Rheumatology Flashcards
1
Q
What are 3 long term complications of JIA
A
uveitis flexion contracture leg length discrepancy of involved leg joint erosions muscle wasting growth disturbances delay of motor development malalignment of joints TMJ disease
2
Q
what are the criteria for Kawasaki disease
A
fever >5 days and 4/5 of:
C- conjunctivitis (bilateral, non exudative)
R- rash (polymorphous)
A- adenopathy (>1.5cm)
S- skin changes (fissured lips, strawberry tongue)
H- hands and feet (edema, erythema) and perineal (erythema/peeling)
typical age 3 mon- 5 years
3
Q
what do we worry about for familial Mediterranean fever? what is the treatment?
A
amyloidosis, especially renal amyloidosis
tx: colchicine
4
Q
what rash is seen with dermatomyositis? what type of weakness? what nail changes? nodules?
A
heliotrope rash over the eyelids (purple)
gottron’s papules (dorsal surface of the knuckles)
symmetric proximal weakness
can get nailfold changes- drop out, dilatation, tuortuosity
calcinosis- calcium nodules
5
Q
what 3 investigations would you do for dermatomyositis? Tx?
A
EMG- denervation and myopathy
muscle biopsy- necrosis and inflammation
lab work ( elevated CK, AST, LDH, aldolase)
MRI-can help identify muscle inflammation
Tx: corticosteroids
6
Q
what are 2 renal complications associated with lupus?
A
HTN Proteinuria edema renal failure hematuria nephrotic syndrome
7
Q
what are the criteria for rheumatic fever?
A
2 major or 1 major and 2 minor with evidence of GAS infection (positive throat culture or elevated or rising ASOT) J- joints (migratory arthritis) O- carditis N- nodules (subcutaneous) E- erythema marginatum S- syndenham's chorea
Minor: arthralgia, fever, ESR, CRP, prolonged PR
8
Q
what are the 7 types of JIA
A
Age <16 and persists for > 6 weeks
- oligo
- polyarticular RF +
- polyarticular RF -
- systemic JIA
- enthesitis related arthritis
- psoriatic arthritis
- undifferentiated arthritis
9
Q
what is the definition of arthritis
A
joint swelling/ effusion
OR
limited ROM with joint line tenderness or painful ROM
10
Q
what is the most common type of JIA
-most frequent joints involved? (4)
A
oligoarthritis
- most frequent joints involved:
1. Knees
2. Ankles
3. Wrists
4. Elbows
11
Q
what are the two types of oligoarthritis
A
persistent oligoarthritis- <4 joints
extended oligoarthritis- >4 joints after first 6 months
12
Q
what increases risk of uveitis with oligoarticular JIA (4) and what is the treatment for uveitis?
A
ANA positive young onset age girls oligoarticular JIA tx: topical steroids
13
Q
What causes neonatal lupus? why do we worry about neonatal lupus?
A
maternal autoantibodies anti- ro and la
worry about 3rd degree heart block
14
Q
what is the treatment for Kawasaki disease?
A
acute phase:
IVIG x 2 (if poor response to first dose)
steroids for persistent symptoms unresponsive to IVIG x2, symptomatic myocarditis
regular dose ASA
subacute phase:
low dose ASA
echo surveillance in acute phase and at 6 weeks
15
Q
uveitis in JIA is most common with what type?
A
oligoarthritis
associated with positive ANA
usually ASYMPTOMATIC
16
Q
what type of JIA is associated with symptomatic uveitis
A
enthesitis related arthritis
17
Q
what are 4 complications of uveitis
A
synechiae- irregular pupil
glaucoma
cataract
visual loss
18
Q
what can be seen on x ray with JIA
A
acceleration of growth acceleration of maturation growth inhibition osteoporosis erosions loss of cartilage
19
Q
what can be seen on MRI with JIA
A
bone marrow edema
erosions
synovitis
cartilage
20
Q
what is required for systemic JIA
A
2 weeks of fever arthritis and at least one of: - rash (evanescent, salmon coloured) - generalized lymphadenopathy - hepatosplenomegaly - serositis
21
Q
what lab findings are associated with MAS? on bone marrow?
A
massive release of pro inflammatory cytokines
accumulation of macrophages in liver, spleen, LN and CNS
elevated ferritin elevated lipids elevated CRP decreased ESR elevated d-dimer decreased fibrinogen
on bone marrow- hemophagocytosis
22
Q
what is the treatment for oligo JIA? poly JIA? systemic JIA
A
oligo-NSAIDS, joint injection
poly-NSAIDS then MTX and/or SSZ, Biologic
systemic- NSAIDS then corticosteroids and/or MTX then biologic (often tx now with anti IL-1 or anti IL-6 first)
23
Q
can live vaccines be given with MTX and biologics??
A
NO!
24
Q
what antibiotic cannot be given with MTX except in prolyphactic dosing?
A
sulpha antibiotics
25
what is the treatment for rheumatic fever?
penicillin x 10 days
ASA/NSAIDS for arthritis
ASA/steroids for carditis
without carditis: prophylactic penicillin for at least 5 years or until 21 years
with carditis: up to 21 yo or 10 years post attack
residual heart disease: up to age 40 or 10 years post initial attack (essentially life long)
26
what is the most common vital organ involvement in SLE?
kidney- glomerulonephritis
27
what are the indications for testing for APS
thrombosis
SLE yearly
unexplained PTT prolong
clinical features of APS
28
what is the treatment for HSP?
supportive
NSAIDs
systemic steroids- considered if severe abdominal pain, severe renal disease
29
what are the criteria for HSP
palpable purpura with lower limb predominance and without thrombocytopenia or coagulopathy and at least 1 of the following
●Abdominal pain (usually diffuse, with acute onset)- can see intusseception
●Arthritis or arthralgia (acute onset)
●Renal involvement (proteinuria, hematuria)
●Leukocytoclastic vasculitis or proliferative glomerulonephritis, with predominant IgA deposition
recurrences common in approx 30% in the first 1-2 months
30
what investigation is typically done before treating someone with systemic JIA
bone marrow aspirate
31
what are the characteristics of growing pains
```
age 3-10 years
bilateral leg pain
occurs at night
no interference with activities
normal physical exam
normal lab tests (if done)
```
32
what is the common cause of hip pain age 2-6
transient synovitis
| - may follow viral infection
33
what is the common cause of hip pain age 4-10
legg calves perthes
34
what is the common cause of hip pain age 10-14
slipped capital femoral epiphysis
= SURGICAL EMERGENCY
No weight bearing until pinned
35
what is complex regional pain syndrome? treatment?
girls>> boys
may follow trauma
very painful, tender extremity
autonomic changes (cool, sweaty, swollen, discoloration)
two types: no nerve lesion versus definable nerve lesion
treatment: physio, psychotherapy, pharmacotherapy
36
peak age for oligo? common joints?
peak age 1-3
girls>boys
affects large joints (knee most common- also ankles, wrists, elbows)
37
How often do we screen for uveitis for oligo
age of onset of arthritis <6 and ANA +: every 3 months initially then every 6 months
age of onset >6 and ANA +: every 6 months
38
what joints are affected with RF-neg polyarticular JIA
small and large joints
| TMJs
39
what are the characteristics of RF positive polyarticular JIA
teenage girls
symmetrical small and large joint arthritis
rheumatoid nodules over pressure points in 30%
ANA may be positive
worse prognosis with erosive disease
40
what are the characteristics of enthesitis related arthritis
```
arthritis and enthesitis
older boys >> girls
lower limb arthritis
sacroilitis
family history
HLA-B27 positive
```
41
enthesitis related arthritis may be associated with what 3 things
IBD
psoriasis
symptomatic uveitis
42
dactylitis is associated with what type of arthritis?
psoriatic arthritis
43
what are the characteristics of systemic JIA
fever (x 2 weeks for diagnosis)- must be 3d in a row over the 2 weeks
rash
arthritis- may be delayed*
hepatosplenomegaly
lymphadenopathy
serositis
lab work: anemia, high ESR, CRP, platelets
44
what type of fever is seen with systemic JIA
quotidian, daily, predictable (every night or morning and night)
45
what type of rash is seen with systemic JIA
evanescent- salmon colored rash
46
what are the clinical features of MAS
```
persistent fever
hepatosplenomegaly
lymphadenopathy
hermorrhagic skin rash
CNS dysfunction
```
47
what lab findings are associated with MAS
```
cytopenias
hypofibrinogenemia
transaminitis
coagulopathy
hypertriglyceridemia
marked increase ferritin
increase CRP
decreased ESR
low NK cell function
high CD 25
```
48
what 3 rheumatic conditions are associated with MAS
SLE
systemic JIA
kawasaki disease
49
what are the treatment options for MAS
```
high dose corticosteroids
IVIG
cyclosporine
etoposide
ATG
IL-1 inhibition
```
50
what side effects are associated with MTX
nausea
oral ulcers (typically do not get because they are also on folic acid)
hepatotoxicity
leukopenia
infection
pulmonary toxicity
adolescents- try to avoid EtOH entirely, contraception required if sexually active
51
what must you rule out before starting anti-TNF agents?
TB!
52
what antibodies are highly specific for SLE
anti-ds DNA
| anti-sm
53
what is highly sensitive for lupus?
ANA- sensitive
| >95% of patients with lupus are ANA +
54
antiphospholipid antibody syndrome is associated with what? causes what 2 things?
associated with lupus
| causes arterial and venous thrombosis
55
what are the 2 types of lupus nephritis
mesagnial lupus nephritis
focal lupus nephritis
diffuse lupus nephritis
56
what do we monitor on labs for lupus
complement
ANA, anti-DNA antibodies
ESR- may reflect overall disease activity
CBC: anemia, thrombocytopenia and lymphopenia
* high CRP often suggests infection, serositis or MAS
57
what is the treatment for lupus
mild disease:
NSAIDS
plaquenil
low dose steroids
severe disease:
high dose steroids, immunosuppressive therapy (cyclophosphamide, rituximab etc)
58
what drugs can trigger drug-induced lupus?
anticonvulsants, minocycline, hydralazine, anti-TNF
| tx: stop the drug
59
what are the 3 D's associated with dermatomyositis affecting smooth muscle
dysphagia- difficulty swallowing
dysphonia- nasal speech
dyspnea- respiratory failure with severe weakness
60
what is the treatment of dermatomyositis
steroids
- pulse steroids
- MTX
- IVIG for more severe weakness
61
what should be monitored for HSP for 6 months min?
BP
| urinalysis
62
what lab features are associated with kawasaki disease
```
neutrophilic leukocytosis
anemia
thrombocytosis (day 7+), occ thrombocytopenia
elevated ESR, CRP
elevated transaminases
hypoalbuminemia*
sterile pyuria
```
63
what are the two main risk factors associated with developing coronary aneurysms from kawasaki disease
age <1*and >9
| prolonged fever*
64
what are other manifestations of kawasaki disease
```
aseptic meningitis
anterior uveitis
oligo-or polyarthritis
hydrops of the gallbladder
meatitis and dysuria
diarrhea in approx 25%
peeling in the perineal area
```
65
what is the main cardiac complication associated with kawasaki disease?
```
coronary artery dilation
coronary artery aneurysms
also associated with:
myocardial infarction
myocarditis
pericarditis
valvular dysfunction
arrhythmias
```
66
what does PFAPA stand for? what is the typical history?
periodic fever, apthous stomatitis, pharyngitis, adenitis
onset <5years (self limiting within 5 years)
fever every 3-6 weeks, regular recurrence
fever typically lasts 5 days
associated with apthous stomatitis and or pharyngitis (strep swab -ve)
completely well between episodes
no genetic or ethnic associations!!
tx: single dose prednisone
67
what are the clinical features of FMF
*fever + serositis!
most common hereditary autoinflammatory disease
AR
```
fever episodes present age <20
short duration of fever <72h, irregular
associated with serositis (peritonitis>pleuritis? pericarditis)
erysipeloid erythema
arthralgia>arthritis
mylagia
orchitis (5%)
lack of eye involvement
```
68
what are the cardinal features of a joint infection
redness
fever
swelling
pain and tenderness
69
what is the joint position for joint infection
abduction
| external rotation
70
what are the most common causes of septic joint
staph and strep
| also get gram neg bacteria- meningococcus, kingella
71
what are the kocher criteria
unable to weight bear
fever >38.5
wbc greater than 12000
elevated inflammatory markers (ESR >40, CRP >20)
helps differentiate transient synovitis from septic arhtritis
72
what rash is seen with lyme disease?
erythema migrans
73
what two infections can mimic monoarthrtitis
```
TB
Lyme disease (borrelia burgdorferi)- tx: amoxicillin or doxycycline if >10 yrs or ceftriaxone for disseminated
```
74
boy with slapped cheek rash and arthritis
parvovirus arthritis
75
Ddx erythema nodosum (6)
```
Group A strep
TB
Sarcoidosis
IBD
OCP
SLE
```
76
what type of anemia is seen with lupus?
autoimmune hemolytic anemia
77
anti ro/la is associated with what 3 conditions
neonatal lupus
sjogren
SLE
78
c ANCA
wegener granulomatosis
79
p ANCA
```
microscopic polyangitis
UC
PSC
SLE
Churg-Strauss syndrome (EGPA)
```
80
Associations with HLAB27 (5)
```
ankylosing spondylitis
JIA enthesitis related, psoriatic
IBD
reactive arthritis
isolated acute anterior uveitis
```
81
general definition of JIA
arthritis of unknown ethology that begins before 16th birthday and lasts more than 6 weeks
82
definition of oligoarthrtitis
arthritis affecting 1-4 joints during the first 6 months
83
what is the major complication of systemic JIA
Macrophage activation syndrome
84
what is the criteria for enthesitis related arthritis
Arthritis and enthesitis
Or, arthritis or enthesitis with at least 2 of the following:
- Presence or history of sacroiliac joint tenderness and/or inflammatory back pain
- Presence of HLA B27 antigen
- Onset of arthritis in a male over 6 years of age
- Acute (symptomatic) anterior uveitis
- History of ankylosing spondylitis, enthesitis related arthritis, sacroiliitis with inflammatory bowel disease, or acute anterior uveitis in a first-degree relative
85
what is the hallmark of psoriatic arthritis
dactylitis "sausage digit"
86
what is the criteria for psoriatic arthritis
```
Arthritis and psoriasis
Or, arthritis and at least 2 of the following:
- Dactylitis
- Nail-pitting or onycholysis
- Psoriasis in a first-degree relative
```
87
what are characteristics of Raynaud phenomenon? triggers?
| tx?
vascular spasm leading to triphasic color sequence (white, blue, red)
well demarcated areas of color change
usually fingers and toes
triggers= cold, stress, caffeine, smoking
Treatment:
-avoid precipitants
-may need nifedipine (peripheral vasodilator)
88
what organisms cause reactive arthritis
```
salmonella
shigella
yersinia
campylobacter
chlamydia
ureaplasma
```
89
Criteria for Lupus
- what do you see in complement level in SLE?
- what lab markers are used to monitor disease activity (2)?
SOAP BRAIN MD
4/11 criteria
Serositis – Pleurisy, pericarditis
Oral ulcers- usually painless
Arthritis- non erosive, 2 or more joints
Photosensitivity
Blood disorders (hemolytic anemia, leukopenia, thrombocytopenia)
Renal involvement- proteinuria, cellular casts
Antinuclear antibodies
Immunologic phenomena (dsDNA or anti-sm; anticardiolipin, lupus anticoagulant, false positive syphilis >6mo)
Neurologic disorder- seizures OR psychosis
Malar rash
Discoid rash
* **Complement is LOW in SLE
- C3 and C4 are used to monitor disease activity = levels fall during a flare and return to normal after appropriate treatment
90
criteria for APS
Vascular thrombosis: ≥1 clinical episode of arterial, venous, or small vessel thrombosis in any tissue or organ confirmed objectively by validated criteria
91
What is the differential diagnosis for monoarthritis?
```
Trauma/Mechnical/Orthopedic
Infection (Acute-Septic/OM, Chronic- TB/Lyme)
Inflammatory (JIA, FMF)
Tumor
Hemarthrosis
```
92
What is the differential diagnosis for polyarthritis
Inflammatory (JIA/SLE/Vasculitis)
Infection
Malignancy
Mechnical Pain syndrome
93
What is rheumatoid factor?
IgM autoantibody reacts to Fc portion of IgG antibodies
94
Top common causes of septic arthritis? (3)
In neonates?
In sickle cell?
In teenagers?
1. Staph aureus
2. Strep pneumonia
3. Non Group A strep
4. Kingella Kingae
In neonates: GBS and gram negatives
Sickle cell: Salmonella
Teenagers: gonorrhea
95
Antibiotic choice for osteomyelitis/septic arthritis:
- neonate
- 1-3 month old
- child
- teenager
- sickle cell
- Neonate: Cloxacillin and Gentamicin
- 1-3 month old: Cefuroxime (because of more gram negatives)
- Child: Cefazolin
- Teenager: ceftriaxone OR cefixime + azithromycin
- Sickle cell: cefotaxime (salmonella coverage)
96
3 circumstances where diagnosis of ARF can be made without Jones criteria:
1. Chorea (may occur as the only manifestation)
2. Indolent carditis
3. Recurrent episodes
97
What is the timing of development of acute rheumatic fever in relation to when the GAS infection occurred?
2-4 wks after GAS pharyngitis
- 1/3 of patients with ARF have no history of antecedent pharyngitis
- only 10-20% of throat culture or rapid strep antigen tests will be positive at the time of presentation of ARF; this is why ASOT is needed!
98
What is the best confirmatory test for juvenile dermatomyositis?
-other tests?
EMG = denervation & myopathy
- other tests:
1. Muscle biopsy = perifascicular atrophy
2. CK can be normal or high
3. ANA: 80% can be positive
99
What are 3 ways that maternal SLE can affect neonate?
1. Rash
2. Congenital heart block
3. Thrombocytopenia
100
What are physical exam findings of ankylosing spondylitis? (4)
1. Loss of normal lumbar lordosis
2. Positive Schober test (decreased forward flexion)
3. Anterior uveitis
4. Enthesitis (ie. patellar tendon and achilles tendon)
101
What is the pathophysiology behind acute phase reactants?
- which lab markers increase in acute phase response? (11)
- which lab markers decrease in acute phase response? (3)
Acute phase reactants: plasma proteins produced by the liver that change production during acute phase of inflammation (mediated by cytokines)
- increased lab markers:
1. CRP
2. ESR
3. Complement proteins
4. Fibrinogen
5. Ferritin
6. Ceruloplasmin
7. Haptoglobin
8. G-CSF
9. IL-1 receptor antagonist
10. Serum amyloid
11. Coag factors
- decreased lab markers:
1. Albumin
2. Transferrin
3. IGF-1
102
A patient with JIA has a positive ANA. What 3 things does this put them at risk for?
1. Earlier disease onset
2. Asymmetric arthritis
3. Uveitis
103
What level is considered a positive ANA?
| -when should specific antibodies (ie. anti-dsDNA) be ordered?
>1:160
| -specific antibodies should only be ordered if ANA is positive AND a disease other than JIA is suspected (ie. SLE)
104
What 4 antibodies are SPECIFIC for SLE?
| -what antibody is SENSITIVE for SLE?
- most sensitive antibody: ANA (if negative, very unlikely to have SLE
- most specific:
1. anti-dsDNA
2. anti-Ro
3. anti-La
4. anti-smith
105
What specific auto-antibodies (3) can often be seen with juvenile dermatomyositis?
1. Anti-Jo1
2. Anti-SRP
3. Anti-Mi-2
106
What specific group of auto-antibodies are associated with increased risk of thrombosis?
-two examples of these?
Antiphospholipid antibodies
- this is why people with antiphospholipid antibody syndrome are at increased risk of clots and pregnancy loss secondary to clots
- ex. lupus anticoagulant, anticardiolipin
107
A patient presents to you with dactylitis. What is your differential diagnosis? (3)
1. Sickle cell anemia
2. JIA (ie. enthesitis related, psoriatic arthritis)
3. Trauma
108
What is the diagnostic criteria for RF-negative-polyarthritis?
-what about RF-positive polyarthritis?
RF-negative polyarthritis:
1. Arthritis affecting 5 or more joints during first 6 months of disease
2. RF testing negative
RF-positive polyarthritis:
1. Arthritis affecting 5 or more joints during first 6 months of disease
2. 2 or more positive tests for RF at least 3 months apart during first 6 months of disease
109
Which population is usually affected by RF positive polyarthritis?
Adolescent girls = this is essentially adult-type rheumatoid arthritis presenting in a child or adolescent
-usually involve the PIP and MCP joints
110
What is the triad of Felty's syndrome?
1. Rheumatoid arthritis
2. Neutropenia
3. Splenomegaly
111
What are possible long term complications of SLE?
1. Death from infection, renal, CNS, cardiac or pulmonary disease
2. Early onset coronary artery disease from vasculitis
3. Bone disease --> osteopenia, avascular necrosis from steroid use
4. Malignancy from use of immunosuppressants/biologics
112
What are the clinical features of NLE?
1. Cardiac: complete congenital heart block (associated with non-immune hydrops)
2. Annular, erythematous papulosquamous rash with fine scale and central clearing
- usually photosensitive
- can be present at birth or develop within first few weeks of life
- predilection for face and scalp
- self-resolving
3. Hematologic: thrombocytopenia (most common), neutropenia and anemia less common
4. Hepatic involvement: cholestatic hepatitis with elevated liver enzymes, hepatomegaly
5. Neurologic: macrocephaly, hydrocephalus, spastic paraparesis, vasculopathy
113
What is the treatment for NLE
If fetal bradycardia is found during pregnancy, need fetal ECHO and may require treatment with Dex +/- sympathomimetics
-neonates with complete heart block may need pacemaker
114
What are types of:
- large vessel vasculitis (2)
- medium vessel vasculitis (2)
- small vessel vasculitis (4)
Large vessel vasculitis:
1. giant cell arteritis (older adults)
2. takayasu arteritis
Medium vessel vasculitis:
1. Kawasaki
2. Polyarteritis nodosa
Small vessel vasculitis:
1. Wegener's granulomatosis
2. Churg-Strauss
3. Microscopic polyangitis
4. HSP
115
What are the clinical features of takayasu arteritis?
- diagnostic criteria?
- treatment?
Large vessel vasculitis involving the aorta and its branches (thoracic, abdominal, carotid)
-initially presents as non-specific inflammatory illness with fever, then evolves into chronic, fibrotic phase with signs and symptoms of chronic vascular insufficiency (pulse deficit, claudication, BP discrepancy, bruits)
***Diagnostic criteria:
Needs:
1. Angiographic abnormalities (MRI) of aorta or its main branches (aneurysm/diltation, narrowing or thickened arterial wall)
AND
1 of the following:
1. Pulse deficit or claudication
2. Discrepancy of 4 limb BP > 10 mm Hg
3. Bruits
4. Hypertension
5. Acute phase reactants elevated (ESR or CRP)
-treatment: corticosteroids plus second line agent: cyclophosphamide, methotrexate, etc.
116
What is the diagnostic criteria for:
- incomplete KD
- atypical KD
- alternate way to make diagnosis for KD aside from the 5 or more days of fever + 4/5 features
Incomplete KD: if 5 or more days of fever with 2-3 features instead of 4
-commonly seen in infants who are at higher risk of coronary artery involvement
Atypical KD: if KD with unusual manifestation (eg. renal failure)
Alternate way to diagnose KD: in presence of fever AND coronary artery involvement on echo
117
What is the usual age group for KD?
| -what is the pathophysiology?
3 mo-5 yo
| -small vessel vasculitis with predilection for coronary arteries; thought to be triggered by infectious agent
118
What is the diagnostic criteria for Wegener Granulomatosis?
***Overall, think small vessel vasculitis primarily affecting upper airway, lungs, and kidneys!!!!
Need 3/6:
1. Histopathology showing granulomatous inflammation within wall of artery
2. Upper airway involvement: chronic purulent or bloody nasal discharge, recurrent epistaxis, nasal septum perforation, saddle nose deformity, chronic sinus inflammation
3. Laryngo-tracheo-bronchial involvement: subglottic, tracheal or bronchial stenosis
4. Pulmonary involvement: nodules, cavities or pulmonary infiltrates (SOB, chronic cough, hemoptysis/alveolar hemorrhage)
5. ANCA positive
6. Renal involvement: proteinuria, elevated Cr, hematuria
119
What is the treatment for Wegener granulomatosis? (2)
Initial therapy: Corticosteroids and cyclophosphamide
Maintenance therapy: methotrexate, azathioprin, mycophenolate and low dose corticosteroids
120
What is the most common vasculitis in children?
HSP
121
What does the prognosis of HSP depend on?
Depends on severity of nephritis (worse prognosis with nephrotic syndrome)
-end stage renal disease occurs in 1-3% of patients
122
What is CREST syndrome?
| -most important complication?
Aka Limited systemic sclerosis
1. Calcinosis
2. Raynaud phenomenon
3. Esophageal dysmotility
4. Sclerodactyly
5. Telangiectasias
- most important complication: future development of pulmonary hypertension
123
A patient presents to you with isolated Raynaud phenomenon. What are the 2 best predictive factors for future development of autoimmune diseases?
1. Positive ANA
| 2. Abnormal nail fold vasculature
124
What is the most common form of localized scleroderma in the pediatric population?
Linear scleroderma: characterized by one or more linear streaks (following dermatomal distribution) extending over upper or lower extremity
- can involve face or scalp (en coup de sabre), usually forehead
- Parry-Romberg syndrome: progressive hemi-facial atrophy, disfiguring
125
What is the minimum duration of fever to be called fever of unknown origin?
Fever needs to last > 14 days with standard investigations not resulting in a clear diagnosis
126
What are the clinical features of TRAPS?
| -treatment?
TNF-receptor associated periodic syndrome
1. Long duration of fevers lasting 3-4 weeks and occur at irregular intervals
2. Migrating erythematous, maculopapular rash
3. Severe migratory myalgias with rash, arthralgias
4. Conjunctivitis, periorbital edema
5. Severe abdo pain
- treatment: unproven therapy; steroids provide symptomatic relief but do not change frequency
127
What are examples of DMARDs? (7)
DMARDs = disease-modifying anti-rheumatic drug
1. Azathioprine
2. Cyclophosphamide
3. Cyclosporine
4. Hydroxychloroquine
5. Methotrexate
6. Mycophenolate mofetil
7. Sulfasalazine
128
What monitoring needs to occur when patients are on hydroxychloroquine (plaquenil)?
Eye exams q6mo to assess for retinal deposits (retinal toxicity)
129
Name 2 classes of medications that can trigger drug-induced lupus.
antibiotics (ex: tetracyclines)
| anticonvulsants
130
Name 1 non-medication cause that can exacerbate lupus (2)
1. sun exposure
| 2. pregnancy
