RHEUMA Flashcards
1
Q
Polarized light microscopy can identify most typical crystals except for
A
Apatite
2
Q
Gpout is caused by deposition of what crystals
A
MSU crystals
3
Q
The most common early clinical manifestation of gout
A
Acute arthritis
Only one joint is affected initially
4
Q
Inflamed nodes which may be a first manifestation of gouty arthritis (2)
A
Heberden’s or Bouchard’s nodes
5
Q
Women represent only ____ of all patients with gout
A
5–20%
Mostly postmenopausal and elderly
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
7
Q
Thick pasty or chalky joint fluid in gout/tophi is due to
A
large number of crystals
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
9
Q
Ultrasound of gout show what sign?
A
double contour sign overlying the articular cartilage
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
11
Q
Mainstays of treatment of acute gout (3)
A
- NSAIDs
- Colchicine
- Glucocorticoids
12
Q
Intraarticular glucocorticosteroids used in acute gout (2)
A
- Triamcinolone acetonide (20–40 mg)
* Methylprednisolone (25–50 mg)
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)
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
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
16
Q
In patiets taking probenecid, urine volume should be maintained by ingestion of _____ of water every day
A
1500 mL
17
Q
Nonspecific xanthine oxidase inhibitor used in gout
A
Allopurinol
18
Q
Specific xanthine oxidase inhibitor used in gout
A
Febuxostat
19
Q
Most commonly used hypouricemic agent
A
Allopurinol
20
Q
Best drug to lower serum urate in overproducers, urate stone formers, and patients with renal disease
A
Allopurinol
21
Q
Dose of allopurinol in gout
A
100 mg initially and increasing up to 800 mg if needed
22
Q
Allopurinol toxicity is increased in patients with (2)
A
- Intake of thiazide
2. Allergy to penicillin and ampicillin
23
Q
A pegylated uricase for gout patients who do not tolerate or fail full doses of other treatments
A
Pegloticase
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
25
Calcium pyrophosphate deposition disease is most common in what group of patients
Elderly
26
Mutations in the ANKH gene will cause
Calcium pyrophosphate deposition disease
27
A minority of patients with CPPD arthropathy have hereditary CPP disease or these metabolic abnormalities (4)
* Hyperparathyroidism
* Hemochromatosis
* Hypophosphatasia
* Hypomagnesemia
28
Originally was termed pseudogout
Acute calcium pyrophosphate deposition disease arthritis
29
The joint most frequently affected in CPPD arthropathy
Knee
```
Other sites:
• Wrist
• Shoulder
• Ankle
• Elbow
• Hands
• Temporomandibular joint
```
30
Punctate and/or linear radiodense deposits within fibrocartilaginous joint menisci or articular hyaline cartilage seen in radiograph or ultrasound.
Chondrocalcinosis
31
Chondrocalcinosis is presumptive of
CPPD
DDx: CaOx in some patients with chronic renal failure
32
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
CPPD
33
Acute attacks of CPPD arthritis may be precipitated by (3)
1. Trauma
2. Severe medical illness
3. Surgery
34
Acute attacks of CPPD arthritis may be precipitated by surgery most especially,
Parathyroidectomy
causes rapid diminution of serum calcium concentration
35
Aka basic calcium phosphate disease
Calcium apatite deposition disease
Aka apatite arthropathy
36
the primary mineral of normal bone and teeth
Apatite
37
In CKD, this contribute to extensive apatite deposition both in and around joints
Hyperphosphatemia
38
Apatite aggregates are commonly present in synovial fluid in an extremely destructive chronic arthropathy of the elderly that occurs most often in the
shoulders (Milwaukee shoulder)
may also occur in hips, knees, and erosive osteoarthritis of fingers
39
30–50% of patients with ________ have apatite microcrystals in their synovial fluid
Osteoarthritis
40
Acute attacks of bursitis, tendinitis or synovitis is seen in what joint disease
Calcium apatite deposition disease
41
A rare hereditary metabolic disorder that may lead to nephrocalcinosis and renal failure
Primary oxalosis
42
Common cause of secondary oxalosis
Ascorbic acid supplements
43
Bipyramidal crystals with strong birefringence and may stain with alizarin red S
CaOx crystals
44
____ of SLE cases occur in women of child-bearing age
90%
45
Genetic “signature” in peripheral blood cells of 50-80% of SLE patients
Upregulation of genes induced by IFNs
46
The most characteristic gene expression pattern of SLE patients
Influence the IFN production
47
Female sex is permissive for SLE because of (3)
1. Hormone effects
2. Genes on the X chromosomes
3. Epigenetic differences between genders
48
SLE is associated with prolonged occupational exposure to
crystalline silica
49
Social behavior or lifestyle that reduces risk of SLE
Alcohol (2 glasses of wine a week or ½ of an alcoholic drink daily)
50
Biopsy of SLE shows (3)
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
51
Class of lupus nephritis that requires aggressive immunosuppression (3)
1. Class III
2. Class IV
3. Class V, accompanied by III and IV
52
Class of lupus nephritis that does not require aggressive immunosuppression (3)
1. Class I
2. Class II
3. With extensive irreversible changes
53
Most common pattern of vasculitis in SLE
Leukocytoclastic vasculitis
54
Antibodies that are specific for SLE (2)
High-titer Anti-dsDNA
| Anti-Sm
55
T or F. Permanent complete remission is rare in SLE
True
Permanent complete remissions – absence of symptoms with no treatment
56
Systemic symptom that is present most of the time in SLE (2)
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
Musculoskeletal manifestation that is present in most SLE patients
Intermittent polyarthritis
58
Rheumatoid-like arthritis with erosion that fulfill criteria for both RA and SLE
Rhupus
59
Most common reason that patients increase their dose of glucocorticoids in SLE
Joint pain
60
Pain persists in a single joint, such as knee, shoulder, or hip with no other manifestations of active SLE
Ischemic necrosis of bone
61
Most common chronic dermatitis in lupus
Discoid lupus erythematosus (DLE)
62
Roughly circular with slightly raised, scaly hyperpigmented erythematous rims and depigmented, atrophic centers in which all dermal appendages are permanently destroyed
Discoid lupus erythematosus (DLE)
63
Treatment of discoid lupus erythematosus (DLE) (2)
1. Topical or locally injected glucocorticoids
| 2. Systemic antimalarials
64
Consist of scaly red patches similar to psoriasis that is exquisitely photosensitive
Subacute cutaneous lupus erythematosus (SCLE)
65
Antibodies that is usually present in subacute cutaneous lupus erythematosus (SCLE)
anti-Ro (SS-A)
66
Usually the most serious manifestation of SLE
Nephritis
67
Test that is recommended for every SLE patient with any clinical evidence of nephritis
Renal biopsy
68
Cardiovascular event that is seen in most people with lupus nephritis after several years of the disease
Accelerated atherosclerosis
69
Most common manifestation of diffuse CNS lupus
Cognitive dysfunction
70
Neurologic manifestation that can be the dominant manifestation of SLE but must be distinguished from glucocorticoid-induced forms
Psychosis
71
MI in SLE is primarily a manifestation of
Accelerated atherosclerosis
Increased risk for vascular events is 3- to 10-fold
72
Most common pulmonary manifestation of SLE
Pleuritis with or without pleural effusion
73
Life threatening pulmonary manifestations of SLE (4)
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
Most frequent cardiac manifestation of SLE
Pericarditis
75
Cardiac manifestations of SLE (4)
1. Pericarditis
2. Myocarditis
3. Libman-Sacks endocarditis
4. Myocardial infarction
76
Most frequent hematologic manifestation
Anemia
Usually normochromic, normocytic – reflect chronic illness
77
T or F. Leukopenia in SLE is usually granulocytopenia
False. Almost always lymphopenia, not granulocytopenia
78
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)
1. Rituximab
2. Platelet growth factors
3. Splenectomy
79
Serious ocular manifestations of SLE (2)
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
The most important autoantibodies to detect in SLE
ANA
Positive in >95% of patients, usually at the onset of symptoms
81
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
Antiphospholipid antibodies
82
3 widely accepted tests for antiphospholipid antibodies
Anticardiolipin
Anti-β2-glycoprotein
Lupus anticoagulant
83
APS criteria (2)
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
Women with child-bearing potential and SLE should be screened for these 2 antibodies because both antibodies have the potential to cause fetal harm
antiphospholipid antibodies
| anti-Ro
85
Goal in the management of SLE
low-level disease activity (mild symptoms on the lowest possible doses of medications)
86
Low-level disease activity can be achieved for at least a year in ____ of SLE patients
30-50%
87
Mainstays in the treatment of non-life threatening SLE
1. Analgesics (NSAIDs and Acetaminophen)
| 2. Antimalarials
88
Antimalarials used in SLE (3)
1. Hydroxychloroquine
2. Chloroquine
3. Quinacrine
89
ADR of hydroxychloroquine
Retinal toxicity
in 6% of patients after cumulative doses of 1000 g, ~5 years of continuing therapy
90
Mainstay treatment of life-threatening SLE
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
An alkylating agent that is an acceptable choice for induction of improvement in severely ill patients
Cyclophosphamide
92
2 regimens of cyclophosphamide in SLE
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
Common adverse effect of high-dose cyclophosphamide regimen in women SLE patients
ovarian failure
Treated with a gonadotropin-releasing hormone agonist (e.g. leuprolide 3.75 mg IM) prior to each monthly cyclophosphamide dose
94
A relatively lymphocyte-specific inhibitor of inosine monophosphatase and therefore of purine synthesis
Mycophenolate mofetil
Acceptable choice for induction of improvement in severely ill SLE patients
95
A purine analog and cycle-specific antimetabolite that is used in treatment of SLE
Azathioprine
May be effective but is associated with more flares
96
Management of ISN grade III or IV lupus nephritis that reduces progression to ESRD
glucocorticoids + cyclophosphamide
97
A folinic acid antagonist that may have a role in the treatment of arthritis and dermatitis in SLE
Methotrexate
Not effective in nephritis or other life-threatening disease
98
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
Hydroxychloroquine
99
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
Belimumab
100
Lupus nephritis that has worse prognosis
Crescentic lupus nephritis
Presence of cellular or fibrotic crescents in glomeruli with proliferative glomerulonephritis
101
Induction therapy of choice for crescentic lupus nephritis
High dose cyclophosphamide
102
T or F. Fertility rates for men and women with SLE are is usually low
False. Fertility rates for men and women with SLE are probably normal
103
Antiphospholipid antibody that is most often associated with fetal loss
Lupus anticoagulant
Other antiphospholipid antibodies are also associated with fetal loss
104
Active SLE in pregnant women should be controlled with ______ and, if necessary, ______ for the shortest time required
Hydroxychloroquine
Prednisone/prednisolone at the lowest effective doses
Azathioprine may be added if these treatment do not suppress disease activity
105
T or F. Breastfeeding is allowed while patient is on SLE therapy
False
106
Treatment for SLE patients with antiphospholipid antibodies and prior fetal losses
Heparin
Usually LMW Heparin
May add low-dose aspirin
107
Antibodies that is associated with neonatal lupus
Anti-Ro antibodies
108
Target INR for APS patient with 1 episode of venous clotting while on warfarin
2-2.5
109
Target INR for APS patient with recurring clots or arterial clotting, particularly in the CNS while on warfarin
3-3.5
110
Microvascular thrombotic crisis in SLE includes (2)
1. TTP
| 2. HUS
111
Clinical features of microvascular thrombotic crisis in SLE (3)
1. Hemolysis
2. Thrombocytopenia
3. Microvascular thrombosis in kidneys, brain, and other tissues
112
TTP and HUS in SLE is common in what subset of patients
young individuals with lupus nephritis
113
Most useful laboratory test in microvascular thrombotic crisis in SLE (3)
1. Schistocytes on PBS
2. Elevated LDH
3. Antibodies to ADAMS13
114
Treatment of microvascular thrombotic crisis in SLE
Plasma exchange or extensive plasmapheresis
Concomitant glucocorticoid
No evidence that cytotoxic drugs are effective
115
Useful strategy in SLE dermatitis patient with inadequate improvement on topical glucocorticoids and antimalarials
Systemic retinoic acid
ADRs are potentially severe – fetal abnormalities
116
T or F. All types of vaccination is allowed in SLE patients.
False. Attenuated live viruses is generally discouraged
117
Reduce deaths from cardiac events in SLE patients
Statin
118
Leading cause of death in the 1st decade of SLE (4):
1. Systemic disease activity
2. Renal failure
3. Infections
4. Thromboembolic events
119
Antibodies that is usually associated with drug-induced lupus
antibodies to histones
120
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
Anti-Ro (SS-A)
121
Lupus nephritis ISN Class I
Minimal Mesangial Lupus Nephritis
122
Lupus nephritis ISN class that is characterized by normal glomeruli by light microscopy, but mesangial immune deposits by immunofluorescence
Class I
123
Lupus nephritis ISN Class II
Mesangial Proliferative Lupus Nephritis
124
Lupus nephritis ISN Class III
Focal Lupus Nephritis
125
Lupus nephritis ISN Class IV
Diffuse Lupus Nephritis
126
Lupus nephritis ISN Class V
Membranous Lupus Nephritis
127
Lupus nephritis ISN Class VI
Advanced Sclerotic Lupus Nephritis
128
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.
Class II
129
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.
Class III
130
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.
Class IV
131
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
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
Lupus nephritis ISN class that is characterized by ≥90% of glomeruli globally sclerosed without residual activity
Class VI
