibut diets to be anti-inflammatory. - Lose 8 strength per week by immobilizing.
- Temperature in joint increases with applied heat.
- Glucosamine/chondroitin are mildly
anti-inflammatory and reduce the pain of OA.
Acupuncture reduces pain, not inflammation.
53Case 39
- SSc is associated with esophageal hypomotility,
leading to dysphagia and dyspepsia. - SSc pt with increasing dysphagia and wt loss
needs evaluation of esophagus for stricture or
obstruction (not an UGI).
54Case 40Remitting, Seronegative Symmetrical
Synovitis with Pitting Edema (RS3PE)
- Usually in older persons.
- Pitting edema of hands.
- Swelling reduces the ability to make a fist.
- May be related to polymyalgia rheumatica.
- Very responsive to prednisone and not to standard
NSAIDs. Remember to start bisphosphonate or
calcitonin to prevent bone loss.
55Case 41
- Patient quotes
- I hurt all over.
- I feel like a truck hit me.
- This fatigue is the worst.
- I dont sleep worth a damn.
d
d
56Case 42
- The history and physical findings suggest an
ankle strain, caused by putting her weight down
on her left foot in a particularly uneven area. - Adequate exam and evaluation of range of motion
is hindered by acute swelling.
- Treatment plan
- Follow-up exam
- Apply ice
- Use brace to promote early ambulation
57Case 43
- Migratory polyarthritis.
- Skin pustules.
- Joint effusions are often inflammatory (wbcgt2.0)
but usually sterile.
- Culture portals of entry (pharynx, anus,
urethra). Culture pustule also. - Discount a sexual history which is negative.
58Disseminated Gonococcal Infection
- In subjects lt40 years old, GC is the biggest
cause of joint infection. - Since STDs tend to occur together, test for
chlamydia, syphilis and HIV. - Also Rx blind for chlamydia.
- Dx by clinical suspicion and culturing portals of
entry (throat, anus and urethra). - Best culture is directly onto Thayer-Martin
medium at bedside.
59Case 44
Ruptured Bakers Cysts
Crescent sign
60Pseudo-phlebitis of ruptured popliteal (Bakers)
cyst
- Some signs of ruptured cyst may resemble
phlebitisAbrupt onset of calf swelling,
tenderness, positive Homans sign. - Other characteristics of ruptured cyst differ
- Knee effusions common.
- Best lab test is ultrasonographylooking for
Bakers cyst and/or phlebitis of calf and thigh. - Best Rx is steroid injection into suprapatellar
bursa or medial knee jointknee hydraulics will
pump it into posterior knee.
61Case 45
- Pulmonary hypertension
- occurs in 30-40 of SSc on echo.
- severe PHT seen in 10.
- often as isolated PHT without significant ILD.
- Isolated PHT occurs predominantly in limited SSc
of long duration. - Low DLCO is the clue (often lt50-55 predicted).
Prominent Pulmonary Artery
62Case 46
- Synovial fluid essential for Rx of acute
inflammatory synovitis - Hemarthrosis
- Crystal-induced
- Septic arthritis
- Reactive arthritis
- Traumatic arthritis
- Overuse from traveling
- When INR in Rx range (2-3), it is safe to do
arthrocentesis.
63Case 47
- X-ray shows flattening of head and joint
incongruity later it will show OA. - 2-5 of alcoholics affected.
- Steroids other major riskespecially in SLE or
vasculitis. - If suspect AVN and plain x-ray normal, get
MRIbest technique for showing early AVN.
64Case 48
- In the NIH trials, the probability of avoiding
renal failure at 10-12 years in SLE DPGN was - 90 with cyclophosphamide treatment
- 60 with azathioprine (this was not significantly
different than cyclophosphamide - only 20 with prednisone
65CTX Rx of SLE DPGN
Short course 6 monthly infusions Long course 6
monthly infusions, followed by quarterly infusions
for 2 more years.
66Case 49
67Tip-offs to lumbar stenosis Body maneuvers which
extend the L/S spine narrow the canal (and
compress the nerve roots), while spine flexion
opens the canal).
- Increases symptoms
- Walking (pseudoclaudication)
- Walking down hill
- Leaning backwards
- Lying prone in bed
- Pulses should be intact and ankle-arm index ?
1.0
- Decreases symptoms
- Sitting still
- Bending forward while walking (shopping cart)
- Walking with cane
- Walking uphill
- Lying supine in bed
68Case 50
- Cheiroarthropathy
- Sclerodactyly
- No Raynauds
- Capillary loops normal
- ANA negative
- Flexion contractures of fingers give prayer
sign - Usually type I Diabetes
69Diabetes mellitus
- Other MSK complications of diabetes
- Dupuytrens
- Trigger fingers
- Adhesive capsulitis (frozen) of shoulder
70Case 51
- Enzyme profile in PM
- (with or without SLE)
- CPK 95
- Aldolase most cases
- ALT most cases
- AST most cases
- LDH most cases
71Case 52
- In RF patient with inflammatory polyarthritis
of the rheumatoid type (RA-like), check for Hep C
first. -
- If Hep C test is negative, can use MTX.
- Therapeutic progression (first year of RA)-- keep
adding therapies until RA controlled - NSAID and/or low dose prednisone.
- Add hydroxychloroquine and/or sulfasalazine.
- Add methotrexate (7.5 to 25 mg weekly) with folic
acid QD. - If max doses of MTX dont control, add anti-TNF-?
inhibitor or leflunomide.
72Case 53
-
- Scleroderma ?BP ? Creatinine
-
Renal Crisis
Until proven otherwise
73Case 53
74Case 54
- Two FDA-approved saliva stimulators
- Pilocarpine (Salagen) 5 mg qid
- Cevimeline (Evoxac) 30 mg tid
75Case 55
- Hypothyroidism
- Carpal tunnel syndrome
- Non-inflammatory synovial fluid
- Elevated CPKs
- Fibromyalgia
Numb areas in CTS
76Case 56
- Ig proliferative GN
- Proteinuria (gt500 mg/d)
- Often pedal edema
- RBCs and WBCs in RUA
- ANA
- Often anti-dsDNA Ab
- Normal-low C3, C4
- Creatinine nl or up
77Case 57
- Mild osteoarthritis
- Crepitus OA
- Good exercises
- Walking
- Isometric quad strengthening
- Consider acetaminophen also
Normal knee x-ray
78Case 58Lupus-like syndrome Drugs implicated
- Common
- Procainamide
- Hydralazine
- Uncommon
- Minocycline
- ?-blockers
- D-penicillamine
- Isoniazid
- Quinidine
- PTU
- Hydantoins
- Trimethodione
- Chlorpromazine
79Case 59
80Case 60
- Total protein albumin globulin
- (which in this case is elevated).
- Anemic
- High ESR, CRP, SAA, ?-glob,
- fibrinogen, haptoglobin, etc.
- Dx multiple myeloma
- ESR in female
D
Age 10 2
81Case 61
- On too much steroid!
- Hydroxychloroquine is good for SLE rash and
arthritis. - SLE arthritis is usually NON-erosive.
82Case 62
83PolychondritisEpisodic inflammation of hyaline
cartilage
- Ears and/or nose common
- Larynx and tracheal cartilage most
life-threatening - Non-erosive arthritis
- Eyes (scleritis)
- Aortic regurgitation
- Panniculitis of skin
84Case 63
- Antiphospholipid syndrome suggested by
- Blood clots
- 2 or more miscarriages or abortions
- 2 or 3 of these lab abnormalities for APS
- Anticardiolipin Abs (IgG or IgM)moderate to high
level - Lupus anticoagulant (DRVVT)
- ?2-glycoprotein
- Lupus anticoagulant cannot be assessed when the
patient is being treated with heparinwarfarin
yes.
85Case 64
- Chondrocalcinosis on x-ray suggests CPPD.
- OA-like changes of 2nd and 3rd MCP suggest CPPD.
- Diff dx of CPPD includes
- Hemochromatosis
- Hyperparathyroidism
- Acromegaly
- Diabetes mellitus
- Rx of 2 diagnoses changes lives
- Hemochomatosis (Fe, transferrin/ IBC, ferritin)
- Hyperparathryoidism (Ca, Ph, PTH)
86Case 65
87Case 65
- Reflex Sympathetic Dystrophy (RSD)
- Follows injury (sometimes minor)usually
extremity. - Constant dysesthesia, often disproportionate to
injury. - Vasomotor changes are frequent
- Early hyperemia and swelling of skin, exquisite
tenderness - Late cold, hyperhidrotic skin atrophy of skin
and muscle contractures osteopenia - Lab and path studies usually negative.
- Three-phase radioactive bone scan often positive.
88Case 66
Mitral valve vegetations
- APS often affects veins and arteries.
- TIAs and strokes in SLE are often emoblic from
valve vegetations.
89Case 67
- Differential dx of E.Nodosum
- TB, Fungal
- Strept, Yersinia
- Birth control pills, Sulfa
- Behcets, IBD
- Sarcoid
90Hilar adenopathy
91Case 68
- FMF characterized by two phenotypes
- Brief attacks of fever, recurring at irregular
intervals accompanied by peritonitis, arthritis
or pleuritis. - Insidious development of systemic AA amyloidosis
(nephropathy) and renal failure. - Chronic colchicine treatment prevents both
clinical attacks and amyloidosis.
92(No Transcript)
93Case 69
94Behcets Syndrome
- Recurrent oral ulcers (3x/yr)
- And 2 of the following
- Recurrent genital ulcers
- Eye lesions
- Skin lesions
- E. nodosum
- Pseudofolliculitis
- Papular pustular lesions
- Acneiform lesions
- Pathergy
95Case 70
- OA of knee
- Joint space narrowing often occurs first in the
medial compartment. - Quad strengthening first line of therapy.
- Acetaminophen first choice for drug therapy.