Rheumatology in Primary Care N. Buttar MD

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Rheumatology in Primary CareN. Buttar MD

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• Facts
• Several studies have shown the overuse of ANA
  and Rheumatoid factor testing and unnecessary
  referrals.
• Failure to use these tests in thoughtful and
  knowledgeable manner leads to diagnostic
  confusion and increased cost.

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• Facts
• Many serum rheumatological tests are
• available for fewer than 15 years
• Many physicians are not aware of
• Indicators
• Sensitivity
• Specificity
• Cost
• Clinical utility

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Definition of Terms

• Sensitivity
• Proportion of patients with a disease who have a
  positive test.
• Specificity
• Proportion of patients without disease who have
  a negative test result.

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Sensitivity and specificity

• They are valuable when very high greater than
  95
• A highly Sensitive test that is Negative tends to
  Rule Out the disease SnNOut
• A highly Specific test that is Positive tends to
  rule In the disease SpPIn

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Definition of Terms

• Test with high sensitivity rules out the disease
  and test with high specificity rules in the
  disease.

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• Definition of Terms
• Positive predictive value
• Positive test on a test with high positive
  predictive value indicates that the patient has a
  high probability of disease.
• Negative predictive Value
• High value means patients with negative test
  results most likely do not have the disease

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• Definition of Terms
• Sensitivity and specificity are independent of
  disease prevalence.
• But predictive value is markedly affected by
  disease prevalence.
• This emphasize the importance of limiting testing
  of patients with reasonable pretest possibility
  of disease.
• Example Predictive value of a positive test in
  patients with poly-arthralgia is likely to be
  higher in rheumatological practice than in
  general practice.

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Human cell
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Rheumatoid Factor

• IgM antibody against Fc (crystallizable fragment)
  portion of IgG.
• Others likely IgG and IgA can be tested
• Positive in a lot of rheumatological and
  non-rheumatological conditions
• Reported in titers

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Rheumatoid Factor

• Rheumatological Conditions
• Rheumatoid arthritis 50-90
• Sjogrens syndrome 75-95
• Cryoglobulinemia 40-100
• MCTD 50-60
• SLE 15-35
• Systemic sclerosis 20-30

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Rheumatoid Factor

• Non-rheumatologic conditions
• Aging
• Infections
• Syphilis, Bacterial endocarditis,
• Viral infections (MMR, Influenza),
• Parasitic diseases
• Pulmonary diseases
• Sarcoidosis, Pulmonary fibrosis
• Silicosis, Asbestoses
• Miscellaneous
• Primary biliary cirrhosis
• Malignancy especially leukemia and colon
  cancer.

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Rheumatoid Factor

• Prevalence of RF in healthy elderly could be 10
• But titers are low, 140 or lower
• 20 of patients with RA are RF negative
• 40 may have negative RF in early stages of
  disease
• Not helpful in low clinical suspicion (i.e.
  Absence of joint inflammation)

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Rheumatoid Factor

• Patients with Rheumatoid arthritis
• RF titer correlates with extra-articular
  manifestation and disease severity, but not
  disease activity.
• RF test may be prognostic value
• Not helpful in following disease progression

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Rheumatoid Factor

• Specificity - 80-95
• Sensitivity - 90 in Sjogrens syndrome and
  Cryoglobulinemia
• - 80 in RA -10 in
  polymyositis
• Serial measurement helpful in Sjogrens syndrome.
  
• Disappearance may indicate onset of Lymphoma.

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ANA

• Use of indirect immunofluorescence to detect
  antibodies to various nuclear antigens.
• Most labs employ HEp-2 cells (human epithelial
  cells)
• Reported in titers.
• Higher value more likely to represent true
  positive results (greater than 1320)
• Low titers like 120 or 140 rarely related to
  rheumatic conditions.
• Titers do not correlates with disease activity
• So serial measurements not helpful.

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ANA

• Rheumatic conditions
• Conditions Sensitivity
• Drug induced lupus 100
• SLE 99
• Scleroderma 97
• Sjogrens syndrome 96
• MCTD 93
• Polymyositis
• Dermatomyositis 78
• Systemic valculitis 15

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ANA

• Patterns of ANA
• Homogenous Specific for SLE
• Rim Specific for SLE
• Speckled Sjogrens syndrome,
  MCTD
• Diffuse Nonspecific
• Nucleolar Diffuse scleroderma
• Anti-centromere CREST syndrome highly
• With more specific auto antibodies tests
  available, these patterns are less helpful

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ANA

• Negative ANA does not R/O SLE completely.
• Rarely people with anti-Ro or anti-single
  stranded DNA (anti ssDNA) have negative ANA test.
• Patients with anti-phospholipid syndrome may have
  a negative ANA

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ANA

• Non-Rheumatic conditions
• HIV
• Bacterial endocarditis
• Viral infections
• Liver disease
• Type 1 DM

• Pulmonary fibrosis
• Multiple sclerosis
• Silicone gel implants
• Pregnancy
• Elderly

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Chromatin associated antibodies

• Anti-ssDNA anti-single stranded antibodies
• Anti-dsDNA (anti-double stranded DNA)
• Anti-histone

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Anti-double stranded DNA (Anti-dsDNA)

• High tiers are highly specific for SLE.
• Only 60 of SLE patients have high titers.
• Titer absence does not R/O SLE.
• Low titers can be present in
• -Normal population
• -Sjogrens syndrome
• -RA

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Anti-double stranded DNA (Anti-dsDNA)

• Anti ds DNA level correlates with disease
  activity in SLE
• Its presence correlates with lupus nephritis
• Testing not recommended with negative ANA

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Anti ssDNA antibodies

• They are non-specific and have little clinical
  utility

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Anti-histone antibodies

• Nonspecific
• Sensitive for drug induced lupus
• 50 of SLE have positive antibodies
• Common Drugs
• Procainamide
• INH

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Ribonucleoproteins

• Anti small nuclear ribonucleoprotiens
• Anti-Ro and anti-LA
• Anti-Ribosome

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Anti small nuclear ribonucleoprotiens

• 1 Anti-Smith antibodies (anti-Sm antibodies)
• Specific for SLE but present in only 20-30

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Anti small nuclear ribonucleoprotiens

• 2. Anti-U1 snRNP
• Associated with 100 of cases of MCTD
• Present in 30-40 of SLE patients
• In SLE associated with
• -Disease activity
• -Myositis
• -Esophageal hypomotility
• -Sclerodactyly
• -Raynauds phenomenon
• -Arthritis

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Anti-Ro (anti ss-A)

• Sjogrens syndrome 70
• SLE 40
• Associated with SLE with
• - Photosensitive skin rash
• - Pulmonary disease
• - Lymphopenia

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Anti LA (Anti ss-B)

• Sjogrens syndrome 40
• SLE 10-15
• Associated with
• - Late onset of SLE
• - Secondary Sjogrens syndrome
• - Neonatal lupus syndrome

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Anti-Ro and Anti-LA

• Can help to confirm diagnosis of Sjogren's
  syndrome.
• Related to extra glandular manifestations of
  disease.
• Helpful in suspected SLE and positive ANA.

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Anti-Ribosome antibodies

• Highly specific for SLE.
• But present only in 10-20 of cases.
• Associated with lupus psychosis.

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Scleroderma antibodies

• Anti-centromere
• Anti-topoisomerase 1

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Anti-centromere

• 20-40 of patients with scleroderma
• Presence associated with Raynauds phenomenon,
  CREST syndrome, limited skin involvement
• Also present with primary biliary cirrhosis

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Anti-topoisomerase 1 (Anti Scl-70)

• Highly specific
• 20-40 of patients with scleroderma
• Its presence correlated with
• - Diffuse cutaneous disease
• - Pulmonary fibrosis
• - Cardiac involvement
• - Longer disease duration

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Other Antibody Test

• Anti-Jo1 histidyl-tRNA synthetase
• 30 patients of polymyositis and dermatomyositis
• Associated with
• - Pulmonary fibrosis
• - Raynaulds phenomenon

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Anti-Neutrophilic Cytoplasmic
Antibody (ANCAs)

• I. cANCA - cytoplasm
• 2. pANCA perinuclear
• cANCA Antibody to enzyme proteinase 3 and highly
  sensitive and specific for Wegener's
  granulomatosis.
• pANCA Antibodies against myeloperoxidase
• - Associated with polyangiitis and necrotizing
  glomerulonephritis
• - Has less sensitivity and specificity
• - Present in several other rheumatic
  diseases

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Human Leukocyte Antigen B27
(HLA-B27)

• Associated with spondyloarthropathies especially
  in ankylosing spondylitis
• 95 sensitive for AS
• 80 for Reiters syndrome
• 70 for spondylitis with psoriasis
• Only present in 6-10 Caucasian population, order
  only if suspicion is high.

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Complement deficiency

• Component Disease
• Classical pathway
• C1q,C1r,C1s,C4 Immune complex
  syndrome
• C2
  Pyogenic infections
• C1 inhibitor
• C3 and Alternative pathways C3
• C3
  Immune complex syndrome
• 
  Pyogenic infections
• D
  Pyogenic infections
• Properdin Neisseria
  infections
• I
  Pyogenic infections
• H HUS

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Complement deficiency

• Component
  Disease
• Membrane attach complex
• C5, C6, C7, C8 Recurrent
  Neisseria infections
• 
  Immune-complex diseases
• C9 Rare
  Neisseria infections

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Acute phase reactants

• Heterogeneous group of proteins
• Synthesized in liver
• Rapidly induced in inflammation and tissue
  necrosis.

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Acute phase reactants

• Coagulation proteins
• - Fibrinogen
• - Prothrombin
• Transport proteins
• - Hepatoglobins
• - Transferrin
• - Ceruloplasmin

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Acute phase reactants

• Complement components
• - C3 and C4
• - Protease inhibitor
• Miscellaneous
• - Albumin
• - Fibronectin
• - CRP
• - ESR
• - Serum amyloid-A related
  proteins

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Acute phase reactants

• Most commonly used
• CRP and ESR
• CRP responds rapidly than ESR
• However ESR takes an hour and CRP takes a day.

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Acute phase reactants

• ESR
• Measures ht of RBCs that settle in one hour.
• Upper limits
• Less than 50 yrs.
• Men lt 15 mm
• Women lt 20 mm
• More than 50 yrs.
• Men lt20
• Women lt30
• Male- age/2 and Women age10 /2

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ESR

• Diagnostic criteria for
• - Temporal arteritis
• - Polymyalgia rheumatica
• Tends to correlates with clinical activity of
  disease
• Used as a mean to stage RA.
• Sensitivity 50 in RA but specificity is very low.

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ESR

• Factors increasing ESR
• -Old age
• - Female
• - pregnancy
• - Anemia
• - Macrocytosis
• - Elevated fibrinogen levels
• Infections, Inflammation,
  Malignancy
• - Technical factors
• Dilution, temp., tilted
  tube

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ESR

• Decreased ESR
• - Polycythemia
• - Leukocytosis
• - Sickle cell disease
• - Protein abnormalities
• 
  Hypofibrinogenimia
• 
  Hypogammaglobulinemia
• Dysproteinemia
• - Microcytosis,Spherocytosis,Aniso
  cytosis

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Auntoantibodies Detected in Patients with
Connective Tissue Disease
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• RHEUMATIC DISEASE (SUSPECTED)

HP Exam
Laboratory Evaluation Inducing ANA
Positive ANA
Negative ANA
Further Laboratory Evaluation
Antibody to dsDNA
Antibody to Exstractable nuclear antigens
Antibody to chromosome centromere
Antibody to Histone proteins
Anti-Sm
Anti-RNP
Anti-Ro/LA
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TO SUM UP

• Initial labs
• CBC
• ANA
• CRP
• ESR
• RF

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Further testing.

• 1 Chromatin Antibodies 3
  Scleroderma Antibodies
• -Anti ds-DNA -
  Anti-centromere
• - Anti-histone
  - Anti-topoisomerase
• 2 Ribonucleoproteins antibodies 4 Others
• - Anti-Sm
  - Anti-JO
• - Anti-U1 snRNP -
  cANCA
• - Anti-Ro
  - pANCA
• - Anti-La
  - HLA B27
• - Anti-ribosome -
  CH50

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Which one of the following is the best treatment
for a patient who has uncomplicated Sjogrens
syndrome and complains of dry eyes and dry mouth?

• Oral lubricants, artificial tears and instruction
  in oral hygiene techniques to prevent caries
  formation.
• Continuous low-dose corticosteroids to prevent
  further progressive gland destruction and
  dryness.
• High-dose steroids on alternate days for six
  months.
• Plasmaphoresis to remove serum immune complexes
  that contribute to glandular diseases.

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Answer Oral lubricants,
artificial tears, and instruction in oral
hygiene techniques to prevent caries formation.
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Sjogrens syndrome is a condition characterized
by dry eyes, dry mouth, and arthritis. Simple
measures (oral lubricants, artificial tears, and
instruction in oral hygiene) are appropriate in
the absence of complications. The risks of
corticosteroid therapy in any form for long
periods of time preclude its use for dry eyes and
dry mouth alone. Plasmaphoresis has no role in
management of uncomplicated Sjogrens syndrome.
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A 24 -year-old white female complains of a three
monthhistory of joint pains, rash, and fatigue.
Which one of the following test results provides
the greatest support for a diagnosis of
connective tissue disease?

• WBC count 3600/mm3
• Erythrocyte sedimentation rate 75 mm/hr
• Antinuclear antibody (AN positive, titer 180)
• Antinuclear antibody (AN positive, titer 1320)
• Positive rheumatoid factor

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Right Answer Antinuclear antibody (AN positive,
titer 1320)

• A low WBC count, high sedimentation rate, and
  positive rheumatoid factor each suggest the
  diagnosis of connective tissue disease, but are
  nonspecific. The ANA (antinuclear antibodies)
  titer is the diagnostic marker for connective
  tissue disease, but a titer less than 1160 is
  common in healthy older persons and in other
  medical conditions. A titer greater than 1160
  suggests a high likelihood of connective tissue
  disease.

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Which one of the following statements is most
accurate concerning juvenile rheumatoid
arthritis?

• Most patients require corticosteroid treatment
• The disease is characterized by lifelong
  recurrences
• Most patients have a permanent deformity of at
  least one extremity.
• Ten years after the onset of disease, most
  patients have excellent functional status.
• Fever is a rare systemic manifestation.

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Right Answer Ten years after the onset of
disease, most patients have excellent functional
status

• At least 50 of patients followed for up to
  15 years have complete remission of juvenile
  rheumatoid arthritis, and 70 regain normal
  function. A few patients are left with crippling
  joint deformities, but 75 have no significant
  residual deformity. Systemic-onset disease is
  accompanied by high fever, rheumatoid rash,
  polyarthritis, and other systemic manifestations.
  

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A 24 -year-old white male comes to your office
with hemoptysis. A chest film shows bilateral
patchy consolidation. He has iron deficiency
anemia, proteinuria, and hematuria. This
presentation is consistent with?

• Polyarteritis nodosa
• Good pasture's syndrome
• Sarcoidosis
• Wagener's granulomatosis

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• Right Answer Good pasture's syndrome
• Good pasture's syndrome is characterized by
• pulmonary hemorrhage, glomerulonephritis,
• and antiglomerular basement membrane
• antibodies. Hemoptysis, pulmonary alveolar
• infiltrates, dyspnea and iron deficiency
• anemia are frequent presenting features. Within
  days
• or weeks, the pulmonary findings are generally
• followed by hematuria, proteinuria, and rapid
  loss of
• renal function.
• Polyarthritis nodosa may involve the lung.
  Although
• pneumonic episodes may be associated with
  hemoptysis
• in a small percentage of patients, the chest film
  is more likely
• to reveal granulomatosus lesions rather than
  patchy
• infiltrates. Continued.

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• Hemoptysis does not generally occur until
• late in the course of sarcoidosis, and is
• usually related to Aspergillus infection or
• cavitation. Renal involvement rarely results
• in significant proteinuria or hematuria.
• With Wagener's granulomatosis, upper respiratory
  tract
• symptoms and signs are usually prominent. These
  include
• purulent or bloody rhinorrhea, Para nasal sinus
  pain and
• drainage, nasal mucosal ulceration, and otitis
  media with
• hearing loss. Cough, purulent sputum production,
  
• hemoptysis, and pleuretic chest pain are usually
  associated
• with upper respiratory symptoms and occasionally
  precede
• them. Renal findings occur at some time in the
  course of
• most diseases but rarely are the presenting
  feature.

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A 49 -year-old white female comes to your office
complaining of painful, cold fingers tips, which
turn white when she is hanging out her laundry.
While there is no approved treatment for this
condition at this time, which of the following
drugs has been shown to be useful?

• Propanolol (Inderal)
• Nifedipine (Procardia)
• Atenolol (Tenormin)
• Methysergide (Sansert)

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Right Answer Nifidipine (Procardia)

• At present there is no approved treatment for
  Raynauds
• disease. However, patients with this disorder
  reportedly
• experience subjective symptomatic improvement
  with
• calcium channel antagonists. Nifedipine is the
  calcium
• channel blocker of choice in patients with
  Raynauds
• disease. Beta blockers can produce arterial
  insufficiency of
• the Raynauds type, so propanolol and atenolol
  would be
• be contraindicated. Drugs such as ergotamine
• preparations and methysergide can produce cold
• sensitivity, and should, therefore, be avoided in
  patients
• with Raynauds disease.

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For several years, a hypertensive 65-year-old
female has been treated with chlorthoalidone
(Hygroton), 50 mg qd atenolol (Tenormin), 100 mg
qd and hydralazine (Apresoline) 50 mg b.i.d. Her
BP has been well-controlled on this regimen.
Over the past two months, she ahs experienced
malaise, along with diffuse joint pains that
involve symmetric sites in the fingers, hands,
elbows, and knees. A pleural friction rub is
noted on examination. Laboratory testing shows
that the patient has mild anemia and leukopenia,
with a negative rheumatoid factor and positive
ANA titer of 1640. Which one of the following
would be the most appropriate INITIAL treatment?

• Discontinue the thiazide diuretic and switch to a
  loop diuretic such as furosemide (Lasix)
• Discontinue the hydralazine.
• Begin Tx with Prednisone 40 mg po qd
• Treat with hydroxychloroquine (Plaquenil) 400 mg
  qd.
• Obtain renal function studies and anticipate that
  renal Bx will be needed.

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Right Answer Discontinue the hydralazine.

• There are many drugs that can induce a syndrome
  resembling SLE, but the most common offender is
  procainamide, followed by hydralazine. There is
  a genetic predisposition for this drug-induced
  lupus, determined by drug acetylation rates.
  Polyarthritis and pleuralpericarditis occur in
  half of patients, but fortunately, CNS and renal
  involvement are rare. While all patients with
  this condition have positive ANAs and most have
  antibodies to histones, antibodies to
  double-stranded DNA and decreased complement
  levels are rare, which distinguishes drug-induced
  lupus from idiopathic lupus.
• The best initial management is to withdraw the
  drug, and most patients improve in a few weeks.
  For those with severe symptoms, a short course of
  steroids is indicated. Once the offending drug
  is discontinued, symptoms seldom last beyond six
  months.

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Raynauds phenomenon is most frequently
associated with which of the following connective
tissue diseases?

• Progressive systemic sclerosis
• Systemic lupus erythematosus
• Polymyositis
• Rheumatoid arthritis
• Polyarteritis

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Right Answer Progressive systemic sclerosis.

• Raynauds phenomenon is most commonly
• associated with progressive systemic sclerosis,
• with a frequency of approximately 90. The other
  
• connective tissue diseases are likewise
  associated
• with Raynauds phenomenon, but much less
• frequently SLE 20-30 of cases, polymyositis 30
  
• of cases, rheumatoid arthritis less than 10 of
• cases, and polyerteritis rarely.

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Which of the following findings is most
specifically diagnostic of classical rheumatoid
arthritis?

• Elevated sedimentation rate
• Joint swelling
• Malar facial rash
• Rheumatoid joint erosions
• Rheumatoid factor

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Right Answer Rheumatoid joint erosions

• Elevated sedimentation rate, joint swelling
• and rheumatoid factor are all associated
• with rheumatoid arthritis (RA). Malar facial
• rash is not associated with RA. Rheumatoid
• joint erosions are seen ONLY in classic RA.

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Which one of the following is true regarding the
treatment of temporal arteritis?

• Most patients require consultation with
  rheumatologist for arthrocentesis of involved
  joints
• Patients should expect to take medication for 1-2
  years
• Ischemic optic neuritis will not develop if
  treatment is initiated early
• Vascular disease does not occur outside the
  central nervous system

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Right Answer Patients should expect to take
medication for 1-2 years.

• When treated early with prednisone, temporal
• arteritis has an excellent prognosis. Treatment
  should
• continue with low doses of prednisone for 1-2
  years to
• prevent relapse. Joint involvement with effusion
  does not
• occur and there is no need for arthrocentesis.
  Most
• patients achieve complete recovery that persists
  even after
• medications are withdrawn.
• Ischemic optic neuritis is less likely to occur
  while the
• patient is receiving treatment. Claudication,
  MI, aortic
• aneurysm, and visceral infarction have all been
  reported as
• well as strokes.

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The CREST syndrome (calcinosis of the skin,
Raynauds phenomenon, esophageal dysmotility,
sclerodactyly, and telangiectasis) is a subject
of?

• Rheumatoid arthritis
• Systemic sclerosis
• Systemic lupus erythematosus
• Dermatomyositis
• Eosinophilia myalgia syndrome

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Right Answer systemic sclerosis

• A subset of patients with systemic sclerosis
• have CREST syndrome (calcinosis of the skin,
• Raynauds phenomenon, esophageal dysmotility,
• sclerodactyly, and telangiectasis). Patients
  with CREST
• syndrome often possess an anticentromere
  antibody. They
• will also have a higher incidence of pulmonary
• hypertension. The prognosis of patients with
  CREST
• syndrome is better than patients with progressive
  systemic
• sclerosis, but slow progression eventually
  occurs.

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Which one of the following statements regarding
rheumatoid arthritis are true?

• It is caused by the organism Borrelia burgdorferi
• Oral minocycline (Minocin) has been shown to have
  some benefit in treating mild to moderate cases
• Metronidazole (Flagyl) is useful for rheumatoid
  arthritis caused by anaerobic organisms
• Amoxicillin has been shown to be of significant
  benefit in treating rheumatoid arthritis
  involving the large, weight-bearing joints
• Erythromycin can be used to treat the
  pericarditis seen with rheumatoid arthritis.

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Right Answer Oral minocycline (Minocin) has
been shown to have some benefit in treating mild
to moderate cases.

• Minocycline, along with tetracycline, has been
  shown to have some benefit in treating rheumatoid
  arthritis. The exact mechanism for this
  improvement is unclear, but is hypothesized to be
  the result of anti-inflammatory effect rather
  than the drugs anti-infectious properties.

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A 67 -year-old white female comes to your office
complaining of a one month history of fatigue,
weight loss, low grade temperature elevation, and
aching and stiffness in the upper back and
shoulders. Physical examination confirms
weakness in both shoulders with the an otherwise
normal musculoskeletal examination. Her
temperature is 37.7C (99.9F). Which one of the
following would be the appropriate diagnostic
study?

• Radiographs of the spine and shoulders
• Erythrocyte sedimentation rate
• Serologic titers for cytomegalovirus
• Electromyographic studies of the shoulder girdle
  muscles
• Febrile agglutinins

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Right Answer Erythrocyte sedimentation rate

• The symptoms described in this case are most
• consistent with a diagnosis of polymyalgia
  rheumatica, a
• subacute rheumatologic disorder affecting
  primarily the
• elderly. The erythrocyte sedimentation rate is
  the best
• diagnostic study and in most cases confirms the
  diagnosis
• The disease does not cause radiographic changes.
  
• Cytomegalovirus infection would not be part of
  this
• differential. Electromyhography would be
  unlikely to show
• changes in the absence of muscle weakness, and
  febrile
• agglutinins exhibit no relationship to this
  disease.

84
Drug-induced lupus is usually associated with

• The absence of antinuclear antibodies
• The absence of antibodies to double-stranded DNA
• Glomerulonephritis
• Central nervous system involvement
• A clinical course longer than six months

85

• Right Answer The absence of antibodies to
  double-stranded DNA.
• Several drugs can cause syndrome resembling
  SLE in persons without any obvious predisposition
  to the disease. The most common drug implicated
  is procanamide, which produces antinuclear
  antibodies in about 50 of individuals half of
  these develop a lupus-like syndrome. The
  clinical syndrome consists of polyarthralgias and
  systemic symptoms in most patients. Other
  manifestations typical of idiopathic SLE are
  unusual, including nephritis and CNS involvement.
  All patients with drug-induced lupus are ANA
  most have antibodies to histones. Antibodies to
  double-stranded DNA (dsDNA) and
  hypocomplementemia are rare, a helpful point in
  distinguishing drug-induced lupus from idiopathic
  lupus. Anemia, lukopenia, lupus anticoagulant,
  thrombocytopenia, cryoglobulins, rheumatoid
  factor, a false-positive VDRL, and a positive
  direct Coombs test may be seen.
• continued..

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• The initial therapeutic approach should be to
  discontinue the suspected drug most patients
  improve in days or a few weeks. In patients with
  severe symptoms, a short course (2-10 weeks) of
  glucocorticoids is indicated. Clinical symptoms
  rarely persist more than six moths ANA may
  remain positive for years. Most lupus-inducing
  drugs can be used safely in patients with
  idiopathic lupus if there are no suitable
  alternatives.

87
The CREST syndrome (calcinosis of the skin,
Raynauds phenomenon, esophageal dysmotility,
sclerodactyly, and telangiectasis) is a subject
of?

• Rheumatoid arthritis
• Systemic sclerosis
• Systemic lupus erythematosus
• Dermatomyositis
• Eosinophilia myalgia syndrome

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Right Answer Systemic sclerosis

• A subset of patients with systemic sclerosis
• have CREST syndrome. Patients with CREST
• syndrome often possess an anticentromere
• antibody. They may also have a higher incidence
• of pulmonary hypertension. The prognosis in
• patients with CREST syndrome is better than for
• patients with progressive systemic sclerosis, but
  
• slow progression eventually occurs.

89
A 70 -year-old white male presents to your office
complaining that he just cant move. He
reports that the activities of living take
increasing amounts of time. He appears
depressed, his speech is quiet and emotionless,
and he stares without blinking. The most likely
diagnosis is

• Polymyalgia rheumatica
• Alzheimers disease
• Wilsons disease
• Parkinsons disease

90
A 10-year-old boy who has otitis media is treated
with oral penicillin for ten days. Ten days
later, the child develops a fever, urticaria,
multiple joint arthralgia, and prostration. He
denies ear pain. The most likely diagnosis is

• Recurrent otitis media
• Serum sickness syndrome
• Meningitis
• Osteomyelitis

91
A 38 -year-old African-American female comes to
your office complaining of joint pains. The
metacarpophalangeal joints of both hands are red
and swollen. Fluid aspirated from an involved
joint shows 5000 WBC/mm3 which are predominately
neutrophils. Radiographs of the hands show
juxta-articular osteoporosis of the MCP joints.

• Osteoarthritis
• Rheumatoid arthritis
• Gout
• Anklyosing spondylitis
• Gonorrhea-associated arthritis

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An 8 -year-old white male is brought to your
office by his parents, who are concerned about
the number of colds he has had. Your chart review
indicates that he has had approximately 15 URIs
each year. Most were viral, but he has also had
several episodes of sinusitis. Family history
reveals that the mother has similar problems and
has recently been diagnosed with rheumatoid
arthritis. The most likely immune deficiency in
this child is?

• Brutons panhypogammaglobulinemia
• DiGeorges syndrome
• Wiskott-Aldrich syndrome
• Combined immunodeficiency selective IgA
  deficiency