Rheumatology Flashcards
strongest risk factor for SLE
being a female
this hormone promotes B-cell autoreactivity and thus play a role in SLE
Estrogen
hallmark of SLE
generation of autoantibodies directed against self-antigens
most common presenting complaints of children with SLE
-fever
-fatigue
-hematologic abnormalities
-arthralgia
-arthritis
most sensitive test for SLE
ANA
Most specific test for SLE
anti dsDNA and anti-smith
SLICC criteria has higher sensitivity but lower specificity compared to ACR criteria. True or False?
True
this test correlates with disease activity in SLE
anti-dsDNA
Treatment of choice in SLE
Hydroxychloroquine
this medication is reserved for most severe, potentially life threatening SLE manifestations
Cyclophosphamide
most common rheumatic disease in children
Juvenile idiopathic arthritis
most common subtype of JIA
oligoarthritis
most common inflammatory myositis in children
Juvenile Dermatomyositis
How to diagnose Juvenile Dermatomyositis?
Classic rash (heliotrope rash of the eyelids/Gottron papules) +
3 of the following:
-Weakness (Symmetric/Proximal)
-Muscle enzyme elevation (>=1, CK, AST, LDH, Aldolase)
-Electromyographic changes (Short, small polyphasic motor unit potentials/Fibrillations/Positive sharp waves/Insertional irritability)
-Muscle biopsy (Necrosis/Inflammation)
describe what is heliotrope rash in JDM
blue-violet discoloration of the eyelids
describe what are Gottron papules
bright-pink or pale, shiny, thickened or atrophic plaques over the proximal interphalangeal joints and distal interphalangeal joints and occasionally on the knees, elbows, small joints of the toes, and ankle malleoli
this test is present in >80% of children with JDM
ANA
Mainstay of treatment in Juvenile Dermatomyositis
Corticosteroids
Key process in the pathogenesis of both localized and systemic scleroderma
Autoimmunity
most common visceral manifestation of systemic scleroderma
Pulmonary disease
most frequent initial symptom in pediatric systemic sclerosis
Raynaud Phenomenon (RP)
- present in 70% of affected children
What is Raynaud Phenomenon?
classic triphasic sequence of blanching, cyanosis and erythema of the digits induced by cold exposure and/or emotional stress
major criterion in the diagnosis of Juvenile Systemic Sclerosis (JSSc)
Proximal skin sclerosis/induration of the skin proximal to metacarpophalangeal or metatarsophalangeal joints
Classic characteristic feature of advance Ankylosing spondylitis in xray
Bamboo spine
— squaring of the corners of the vertebral bodies and syndesmophyte formation
differentiate reactive arthritis from postinfectious arthritis
reactive arthritis refers to arthritis that occurs following enteropathic/urogenital arthritis while postinfectious arthritis occurs after infectious illnesses such as Group A streptococcus or viruses
when do symptoms of reactive athritis usually appear following infection?
3 days to 6 weeks
diffentiate Poststreptococcal arthritis from Rheumatic fever
RF arthritis manifests as painful migratory polyarthritis of brief duration while Poststreptococcal arthritis is typically oligoarticular
systemic inflammatory disorder manifesting as vasculitis with a predilection for the coronary arteries
Kawasaki Disease (KD)
How many of untreated children with Kawasaki Disease develop Coronary Artery abnormalities (CAA)
20-25%
predictors of poor outcome in Kawasaki Disease
- Young age
- Male gender
- Persistent fever
- Poor response to IVIG
- Laboratory abnormalities: neutrophilia, thrombocytopenia, transaminitis, hyponatremia, hypoalbuminemia, elevated levels of NT pro BNP and CRP
what type of arteries is affected in Kawasaki Disease?
Medium-size arteries (particularly Coronary arteries)
How to diagnose Kawasaki Disease?
Presence of High grade fever of at least 4-5 days duration (temp >38.3) that is remitting and unresponsive to antipyretics +
4 of 5 principal criteria as follows:
1. bilateral nonexudative conjunctival injection with limbal sparing
2. erythema of the oral and pharyngeal mucosa with strawberry tongue and red, cracked lips
3. edema (induration) and erythema of the hands and feet
4. rash of various forms (maculopapular, erythema multiforme, scarlatiniform, psoriatic-like, urticarial or micropustular)
5. nonsuppurative cervical lymphadenopathy, usually unilateral, with node size >1.5 cm
describe the 3 clinical phases of Kawasaki Disease
- ACUTE Phase - fever and signs of illness, lasts 1-2 weeks
- SUBACUTE Phase - assoc with desquamation, thrombocytosis, development of CAA, highest risk of sudden death who develop aneurysms, lasts 3 weeks
- CONVALESCENT Phase - all clinical signs disappeared, continues until ESR becomes normal, 6-8 weeks after the onset of illness
how often is 2D echo be performed in Kawasaki Disease?
at diagnosis
after 1-2 weeks of illness
if normal, repeat 6-8 weeks after onset of illness
diagnosis of KD should be made within ___ days, ideally within __ days of fever onset to improve coronary artery outcomes
10 days, ideally 7 days
treatment of Kawasaki Disease
IVIG - 2 g/kg as single infusion over 10-12 hour within 10 days of disease onset ASAP ++
Moderate (30-50 mkday q6) - high dose (80-100 mkday q6) Aspirin until afebrile
Antithrombotic dose of Aspirin used in KD after 48 hours afebrile
3-5 mkday single dose for 6-8 weeks
KD patients undergoing long-term aspirin therapy should receive what kind of vaccine and what is the purpose of it?
Annual Influenza vaccination to reduce risk of Reye Syndrome
most common vasculitis of childhood
Henoch-schonlein purpura
90% of HSP cases occur in what age group in children
3 and 10 year
hallmark of HSP
RASH — palpable purpura
» starting as pink macules or wheals and develops into petechiae, raised purpura or larger ecchymoses
Criteria for HSP
Palpable purpura (without coagulopathy/thrombocytopenia) + 1 or more of the ff:
- abdominal pain (acute, diffuse, colicky)
- arthritis or arthralgia
- biopsy of affected tissue demonstrating predominant IgA deposition
- Renal involvement (proteinuria >3 g/24 hr), hematuria or red cell casts
major long-term complication in HSP which occur in 1-2% of children
Renal disease
recommended serial monitoring of children with HSP
BP and Urinalysis for at least 6 months after diagnosis
— to monitor development of nephritis
known as pulseless disease
Takayasu Arteritis
What kind of occlusive complication is predominantly seen in Southest asia and Africa in Takayasu Arteritis?
Aneurysm
Most commonly involved aortic branches in Takayasu Arteritis
- Renal artery
- Subclavian artery
- Carotid artery
Crtieria for Pediatric-Onset Takayasu Arteritis
- Angiographic abnormalities of the aorta or its main branches and at least 1 of the ff:
> Decreased peripheral artery pulse and/or claudication of extremities
> BP difference between arms or legs of >10 mmHg
> Bruits over the aorta and/or its major branches
> Hypertension
> Elevated ESR or CRP
Mainstay therapy of Takayasu Arteritis
Glucocorticoids
Systemic necrotizing vasculitis affecting small and medium size arteries
Polyarteritis nodosa
these vessels are usually spared in Polyarteritis Nodosa
Pulmonary Vasculature
gold standard diagnostic imaging study for PAN
Conventional arteriography
— “beads on a string” appearance
Most common among the JIA types
Oligoarthritis (40-50%)
What is the criteria for Juvenile rheumatoid arthritis (based on ACR)
Age at onset: <16 yr old
Arthritis (swelling/effusion/presence of >2 of the ff signs: Limitation of range of motion, tenderness or pain on motion, increased heat) in more than or equal to 1 joint
duration: more than or equal to 6 weeks
onset type defined by type of articular involvement in the 1st 6 mos
exclusion of other forms of juvenile arthritis
What is Quotidian fever
fever that rises to 39 C once daily and returns to 37 C between fever peaks
Rare but potentially fatal complication of sJIA
Macrophage activation syndrome (MAS)
Laboratory features found in MAS
Cytopenia
Abnormal liver function tests
Coagulopathy (hypofibrinogenemia)
Decreased ESR
Hypertriglyceridemia
Hyponatremia
Hypoalbuminemia
Hyperferritinemia
Elevated sCD25 and scD163
Proposed criteria for MAS in sJIA
Serum ferritin >684 ng/mL +
Any 2 of the ff:
Thrombocytopenia (<181 x 10^9)
Elevated liver enzymes (AST >48 U/L)
Hypertriglyceridemia (>156 mg/dL)
Hypofibrinogenemia (<360 mg/dL)
Oldest and least toxic of the DMARDs
Methotrexate
Predictors of severe and persistent disease in JIA
young age at onset
RF seropositivity
Rheumatoid nodules
Presence of anti-cyclic citrullinated peptide antibodies
Polyarticular, especially of hips and hand/wrist
Diagnosis of SLE based on ACR (1997)/SLICC (2012)
ACR: 4 out of 11
1 Malar rash
2 Discoid rash
3 Photosensitivity
4 Oral/Nasal ulcers
5 Arthritis: Nonerosive, >2 joints
6 Serositis: Pleuritis, Pericarditis, Peritonitis
7 Renal manifestations: Consistent renal biopsy/Persistent proteinuria or renal casts
8 Seizure or psychosis
9 Hematologic abnormalities: Hemolytic anemia, Leukopenia (<4,000 leukocytes/mm3), Lymphopenia (<1,500), Thrombocytopenia (<100,000)
10 Immunologic abnormalities: (+) Anti-dsDNA or Anti-smith, False positive rapid plasma reagin test result, positive lupus anticoagulant test result or elevated anticardiolipin IgG or IgM antibody
11 Positive ANA result
SLICC: 4 (at least 1 clinical, 1 immunologic)
Clinical
Acute cutaneous lupus
Chronic cutaneous lupus
Oral or nasal ulcers
Nonscarring alopecia
Synovitis
Serositis: Pleurisy/Pericardial pain >1 day, pleural effusion or rub, pericardial effusion or rub, ECG evidence of pericarditis
Renal: (+) RBC casts or UPCR >500 mg/protein/24 hr
Immunologic
(+) ANA, Anti-dsDNA, (+) anti-smith antibody
(+) Antiphospholipid antibody
Low complement (Low C3, C4, CH50)
(+) Direct coombs test
Hallmark of SLE
Multiorgan Disease
Positive anti-histone antibodies is highly suggestive of this condition and is seen in only 20% of SLE
Drug-induced Lupus
Definite medications associated with drug-induced lupus
Minocycline
Procainamide
Hydralazine
Isoniazid
Penicillamine
Diltiazem
Interferon-alpha
Methyldopa
Chlorpromazine
Etanercept
Infliximab
Adalimumab
These antibodies are associated with sicca syndrome and may suggest diagnosis of sjogren syndrome
Anti-Ro and Anti-La
Potential toxicity of Hydroxychloriquine
Retinal deposition which can cause visual impairment
most common causes of mortality in SLE during the first few years of diagnosis
infection
complications of glomerulonephritis and neuropsychiatric disease
most common causes of mortality in SLE over the long-term
Complications of atherosclerosis and malignancy
Neonatal lupus results from passively acquired autoimmunity or maternal transfer of IgG autoantibodies. At what age of gestation?
12 weeks AOG
Most feared complication of Neonatal Lupus
Congenital heart block
Inflammatory manifestations gradually resolve at what age seen in neonatal lupus
6 months
this is a condition with episodic color changes and development of nodules related to severe cold exposure and spasm-induced vessel and tissue damage; it has been associated with SLE
Chilblains
condition of small vessel necrotizing granulomatous (allergic granulomatosis) vasculitis associated with a history of refractory asthma and peripheral eosinophilia
Eosinophilic granulomatosis with polyangitis// Churg-Strauss Syndrome (CCS)
Criteria for Pediatric-Onset granulomatosis with polyangiitis
Requires 3 out of 6 of the ff:
Histopathology showing granulomatous inflammation
Upper airway involvement
Laryngeal, tracheal or bronchial involvement
ANCA positivity
Renal involvement
Proteinuria, hematuria, RBC casts, necrotizing pauci-immune golemrulonephritis
ANCA antibodies positive in GPA and MPA
GPA: c-ANCA/anti-PR3 antibodies
MPA: p-ANCA
patients with JIA, especially those with oligoarthritis, ANA positive and has onset of arthritis before 6 yr of age are at greatest risk for development of what condition
chronic uveitis
most common cause of mortality in SLE
Atherosclerosis and malignancy
