RHEUMA Flashcards

1
Q

Polarized light microscopy can identify most typical crystals except for

A

Apatite

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2
Q

Gpout is caused by deposition of what crystals

A

MSU crystals

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3
Q

The most common early clinical manifestation of gout

A

Acute arthritis

Only one joint is affected initially

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4
Q

Inflamed nodes which may be a first manifestation of gouty arthritis (2)

A

Heberden’s or Bouchard’s nodes

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5
Q

Women represent only ____ of all patients with gout

A

5–20%

Mostly postmenopausal and elderly

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6
Q

Often demonstrated in the first metatarsophalangeal joint and in knees not acutely involved with gout during acute gouty attacks

A

Needle-shaped MSU crystals

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7
Q

Thick pasty or chalky joint fluid in gout/tophi is due to

A

large number of crystals

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8
Q

In 24-h urine uric acid, this value of of uric acid per 24 h on a regular diet suggests that causes of overproduction of purine should be considered

A

> 800 mg

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9
Q

Ultrasound of gout show what sign?

A

double contour sign overlying the articular cartilage

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10
Q

2 regimens of colchine in gout

A
  • One 0.6-mg tablet given every 8 h with subsequent tapering

* 1.2 mg followed by 0.6 mg in 1 h with subsequent day dosing depending on response

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11
Q

Mainstays of treatment of acute gout (3)

A
  • NSAIDs
  • Colchicine
  • Glucocorticoids
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12
Q

Intraarticular glucocorticosteroids used in acute gout (2)

A
  • Triamcinolone acetonide (20–40 mg)

* Methylprednisolone (25–50 mg)

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13
Q

To prevent recurrent gouty attacks and eliminate tophaceous deposits, we should normalized SUA to

A

<300–360 μmol/L (5.0–6.0 mg/dL)

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14
Q

Factors affecting decision to initiate hypouricemic therapy in gout (4)

A
  • Number of acute attacks (urate lowering may be cost-effective after two attacks)
  • Serum uric acid levels (progression is more rapid in patients with serum uric acid >535 μmol/L [>9.0 mg/dL])
  • Patient’s willingness to commit to lifelong therapy
  • Presence of uric acid stones
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15
Q

Uricosuric agents for patients with good renal function who underexcrete uric acid (<600 mg in a 24-h urine sample)

A

Probenecid

Dose: 250 mg twice daily and increased gradually as needed up to 3 g per day to achieve and maintain a serum uric acid level of <6 mg/dL

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16
Q

In patiets taking probenecid, urine volume should be maintained by ingestion of _____ of water every day

A

1500 mL

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17
Q

Nonspecific xanthine oxidase inhibitor used in gout

A

Allopurinol

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18
Q

Specific xanthine oxidase inhibitor used in gout

A

Febuxostat

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19
Q

Most commonly used hypouricemic agent

A

Allopurinol

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20
Q

Best drug to lower serum urate in overproducers, urate stone formers, and patients with renal disease

A

Allopurinol

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21
Q

Dose of allopurinol in gout

A

100 mg initially and increasing up to 800 mg if needed

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22
Q

Allopurinol toxicity is increased in patients with (2)

A
  1. Intake of thiazide

2. Allergy to penicillin and ampicillin

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23
Q

A pegylated uricase for gout patients who do not tolerate or fail full doses of other treatments

A

Pegloticase

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24
Q

Colchicine anti-inflammatory prophylaxis in doses of 0.6 mg one to two
times daily should be given along with the hypouricemic therapy until (3):

A
  • Patient is normouricemic
  • Without gouty attacks for 6 months
  • As long as tophi are present

Colchicine should NOT be used in dialysis patients

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25
Q

Calcium pyrophosphate deposition disease is most common in what group of patients

A

Elderly

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26
Q

Mutations in the ANKH gene will cause

A

Calcium pyrophosphate deposition disease

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27
Q

A minority of patients with CPPD arthropathy have hereditary CPP disease or these metabolic abnormalities (4)

A
  • Hyperparathyroidism
  • Hemochromatosis
  • Hypophosphatasia
  • Hypomagnesemia
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28
Q

Originally was termed pseudogout

A

Acute calcium pyrophosphate deposition disease arthritis

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29
Q

The joint most frequently affected in CPPD arthropathy

A

Knee

Other sites:
•	Wrist
•	Shoulder
•	Ankle
•	Elbow
•	Hands
•	Temporomandibular joint
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30
Q

Punctate and/or linear radiodense deposits within fibrocartilaginous joint menisci or articular hyaline cartilage seen in radiograph or ultrasound.

A

Chondrocalcinosis

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31
Q

Chondrocalcinosis is presumptive of

A

CPPD

DDx: CaOx in some patients with chronic renal failure

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32
Q

Demonstration of typical rhomboid or rodlike crystals (generally weakly positively birefringent or nonbirefringent with polarized light) in synovial fluid or articular tissue is diagnostic of

A

CPPD

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33
Q

Acute attacks of CPPD arthritis may be precipitated by (3)

A
  1. Trauma
  2. Severe medical illness
  3. Surgery
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34
Q

Acute attacks of CPPD arthritis may be precipitated by surgery most especially,

A

Parathyroidectomy

causes rapid diminution of serum calcium concentration

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35
Q

Aka basic calcium phosphate disease

A

Calcium apatite deposition disease

Aka apatite arthropathy

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36
Q

the primary mineral of normal bone and teeth

A

Apatite

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37
Q

In CKD, this contribute to extensive apatite deposition both in and around joints

A

Hyperphosphatemia

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38
Q

Apatite aggregates are commonly present in synovial fluid in an extremely destructive chronic arthropathy of the elderly that occurs most often in the

A

shoulders (Milwaukee shoulder)

may also occur in hips, knees, and erosive osteoarthritis of fingers

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39
Q

30–50% of patients with ________ have apatite microcrystals in their synovial fluid

A

Osteoarthritis

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40
Q

Acute attacks of bursitis, tendinitis or synovitis is seen in what joint disease

A

Calcium apatite deposition disease

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41
Q

A rare hereditary metabolic disorder that may lead to nephrocalcinosis and renal failure

A

Primary oxalosis

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42
Q

Common cause of secondary oxalosis

A

Ascorbic acid supplements

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43
Q

Bipyramidal crystals with strong birefringence and may stain with alizarin red S

A

CaOx crystals

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44
Q

____ of SLE cases occur in women of child-bearing age

A

90%

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45
Q

Genetic “signature” in peripheral blood cells of 50-80% of SLE patients

A

Upregulation of genes induced by IFNs

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46
Q

The most characteristic gene expression pattern of SLE patients

A

Influence the IFN production

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47
Q

Female sex is permissive for SLE because of (3)

A
  1. Hormone effects
  2. Genes on the X chromosomes
  3. Epigenetic differences between genders
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48
Q

SLE is associated with prolonged occupational exposure to

A

crystalline silica

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49
Q

Social behavior or lifestyle that reduces risk of SLE

A

Alcohol (2 glasses of wine a week or ½ of an alcoholic drink daily)

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50
Q

Biopsy of SLE shows (3)

A
  1. Deposition of Ig at the dermal-epidermal junction (DEJ)
  2. Injury to basal keratinocytes
  3. Inflammation dominated by T lymphocytes in the DEJ and around the blood vessels and dermal appendages

The abovementioned patterns are NOT specific for dermatologic SLE, but they are highly suggestive

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51
Q

Class of lupus nephritis that requires aggressive immunosuppression (3)

A
  1. Class III
  2. Class IV
  3. Class V, accompanied by III and IV
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52
Q

Class of lupus nephritis that does not require aggressive immunosuppression (3)

A
  1. Class I
  2. Class II
  3. With extensive irreversible changes
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53
Q

Most common pattern of vasculitis in SLE

A

Leukocytoclastic vasculitis

54
Q

Antibodies that are specific for SLE (2)

A

High-titer Anti-dsDNA

Anti-Sm

55
Q

T or F. Permanent complete remission is rare in SLE

A

True

Permanent complete remissions – absence of symptoms with no treatment

56
Q

Systemic symptom that is present most of the time in SLE (2)

A
  1. Fatigue
  2. Myalgias/arthralgias

Symptoms associated with severe systemic illness:

  1. Fever
  2. Prostration
  3. Weight loss
  4. Anemia
  5. Other organ-targeted manifestations
57
Q

Musculoskeletal manifestation that is present in most SLE patients

A

Intermittent polyarthritis

58
Q

Rheumatoid-like arthritis with erosion that fulfill criteria for both RA and SLE

A

Rhupus

59
Q

Most common reason that patients increase their dose of glucocorticoids in SLE

A

Joint pain

60
Q

Pain persists in a single joint, such as knee, shoulder, or hip with no other manifestations of active SLE

A

Ischemic necrosis of bone

61
Q

Most common chronic dermatitis in lupus

A

Discoid lupus erythematosus (DLE)

62
Q

Roughly circular with slightly raised, scaly hyperpigmented erythematous rims and depigmented, atrophic centers in which all dermal appendages are permanently destroyed

A

Discoid lupus erythematosus (DLE)

63
Q

Treatment of discoid lupus erythematosus (DLE) (2)

A
  1. Topical or locally injected glucocorticoids

2. Systemic antimalarials

64
Q

Consist of scaly red patches similar to psoriasis that is exquisitely photosensitive

A

Subacute cutaneous lupus erythematosus (SCLE)

65
Q

Antibodies that is usually present in subacute cutaneous lupus erythematosus (SCLE)

A

anti-Ro (SS-A)

66
Q

Usually the most serious manifestation of SLE

A

Nephritis

67
Q

Test that is recommended for every SLE patient with any clinical evidence of nephritis

A

Renal biopsy

68
Q

Cardiovascular event that is seen in most people with lupus nephritis after several years of the disease

A

Accelerated atherosclerosis

69
Q

Most common manifestation of diffuse CNS lupus

A

Cognitive dysfunction

70
Q

Neurologic manifestation that can be the dominant manifestation of SLE but must be distinguished from glucocorticoid-induced forms

A

Psychosis

71
Q

MI in SLE is primarily a manifestation of

A

Accelerated atherosclerosis

Increased risk for vascular events is 3- to 10-fold

72
Q

Most common pulmonary manifestation of SLE

A

Pleuritis with or without pleural effusion

73
Q

Life threatening pulmonary manifestations of SLE (4)

A
  1. Fibrosis
  2. Shrinking lung syndrome
  3. Intraalveolar hemorrhage
  4. Pulmonary hypertension

Other pulmonary manifestations:

  1. Pleuritis with or without pleural effusion
  2. Pulmonary infiltrates
74
Q

Most frequent cardiac manifestation of SLE

A

Pericarditis

75
Q

Cardiac manifestations of SLE (4)

A
  1. Pericarditis
  2. Myocarditis
  3. Libman-Sacks endocarditis
  4. Myocardial infarction
76
Q

Most frequent hematologic manifestation

A

Anemia

Usually normochromic, normocytic – reflect chronic illness

77
Q

T or F. Leukopenia in SLE is usually granulocytopenia

A

False. Almost always lymphopenia, not granulocytopenia

78
Q

Recurring or prolonged hemolytic anemia or thrombocytopenia, or disease requiring an unacceptably high dose of daily glucocorticoids in SLE, should be treated with additional strategies (3)

A
  1. Rituximab
  2. Platelet growth factors
  3. Splenectomy
79
Q

Serious ocular manifestations of SLE (2)

A

Retinal vasculitis and optic neuritis
Blindness can develop over days to weeks

Sicca syndrome (Sjogren’s syndrome) and nonspecific conjunctivitis – common in SLE but rarely threaten vision

80
Q

The most important autoantibodies to detect in SLE

A

ANA

Positive in >95% of patients, usually at the onset of symptoms

81
Q

Antibodies is not specific for SLE but presence fulfills one classification criterion and identify patients at increased risk for venous or arterial clotting, thrombocytopenia, and fetal loss

A

Antiphospholipid antibodies

82
Q

3 widely accepted tests for antiphospholipid antibodies

A

Anticardiolipin
Anti-β2-glycoprotein
Lupus anticoagulant

83
Q

APS criteria (2)

A
  1. One or more clotting episodes and/or repeated fetal losses
  2. At least 2 positive tests for antiphospholipid antibodies, at least 12 weeks apart
84
Q

Women with child-bearing potential and SLE should be screened for these 2 antibodies because both antibodies have the potential to cause fetal harm

A

antiphospholipid antibodies

anti-Ro

85
Q

Goal in the management of SLE

A

low-level disease activity (mild symptoms on the lowest possible doses of medications)

86
Q

Low-level disease activity can be achieved for at least a year in ____ of SLE patients

A

30-50%

87
Q

Mainstays in the treatment of non-life threatening SLE

A
  1. Analgesics (NSAIDs and Acetaminophen)

2. Antimalarials

88
Q

Antimalarials used in SLE (3)

A
  1. Hydroxychloroquine
  2. Chloroquine
  3. Quinacrine
89
Q

ADR of hydroxychloroquine

A

Retinal toxicity

in 6% of patients after cumulative doses of 1000 g, ~5 years of continuing therapy

90
Q

Mainstay treatment of life-threatening SLE

A

systemic glucocorticoids

0.5-1 mg/kg per day PO or 500-1000 mg of methylprednisolone sodium succinate IV daily for 3 days followed by 0.5-1 mg/kg of daily prednisone or equivalent

91
Q

An alkylating agent that is an acceptable choice for induction of improvement in severely ill patients

A

Cyclophosphamide

92
Q

2 regimens of cyclophosphamide in SLE

A
  1. Low-dose cyclophosphamide (500 mg every 2 weeks for total of 6 doses) followed by azathioprine or mycophenolate mofetil maintenance
  2. High-dose cyclophosphamide (500-1000 mg/m2 BSA given monthly IV for 6 months) followed by azathioprine or mycophenolate maintenance
93
Q

Common adverse effect of high-dose cyclophosphamide regimen in women SLE patients

A

ovarian failure

Treated with a gonadotropin-releasing hormone agonist (e.g. leuprolide 3.75 mg IM) prior to each monthly cyclophosphamide dose

94
Q

A relatively lymphocyte-specific inhibitor of inosine monophosphatase and therefore of purine synthesis

A

Mycophenolate mofetil

Acceptable choice for induction of improvement in severely ill SLE patients

95
Q

A purine analog and cycle-specific antimetabolite that is used in treatment of SLE

A

Azathioprine

May be effective but is associated with more flares

96
Q

Management of ISN grade III or IV lupus nephritis that reduces progression to ESRD

A

glucocorticoids + cyclophosphamide

97
Q

A folinic acid antagonist that may have a role in the treatment of arthritis and dermatitis in SLE

A

Methotrexate

Not effective in nephritis or other life-threatening disease

98
Q

Most patients with SLE of any type should be treated with this drug. This also prevents damage in skin and kidney and reduces overall damage scores

A

Hydroxychloroquine

99
Q

Approved by the FDA for use in SLE without active renal disease and indicated in patient with positive SLE autoantibodies which has failed standard treatments

A

Belimumab

100
Q

Lupus nephritis that has worse prognosis

A

Crescentic lupus nephritis

Presence of cellular or fibrotic crescents in glomeruli with proliferative glomerulonephritis

101
Q

Induction therapy of choice for crescentic lupus nephritis

A

High dose cyclophosphamide

102
Q

T or F. Fertility rates for men and women with SLE are is usually low

A

False. Fertility rates for men and women with SLE are probably normal

103
Q

Antiphospholipid antibody that is most often associated with fetal loss

A

Lupus anticoagulant

Other antiphospholipid antibodies are also associated with fetal loss

104
Q

Active SLE in pregnant women should be controlled with ______ and, if necessary, ______ for the shortest time required

A

Hydroxychloroquine
Prednisone/prednisolone at the lowest effective doses

Azathioprine may be added if these treatment do not suppress disease activity

105
Q

T or F. Breastfeeding is allowed while patient is on SLE therapy

A

False

106
Q

Treatment for SLE patients with antiphospholipid antibodies and prior fetal losses

A

Heparin

Usually LMW Heparin
May add low-dose aspirin

107
Q

Antibodies that is associated with neonatal lupus

A

Anti-Ro antibodies

108
Q

Target INR for APS patient with 1 episode of venous clotting while on warfarin

A

2-2.5

109
Q

Target INR for APS patient with recurring clots or arterial clotting, particularly in the CNS while on warfarin

A

3-3.5

110
Q

Microvascular thrombotic crisis in SLE includes (2)

A
  1. TTP

2. HUS

111
Q

Clinical features of microvascular thrombotic crisis in SLE (3)

A
  1. Hemolysis
  2. Thrombocytopenia
  3. Microvascular thrombosis in kidneys, brain, and other tissues
112
Q

TTP and HUS in SLE is common in what subset of patients

A

young individuals with lupus nephritis

113
Q

Most useful laboratory test in microvascular thrombotic crisis in SLE (3)

A
  1. Schistocytes on PBS
  2. Elevated LDH
  3. Antibodies to ADAMS13
114
Q

Treatment of microvascular thrombotic crisis in SLE

A

Plasma exchange or extensive plasmapheresis

Concomitant glucocorticoid
No evidence that cytotoxic drugs are effective

115
Q

Useful strategy in SLE dermatitis patient with inadequate improvement on topical glucocorticoids and antimalarials

A

Systemic retinoic acid

ADRs are potentially severe – fetal abnormalities

116
Q

T or F. All types of vaccination is allowed in SLE patients.

A

False. Attenuated live viruses is generally discouraged

117
Q

Reduce deaths from cardiac events in SLE patients

A

Statin

118
Q

Leading cause of death in the 1st decade of SLE (4):

A
  1. Systemic disease activity
  2. Renal failure
  3. Infections
  4. Thromboembolic events
119
Q

Antibodies that is usually associated with drug-induced lupus

A

antibodies to histones

120
Q

Antiodies not specific for SLE; associated with sicca syndrome, predisposes to subacute cutaneous lupus, and to neonatal lupus with congenital heart block; associated with decreased risk for nephritis

A

Anti-Ro (SS-A)

121
Q

Lupus nephritis ISN Class I

A

Minimal Mesangial Lupus Nephritis

122
Q

Lupus nephritis ISN class that is characterized by normal glomeruli by light microscopy, but mesangial immune deposits by immunofluorescence

A

Class I

123
Q

Lupus nephritis ISN Class II

A

Mesangial Proliferative Lupus Nephritis

124
Q

Lupus nephritis ISN Class III

A

Focal Lupus Nephritis

125
Q

Lupus nephritis ISN Class IV

A

Diffuse Lupus Nephritis

126
Q

Lupus nephritis ISN Class V

A

Membranous Lupus Nephritis

127
Q

Lupus nephritis ISN Class VI

A

Advanced Sclerotic Lupus Nephritis

128
Q

Lupus nephritis ISN class that is characterized by purely mesangial hypercellularity of any degree or mesangial matrix expansion by light microscopy, with mesangial immune deposits. A few isolated subepithelial or subendothelial deposits may be visible by immunofluorescence or electron microscopy, but not by light microscopy.

A

Class II

129
Q

Lupus nephritis ISN class that is characterized by active or inactive focal, segmental or global endo- or extracapillary glomerulonephritis involving ≤50% of all glomeruli, typically with focal subendothelial immune deposits, with or without mesangial alterations.

A

Class III

130
Q

Lupus nephritis ISN class that is characterized by active or inactive diffuse, segmental or global endo- or extracapillary glomerulonephritis involving ≥50% of all glomeruli, typically with diffuse subendothelial immune deposits, with or without mesangial alterations.

A

Class IV

131
Q

Lupus nephritis ISN class that is characterized by global or segmental subepithelial immune deposits or their morphologic sequelae by light microscopy and by immunofluorescence or electron microscopy, with or without mesangial alterations

A

Class V

Class V lupus nephritis may occur in combination with class III or IV, in which case both will be diagnosed. Class V lupus nephritis may show advanced sclerosis.

132
Q

Lupus nephritis ISN class that is characterized by ≥90% of glomeruli globally sclerosed without residual activity

A

Class VI