Rheuma Flashcards
idiopathic synovitis of peripheral joints associated with soft-tissue
swelling and joint effusion
JUVENILE IDIOPATHIC ARTHRITIS (JIA)
Pathophysio of JRA
Vascular endothelial hyperplasia and progressive erosion of articular cartilage
and contiguous bone
Association of JRA
− DR8 and DR5
SSx of JRA
− Morning stiffness; easy fatigability
− Joint pain later in the day, joint swelling, joints warm with decreased motion, and
pain on motion, but no redness
Dx of JRA
− Age of onset: <16 years
− Arthritis in one or more joints
− Duration: ≥6 weeks
− Onset type by disease presentation in first 6 months
Exclude other associations with JRA
Exclusion of other forms of arthritis, other connective tissue diseases and vasculitides,
Lyme disease, psoriatic arthritis, inflammatory bowel disease, lymphoproliferative
disease
Clinical PP factors for JRA
− Young age at onset
− Rheumatoid nodules
− Large number of affected joints
− Involvement of hip, hands and wrists
Lab PP factors for JRA
− RF+
− Persistence of anti-cyclic citrullinated peptide (CCP) antibodies
WHy is systemic JRA difficult to treat?
Systemic onset JRA is the most difficult to control in terms of both articular
inflammation and systemic manifestations (poorer with polyarthritis, fever >3
months and increased inflammatory markers for >6 months)
Categories of JRA
Pauciarticular (oligoarthritis)
Polyarticular, RF negative
Polyarticular RF positive
Systemic Onset
What type of JRA
° Pattern: 1-4 joints affected in first 6 months; primarily knees (++) and ankles
(+), less so the fingers; never presents with hip involvement
° Peak age <6 years
° F:M = 4:1
° % of all: 50-60%
Pauciarticular (oligoarthritis)
Extraarticular SSx of Pauciarticular (oligoarthritis) JRA
Extra-articular: 30% with anterior uveitis
Pauciarticular (oligoarthritis) JRA Labs
ANA+ in 60%; other tests normal; may have mildly increased ESR, CRP
auciarticular (oligoarthritis) JRA Tx
NSAIDs + intraarticular steroids as needed; methotrexate occasionally
needed
What type of JRA?
° Pattern: 5 joints in first 6 months; both UE and LE small and large joints; may have C-spine and TMJ involvement ° Peak age: 6-7 years ° F:M: 3:1 ° % of all: 30%
Polyarticular, RF negative
Association of Polyarticular, RF negative
Extra-articular: 10% with anterior uveitis
Labs of Extra-articular: 10% with anterior uveitis
ANA+ in 40%; RF negative; ESR increased (may be significantly), but
CRP increased slightly or normal; mild anemia
Tx of Polyarticular, RF negative
Treatment: NSAIDs + methotrexate; if not responsive, anti-TNF or other biologicals
(as FDA-approved for children)
What type of JRA
° Pattern: ≥5 joints as above but will be aggressive symmetric polyarthritis
° Peak age: 9-12 years
° F:M: 9:1
° % of all: <10%
Polyarticular RF positive
Extra-articular findings of JRA:
rheumatoid nodules in 10% (more aggressive)
Labs of Polyarticular RF positive JRA
RF positive; ESR greatly, CRP increased top normal; mild anemia; if anti-CCP antibodies are positive, then significantly worse disease
Tx of JRA
Treatment: long-term remission unlikely; early aggressive treatment is warranted
What type of JRA?
Pattern: arthritis may affect any number of joints, but course is usually polyarticular,
destructive and ultimately affecting hips, C-spine and TMJ
° Peak age: 2-4 years
° F:M: 1:1
° % of all: <10%
Systemic Onset
Fever pattern of systemic JRA
For initial diagnosis, in addition to arthritis in ≥1 joint, must have with or be preceded by fever ≥2 weeks documented to be quotidian (daily, rises to 39˚ then back to 37˚) for at least 3 days of the ≥2-week period
Systemic findings of JRA
Evanescent (nonfixed, migratory; lasts about 1 hour) erythematous, salmoncolored
rash (linear or circular), most over the trunk and proximal extremities
Generalized lymph node involvement
Hepatomegaly, splenomegaly or both
Serositis (pleuritis, pericarditis, peritonitis)
Tx of JRA
less responsive to standard treatment with methotrexate and anti-
TNF agents; consider IL-1 receptor antagonists in resistant cases.
Most with pauciarticular disease respond to __________
nonsteroidal antiinflammatory
drugs (NSAIDs) alone
Additional Tx to JRA
Additional treatmentmethotrexate (safest and most efficacious of secondline
agents); azathioprine or cyclophosphamide and biologicals
What is the prognosis?
Polyarticular disease
RF+
Older girls; hand and wrist;
erosions, nodules, unremitting
PP
What is the prognosis?
Polyarticular disease
ANA+ Younger girls
GP
What is the prognosis?
Pauci
ANA+
Younger girls; chronic iridocyclitis
Excellent,
except eyes
What is the prognosis?
Pauci
RF+ Polyarthritis, erosions, unremitting
PP
What is the prognosis?
Pauci
HLA B27/Seronegative
Older males
GP
What is the prognosis?
Systemic Pauciarticular
GP
What is the prognosis?
Systemic Polyarticular
PP
What is the etiolgy of SLE
Autoantibodies, especially against nucleic acids including DNA and other nuclear
antigens and ribosomes; blood cells and many tissue-specific antigens; immune
complex deposition
Immune complex deposition in the dermal/epidermal junction is specific for
SLE (called the___________)
lupus band test)
In SLE
________significantly increases risk for
severe renal morbidity (pathology varies from minimal mesangial changes to
advanced sclerosing nephritis
Diffuse proliferative glomerulonephritis
Difference in adult and child SLE
Compared with adults, children have more severe disease and more widespread
organ involvement
Usual presentation of SLE
Rare age <5 years and only up to 20% present age <16 years, so usual presentation
is mid-to-late adolescence
Diagnosis of SLE—
“M.D. Soap ’n Hair
• Malar rash • Discoid rash • Serositis • Oral ulcers • ANA-positive • Photosensitivity • Neurologic disorders • Hematologic disorders • Arthritis • Immune disorders (LE [lupus erythematosus] prep test, anti-DNA, Smith) • Renal disorders
MC Sx of SLE in children
Most common is a female with fever, fatigue, rash, hematological abnormalities
(anemia of chronic disease or hemolytic; thrombocytopenia, leukopenia)
and arthralgia/arthritis
Non-specific tests for SLE
elevated ESR, CRP, platelets, anemia, elevated WBC or leukopenia/
lymphopenia; decreased CH50, C3, C4 (typically decreased in active disease and
increases with treatment
______present in 95-99% of SLE patients but has poor specificity; does not
reflect disease activity; first screening test
+ANA:
_______: more specific (but not 100%) and may correlate with disease
activity, especially nephritis
+anti-DS-DNA
______ 100% specific but no disease activity correlation
+anti-Smith antibody (anti-Sm):
_________increased with Raynaud’s phenomenon (blanching of fingers) and pulmonary hypertension; high titer may be diagnostic of mixed CT disorder
Antiribonucleoprotein antibodies:
Antiribonucleoprotein antibodies: marker of?
antiribosomal-P-antibody is a marker for lupus cerebritis
_______ IgG maternal antibodies crossing the placenta and produce transient neonatal lupus; may suggest Sjögren syndrome
Anti-Ro antibody (anti-SSA):
________ also increased risk of neonatal lupus; may be associated with
cutaneous and pulmonary manifestations of SLE or isolated discoid lupus; also
seen in Sjögren syndrome
Anti-La (anti-SSB):
What Ab can be present?
° Increased risk of arterial and venous thrombosis
° Livedo reticularis
° Raynaud’s phenomenon
° Recurrent fetal loss
Antiphospholipid antibodies (APL; including anticardiolipin):
_______may give a false-positive serological test for syphilis; also seen in patients with neurological complications
Positive lupus anticoagulant
Association of diff lab tests for SLE
– Coombs positive: ______
– Antiplatelet antibodies: ______
– Antithyroid antibodies: _____
hemolytic anemia
thrombocytopenia
autoimmune thyroiditis
_______: may be found with drug-induced lupus; may act as a trigger in those prone to lupus or cause a reversible syndrome hepatitis is common
Antihistone antibodies
General principles of Tx for SLE
– Sunscreen and direct sun avoidance
– Hydroxychloroquine for all, if tolerated
– NSAIDs for joints
– Corticosteroids for more severe disease, especially renal
– Steroid-sparing immunosuppressives for severe disease
• Passive transfer of IgG across placenta; most is maternal anti-Ro and anti-La
NEONATAL LUPUS
SSx of NEONATAL LUPUS
Mostly presents at age 6 weeks with annular or macular rash affecting the face, especially
periorbital area, trunk and scalp after exposure to any UV light; generally lasts
3-4 months
Neonatal lupus:
May manifest with any SLE finding, but all resolve unless there is _________is permanent; if it is third degree,
pacing is usually required
congenital heart
block (
Genetic susceptibility of KD: highest in ______irrespective of location and in children
and sibs of those with KD
Asians
Age of occurence of KD
80% present at age <5 years (median is 2.5 years) but may occur in adolescence
PP factors of KD
Poor outcome predictors with respect to coronary artery disease: very young age,
male, neutrophilia, decreased platelets, increased liver enzymes, decreased albumin,
hyponatremia, increased CRP, prolonged fever
Pathology of thrombosis in KD
Loss of structural integrity weakens the vessel wall and results in ectasia or saccular
or fusiform aneurysms; thrombi may decrease flow with time and can become
progressively fibrotic, leading to stenosis
Absolute reqt for dx of KD
Absolute requirement: fever ≥5 days (≥101˚ F), unremitting and unresponsive;
would last 1−2 weeks without treatment
Rash pattern of KD
polymorphic exanthema (maculopapular, erythema multiforme or scarlatiniform
with accentuation in the groin); perineal desquamation common ion
acute phase
Coronary Aneursym asstd with KD
up to 25% without treatment in week 2-3; approximately 2−4% with early diagnosis and treatment; giant aneurysms (>8 mm) pose greatest threat for rupture, thrombosis, stenosis and MI; best detected by 2D echocardiogram
Myocarditis asstd with KD
in most in the acute phase; tachycardia out of proportion to the fever and decreased LV systolic function; occasional cardiogenic shock; pericarditis with small effusions.
About 25% with mitral regurgitation, mild and improves over time; best detected by 2D echocardiogram plus EKG
Most important tests at admission of acute KD are (1-2 weeks)
platelet count, ESR, EKG, and baseline 2D-echocardiogram
Tests for Subacute: next 2 weeks
acute symptoms resolving or resolved; extremity desquamation, significant increase in platelet count beyond upper limits of normal (rapid increase in weeks 2-3, often greater and a million); coronary aneurysm,
if present, this is the time of highest risk of sudden death
Most important tests at admission of subacute KD are
Follow platelets, ESR and obtain 2nd echocardiogram.
What KD stage
________: next 2-4 weeks; when all clinical signs of disease have disappeared
and continues until ESR normalizes
Convalescent
What labs to do during Convalescent stage
follow platelet, ESR and if no evidence of
aneurysm, obtain 3rd echocardiogram; repeat echo and lipids at 1 year
Tx of KD
follow platelet, ESR and if no evidence of
aneurysm, obtain 3rd echocardiogram; repeat echo and lipids at 1 year
When to give 2nd infusion of IVIg
If continued fever after
36 hours, then increased risk of aneurysm; give 2nd infusion
KD Preventive Tx if with aneurysms
Small solitary aneurysms: ______ indefinitely;
giant or numerous aneurysms need individualized therapy, including____
continue ASA
thrombolytic
Long-term follow-up with aneurysms from KD:
periodic echo and stress test and perhaps
angiography; if giant, catheter intervention and percutaneous transluminal coronary
artery ablation, direct atherectomy and stent placement (and even bypass surgery)
Prognosis of aneurysms
Overall- 50% of aneurysms regress over 1-2 years but continue to have vessel
wall anomalies; giant aneurysms are unlikely to resolve
Acute KD recurs in ______
1-3%
Most common vasculitis among children in United States; l
HSP
Pathology of HSP
leukocytoclastic vasculitis (vascular damage from nuclear debris of infiltrating neutrophils) + IgA deposition in small vessels (arterioles and venules) of skin, joints, GI tract and kidney
HSP
Skin biopsy shows _____
vasculitis of dermal capillaries and postcapillary venules with
infiltrates of neutrophils and monocytes; in all tissues, immunofluorescence shows
IgA deposition in walls of small vessels and smaller amounts of C3, fibrin and IgM
Arthralgia in HSP
oligoarticular, self-limited and in lower extremities; resolves in about 2 weeks, but may recur
GI Sx in HSP
pain, vomiting, diarrhea, ileus, melena, intussusception, mesenteric ischemia or perforation (purpura in GI tract)
Renal Rx in HSP
Renal: up to 50%: hematuria, proteinuria, hypertension, nephritis, nephrosis,
acute or chronic renal failure
American College of Rheumatology for HSP diagnosis: need 2 of the following: 1 2 3 4
(a) palpable purpura
(b) age of onset <10 years
(c) bowel angina = postprandial pain, bloody diarrhea
(d) biopsy showing intramural granulocytes in small arterioles and venules
Tx of HSP
Treatment: supportive and corticosteroids, but only with significant GI involvement
or life-threatening complications (but steroids do not alter course alter
overall prognosis nor prevent renal disease (c)
Chronic HSP Tx
for chronic renal disease – azathioprine,
cyclophosphamide, mycophenolate mofetil.
