RHEUMA Flashcards
Strong predictor of the presence of rheumatic disease
Arthritis or athralgia
Arthritis
Malar rash that spares the nasolabial folds:
SLE
Malar rash that crosses the nasolabial folds
(Diffuse facial rash)
JDM
Gottron papules and heliotrope rashes
JDM
Which NSAID can cause snakk, hypopigmented depressed scars occuring in areas of minor skin trauma?
A. Aspirin
B. Naproxen
C. Indomethacin
D. Celecoxib
B. Naproxen can cause Pseudoporphyria
Tx: Discontinue naproxen
> Ibuprofen has been asstd w causing aseptic meningitis esp in lupus patient
Salicylates no longer used d/t hepatotoxicity and Reye Syndrome
Anti-malarial drug used in the treatment of rheumatic diseases
Hydroxychloroquine
> usedin SLE, JDM
> can be use in cutaneous manifestations of diseases and lupus flares
Most significant potent adverse effect of Hydroxychloroquine
Retinal toxicity:
> IRREVERSIBLE color blindness
MC rheumatic disease in childhood
JIA
> MC subtype: Oligoarthritis
2. Polyarthritis
3. SystemicJIA
Classic for sJIA
Salmon-colored lesions
> linear or circular
> distributed all over the trunk and prox extremities
> Non pruritic and migratory lasting <1hr
Isolated involvement of which joint almost NEVER a presenting sign of JIA
A. Elbows
B. knees
C. hip
D. IPJ, DPJ
C. Hip
Juvenile arthritis vs spondyloarthritis
Spondyloarthritis involves axial skeleton joints (sacroiliac) and hips
Inflammation of the small joints of the __ is highly suggestive of ERA (enthesitis-related arthritis)
A. wrists
B. Fingers
C. Foot
D. Hips
C. Foot - tarsitis
Typical presentation of reactive arthritis?
A. A/Symmetrical
B. Oligo/Polyarticular
Asymmetric, oligorarticular
> predilection for lower extremities
Most commonly affected joint in septic arthritis
- Knee
- hip
MC etiology of septic arthritis
S. aureus
> If adolescent, can be N. gonorrhea
Characteristic of septic arthritis
- Mono/Polyarticular
- A/febrile
Monoarticular
high grade fever
Characteristic of ARF
- Small/large joints
- Non-/Migratory
Affects large joints
Migratory
JRA criteria (ACR classification) (4)
- Age <16yo
- Persist arthritis in one or more joints
- Lasting =>6weeks
- Exlcusion of other forms of JIA
Features of Systemic JIA
- Fever pattern - quotidian
- Evanescent rash (appear at the height of fever)
- organomegaly - hepato, spleen
- LN enlargement
- Serositis
- ANA/Rf NEGATIVE
Features of oligoarticular JIA
> MC type of JIA
less than 4 jts
highest incidence of uveitis (30%) especially in ANA +
ASYMMETRIC, large joints are affected
Features of polyarticular JIA
> more than 5 jts affected, SYMMETRICAL
Reiter’s syndrome
Can’t pee, can’t see, can’t climb a tree
1. urethritis
2. conjunctivitis
3. arthritis
> under juvenile ankylospondyloarthropathy, reactive arthritis
MC inflammatory myositis in children
JDM
Hallmark of SLE
- Generation of autoab directed against self-ag, particularly nucleic acids
- multiorgan disease
Drug-induced lupus with definite association:
A. Hydralazine
B. Cephalosporins
C. Midazolam
D. Hydroxychloroquine
A. Hydralazine
HIPP
Hydralazine
INH
Procainamide
Penicillamine
Minocycline, methyldopa, diltiazem, chlorpromazine, etanercept, infliximab, adalimumab, IFN A
> reversible
M=F
anti-histone ab
slow drug acetylation
TRUE or FALSE.
ANA is highly specific for detecting SLE.
FALSE.
> ANA is very sensitive (95-99%) but has poor specificity (50%) in detected SLE.
> Anti-dsDNA is specific for SLE.
Presence of these antibodies is associated with Neonatal Lupus (3)
Anti-SSa, anti-SSb or anti-RNP
Cardiac finding in Neonatal Lupus
Congenital Heart Block
> Most feared complication
> may lead to cardiomyopathy
> prolonged PR interval
> irreversible 3rd deg heart block
> can be detected via fetal echo at 16wks
> fetal bradycardia from heart block –> hydrops fetalis
Autoab associated with drug-induced lupus
Anti-histone
Autoab highly specific to detect SLE and correlates w disease activity
Anti-dsDNA
MC cause of death within 1st several yrs of diagnosis of SLE
Infections and complications of glomerulonephritis and neuropsychiatric diseases
Over the years, atherosclerosis and malignancy are the MC causes of death
TRUE or FALSE. Neonatal lupus is an autoimmune disorder of neonates and infants.
FALSE
> It is not an autoimmune disease of the fetus but instead results from passively acquired autoimmunity when maternal IgG autoab cross the placenta and enter fetal circulation
Mainstay treatment for significant manifestations of SLE
Corticosteroids
Period of greatest vulnerability to detect congenital heart block in neonatal lupus
18-24 wks AOG
Most common first symptom in patients with JDM
Rash
> erythema in sun-exposed areas after exposure to UV light
> if rash is over the chest and neck = shawl signs
Rash: blue-violet discoloration of the eyelids asstd w periorbital edema
Heliotrope rash
Bright pink or pale, shiny, thickened or atrophic plaques over the PIJ and DIJ, occ on the knees and elbows, small jts of toes and ankle malleoli
Gottron papules
Diagnosis of JDM
Classic Rash + WMEM (any 3)
> weakness: symmetrical, proximal
Muscle enzyme elev
EMG: short small polyphasic, fibrillations, +sharp waves
Muscle biopsy: necrosis and inflammation
Children w classic rash of JDM but no muscle weakness or inflammation
Amyopathic JDM or dermatomyositis sine myositis
Most common type of scleroderma seen in 95% of children
Juvenile localized scleroderma
> AKA morphea
> MC subtype: linear scleroderma
Most frequent initial symptom in systemic sclerosis. Symptoms?
Raynaud phenomenon
> Classic triphasic sequence: BCE
blanching, cyanosis, erythema of the digits induced by cold or stress
Mechanism: CSD
1. arterial vasoconstriction (hypoperfusion, pallor- blanching)
2. venous stasis (cyanosis)
3. reflex vasodilation (erythema)
Most frequent initial symptom of Behcet Disease
Painful oral ulcers
Classic symptom of Sjogren Syndrome (2)
- Dry eyes (keratoconjunctivitis sicca)
- Dry mouth (xerostomia)
> rare in children
common in middle aged women
chronic inflamm autoimm dse - plasma and lymphocytic infiltration of exocrine glands
MC manifestations of Sjogren syndrome in children
Recurrent parotid gland enlargement and parotitis
> Sicca syndrome (dry mouth, painful mucosa, sensitive to spicy, halitosis, dental caries) seen in adults
MC organ affected by AA Amyloidosis
Kidneys
Saccular aneurysms seen in what phase of Kawasaki Disease?
1st phase: neutrophilic necrotizing arteritis
2nd phase: subacute/chronic vasculitis - Fusiform aneurysm
Principle criteria of Kawasaki Disease
CRASH+ Burn
1. Fever 38deg, at least 5 days
2. Conjunctivitis, bilateral non exudative w limbal sparing
3. Rash - polymorphic
4. Adenopathy - unilateral cervical at least >1.5cm
5. Strawberry tongue - erythema of oral and pharyngeal mucosa, dry cracked cherry lips
6. Hands and feet - edema and erythema
Highest risk of sudden death is seen in which phase of Kawaski disease?
- Subacute phase
* periungual desquamation of fingers and toes: 2-3 wks
* thrombocytosis
* development of CAA
* highest risk of sudden death
* lasts 3 weeks - acute febrile phase
* fever lasting 1-2wks
* acute phase: perineal desquamation - Convalescent phase
* all clinical signs of illness disappeared
* continues until ESR returns to normal
* 6-8wks after onset of illness
Best time to give live vaccination to a patient who received IVIG administration?
> IVIG may interfere with the immune response to live-virus vaccines as a result of specific antiviral antibody
So the measles-mumps-rubella and varicella vaccinations should generally be deferred until 11 mo after IVIG administration
non live vaccines don’t need to be delayed
