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1. Rheum RC > Peds > Flashcards

Peds Flashcards

1
Q

** Peds joint pain differential **

A

PRIME BONE PAIN

-P: pharm: serum sickness, DLE
-R: rheum: JIA, SLE, SS, SSc, MCTD, vasculitis, DM, sarcoid
-I: infxn: bacterial (OM, diskitis, septic, rheumatic fever, ReA, lyme), viral (hep b/c, hiv, parvo, EBV, herpes, rubella)
-M: metabolic/genetic: marfan
-E: episodic: autoinflammatory (FMF, HIDS, TRAPS, CAPS, BLAU, PAPA, DIRA)

-B: blood/heme: SS, hemophilia
-O: ortho: osgood schlatter (apophysitis of tibial tubercle), Perthes, SCFE
-N: neoplastic: leuk/lymphoma, sarcoma, osteoma, osteochondroma, mets, neuroblastoma
-E: endocrine: hypothyroid, hypercortisol, DM, ricketts

-P: CRPS, fibro
-A: accidental/trauma
-I: inflamm: IBD
N: normal variant (growing pain), hypermobility

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2
Q

** DDX Fever & polyarthritis **

A

Polyarticular JIA (RF+, RF-)
-Systemic JIA, SLE, MCTD
-ERA, Juvenile PsA, ReA
-Sjogrens
-Sarcoidosis

-Rarely Lyme disease
-Acute rheumatic fever (ARF), Post Strep arthritis
-Endocarditis
-Parvo, EBV

-Sickle cell disease
-Leukemia/lymphoma
-Serum sickness

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3
Q

Organic vs nonorganic joint pain in Peds

A

Day+night (vs only night)
-Wkend + vacay (vs only school days)
-Interrupts play (vs can carrying normal activities)
-In joint (vs between joints)
-Limbs/refuses to walk (vs bizarre gait)
-Description fits anatomic explanation (vs illogical or dramatic)
-Other signs of systemic illness (vs isolated pain in othewise well)

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4
Q

DDx acute monoarthritis peds

A

Early rheum dz: oligoarticular JIA, ERA
-Infxn: septic, ReA, Lyme
-Ca: Leuk, neuroblastoma,
-Hemophilia
-Trauma
-Gout

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5
Q

DDx chronic monoarthritis peds

A

JIA: ERA, juvenile PsA, oligoarticular
-Sarcoid
-Infxn: TB, lyme
-Hemarthrosis: PVNS, hemophilia hemangioma
-Noninflamm: lymphangioma, synovial chondromatosis, lipomatosis arborescens

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6
Q

DDx chronic polyarthritis peds

A

-Polyarticular JIA, Sjogren’s
- ERA, Juvenile PsA
-SLE, MCTD

-Inxn: rheum fever, ReA, Lyme,
-Sarcoid

-Psueodorheumatoid chondrodyspasia
-Mucopolysacch

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7
Q

Synovial fluid findings
– SLE
– ReA
– Tb
– Septic arthritis

A

SLE: LE cells
-ReA: Reiter cells
-TB arthritis: acid fast bacteria (shld do biopsy)
-Septic arthritis: low glucose, bacteria, >75% PMN, 50k-300k WBC

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8
Q

Measuring leg length in Peds

A

ASIS to medial malleolus

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9
Q

When to suspect leukemia causing polyarthritis

A

High ESR
-LOW platelet
-High LDH, uric acid
-Abnormal smear

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10
Q

Cancer with MSK manifestations Peds

A

Leukemia
-Neuroblastoma
-Ewing sarcoma (monoarticular)
-Lymphoma

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11
Q

Hip pain ddx Peds

A

Cancer: osteoid osteoma, ewing sarcoma, leukemia, neuroblastoma
-Infxn: Septic arthritis, OM, rheumatic fever
-Autoimmune: JIA (ERA, JAS), ReA,
-Trauma, Fracture, AVN
-SCFE, Perthes, Protrusio acetabili

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12
Q

Back pain ddx peds

A

-Acute diskitis (Viral, Staph, Enterobacter, Moraxella)
-Disk herniation
-Spondylolysis +/- spondylolisthesis
-Juvenile discogenic disease
-Cancer (mets, primary bone tumor, leukemia)

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13
Q

** Main types of JIA**

A

-sJIA
-Oligoarthritis
-Polyarthritis (RF+ and RF-)
-PsA
-ERA/JAS (juvenile spondyloarthropathy)
-Undifferentiated arthritis

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14
Q

** JIA pathogenesis - interleukins involved**

A

-IL1
-IL6
-IL18

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15
Q

DDx for migratory arthralgia

A

-Rheumatic fever
-Poststep arthritis
-Gonococcal arthritis
-Lyme

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16
Q

Rashes specific to juvenile arthritis

A

Erythema marginatum = rheumatic fever
-Lower extremity purpura = HSP (IgA vasculitis)
-Evanescent salmon pink macules = sJIA

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17
Q

** sJIA (aka AOSD) clinical characteristics**

A

Arthritis in 1+ joints AND quotidian fever x 2+ wks and 1 of:

-Lymphadenopathy
-Evanescent Rash
-Hepato / splenomegaly
-Serositis (pleural, pericardial, peritoneal)

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18
Q

** Cytokines involved in sJIA**

A

IL 1, 6, 18 , TNF

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19
Q

**DDx sJIA **

A

-SLE, JDM, KD, AAV, PAN
-Sarcoid
-Autoinflammatory: FMF, cryopyrin assoc’d periodic syndromes, TNF-R assoc’d periodic ever syndrome

Infxn (any causing quotidian fever): endocarditis, bartonella, brucellosis, mycoplasma, rheumatic fever, TB

-Malignancy (solid/heme)

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20
Q

MAS manifestations

A

Fevers, LN
-CNS: sz, coma, ataxia, PRES
-Rash
-Hypotension
-ARDS
-Liver dysfcn (jaundice), HSM
-Cytopenias, Bleeding, bruising, purpura
-Renal dysfunction

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21
Q

MAS pathophys

A

Abnormal immune response to infection or autoantigen → exaggerated inflamm response
-Continual expansion of T lymphocytes and macrophages → increased proinflammatory cytokines, IL1/6

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22
Q

MAS causes

A

-Genetics
-Rheum: SLE, JIA, Kawasaki, DM, APS, MCTD
-Cancer
-Infxn: eg EBV
-Idiopathic

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23
Q

MAS Ix

A

Cytopenias, DIC (prolonged PT/PTT)
-High TG, Ferritin, soluble IL2R
-Liver dysfunction → transaminitis, prolonged PT/PTT, LOWER ESR and fibrinogen
-LP: CSF inflammatory
-Bone marrow Bx (hemophagocytosis)
-MRI brain
-Viral/Autoimmune serologies

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24
Q

MAS Biopsy

A

Macrophages show hemophagocytosis in BM, LN, or liver

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25
Q

HLH/MAS Tx

A

GC
-IVIG
-CNI
-Etoposide (chemo)
-Anakinra (Toci if IL1 failure or contraindication)
-PLEX
-Ruxolitinib (JAKi)
-Ritux
-Treat virus/bacteria/cancer

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26
Q

** MAS vs sJIA**

A

MAS not JIA:
– Cytopenia
– Hemophagocytosis on biopsy
– HyperTG
– CNS symptoms

– Liver dysfunction
– Hypofibrinogen and ESR
– Prolonged PT/PTT
– Bleeding, purpura

– Resp distress: ARDS

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27
Q

sJIA Tx

A

Not active:
– SJC<4 = NSAID or IA steroids x1mo → antiIL1/6/TNF, abatacept if refractory
– SJC>4 = MTX/LFN x3mo → biologic as above
-
-Mild active (physician global <5):
– NSAID → GC, anakinra if activity >1mo
-
-Moderate Active:
– Anakinra → toci/canakinumab if refractory @ 1mo;
-OR
– GC → Anakinra/Toci/canakinumab if refractory @ 2wks (+MTX/LN if persistent)

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28
Q

** Outcome measure for JIA **

A

Childhood HAQ (CHAQ)
-For longitudinal studies and clinic ax
-JAQQ for prospective studies

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29
Q

** Desirable features of outcome measures for JIA**

A

Practical, easy to use
-Short completion time by child/parent
-Measures social/psychological fcn
-Measures pain
-Reliable, valid, responsive
-Adaptable for international use

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30
Q

Oligoarticular JIA manifestations

A

1-4 joints during 1st 6mo of disease
-Asymmetric, knee >ankle > wrist (RARE hip/back)
-Chronic nongranulamtous anterior uveitis (asymptomatic) in ANA+ and girls <7yo

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31
Q

Oligoarticular JIA subtypes

A

Persistent : never more than 4 joints involved
-Extended: more than 4 joints ater 1st 5 mo

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32
Q

Oligoarticular JIA labs

A

ANA+
-RF-
-Normal WBC
-Mild inflamm markers

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33
Q

Anterior uveitis in JIA
-types and presentation
- complications and screening

A

Acute: Spondyloarthropathy with HLAB27; pain and redness with few complications and not requiring screening

Chronic: Oligoarticular JIA with ANA; ASYMPTOMATIC → cataract, glaumcoma, band keratopathy REQUIRES SLIT LAMP SCREEN

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34
Q

Poorly localized leg pain sufficient to interrupt sleep and cause a limp, must rule out:

A

Leukemia
-Lymphoma
-Neuroblastoma
-Osteosarcoma

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35
Q

Oligoarticular JIA Tx

A

NSAIDs and IA steroids → MTX → TNFi

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36
Q

Polyarticular JIA RF+ vs RF- subgroup differences

A

RF- : insidious/progressive, asymmetric larger joints, TMJ, chronic anterior uveitis, ANA+ (or ANA- symmetrical polyarthritis)

-RF+: faster onset, symmetric small joints (RA deformities), MORE joints, nodules, vasculitis, felty, RA lung dz, ANA+, ACPA+

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37
Q

RF- polyarticular JIA subgroups

A

ANA+ girls <7yo: oligoarticular JIA but more joints in 1st 6mo, high risk chronic uveitis
-ANA- children >7-9yo: RA like (~RF+ subgroup) w/ symmetric polyarthritis

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38
Q

Differentiate RF-/ANA- and RF+ polyarticular JIA from adult RA

A

Peds:
– More hip, shoulder, C spine, DIP than adults
– Fusion of bones, micrognathia, fusion of C spine apophyseal joints

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39
Q

Polyarticular JIA Tx

A

Low dz = NSAID
-Mod-High dz = DMARD (MTX, SSZ, LFN)
-Biologic (TNF, Toci, abatacept, ritux) if refractory, TNFi monotherapy or in combo w/ MTX

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40
Q

Juvenile PsA classsifcation criteria

A

<16yo w/ dz duration > 6 weeks:

-Psoriasis + Arthritis OR
-Psoriasis OR Arthritis with 2 of:

-Dactylitis
-Enthesitis
-Onycholysis or pitting (minimum 2 pits on 1+ nail)
-FamHx Psoriasis

-*excludes: sJIA, RF+, 1st deg fam hx of seroneg/ERA/anterior uveitis, HLAB27+ w/ arthritis >6yo

41
Q

** ERA classification **

A

Child <16yo
-Arthritis (>6wks) AND Enthesitis
-OR
-Arthritis OR Enthesitis + 2 of following

-And 2 of:
-Inflamm back pain
-HLAB27+
-ACUTE anterior uveitis
-1st deg Fam Hx w/ seroneg (PEAR), ERA, uveitis

-*exclusion: personal or fam hx of PsO , positive RF, or known sJIA

42
Q

** What 3 clinical features apart from sacroiliitis and enthesitis would differentiate ERA from seronegative polyarthritis on one hand and from psoriatic arthritis on the other hand?**

A

Seroneg
– IBD
– ??
-
-PsA
– Dactylitis
– Nail pitting
– Psoriasis
– Fam Hx of PsO

43
Q

ERA Tx

A

NSAIDs
-IA GC
-SSZ
-MTX
-TNFi if axial or refractory

44
Q

** Diff between child and adult SLE **

A

Child SLE:
MORE active at presentation & over time
-MORE likely to have ACTIVE renal dz
-MORE intensive drug therapy (HIGHER daily steroid dose)
-MORE severe disease phenotype & damage
-MORE malar rash, chorea

45
Q

Child SLE Tx

A

HCQ (5mg/kg)
-Pred (1-2mg/kg bc higher metab)
-IV methylpred (30mg/kg; max 1g)
-Aza (1-2mg/kg divided BID)
-CYC (1-2mg/kg; Eurolupus 500mg/m2 q2wks x6)
-MMF 1200mg/m2 divided BID (max 1g BID)
-Cyclosporin 6mg/kg divided BID (caution HTN, renal dz)

46
Q

Child SLE Tx indication PLEX

A

Life-threatening/ Tx-resistant
- Refractory nephritis
- DAH
- NPSLE eg transverse myelitis
- TTP
- CAPS
- Cryo

47
Q

Child SLE Tx indication IVIG

A

CAPS
-MAS
-Immune hemolytic anemia/ thombocytopenia

48
Q

Neonatal lupus manifesetations

A

Photosensitive rash (discoid/subacute cutaneous lupus rash)
-Hepatic dysfcn
-Cytopenia (Neut, Plt)
-Heart block (btwn 18-28 wks)
-Fetal hydrops (cardiomyopathy)

49
Q

Neonatal lupus pathophysiology

A

Transplacental passage of maternal anti-Ro/La IgG AB

50
Q

NLE heart block screening

A

Weekly pulsed doppler echo from 16-28 wks

51
Q

NLE heart block Tx

A

1st / 2nd degree or pericardial effusion = fluorinated steroids eg Dex or betamethasone
-3rd deg: pacemaker

52
Q

NLE heart block prevention

A

HCQ at 6wks GA if previous CHB child or positive Ro/LA

53
Q

Sjogren’s DDx in children

A

-Benign recurrent parotid swelling
-Acquired immunodeficiency
-Sarcoid
-IgG4
-TB, Mumps
-Lymphoma

54
Q

MC SVV childhood vs MC vasculitis

A

HSP = MC SVV
-KD = MC vasculitis

55
Q

**HSP manifestations and duration **

A

Purpura, +1 of:
– Colicky abdo pain,
– Arthritis
– GN (proteinuria, RBC casts, hematuria)

– Testicular swelling/edema

-1-4 months
-Recur in 1/3
-1% ESRD (higher in adults)

56
Q

**HSP criteria **

A

Classification
-MANDATORY palpable purpura/petechia WITHOUT thrombocytopenia/coagulopathy, plus at least ONE:
-
– Acute diffuse abdominal pain
– Acute arthritis or arthralgia
– Kidney involvement (any proteinuria, or hematuria) and IgA deposition
– Any biopsy sample showing LCV, or proliferative GN with predominant IgA deposition

57
Q

HSP pathophys

A

After URTI, AAV, Crohns’ = inflamm at mucosal surface → IgA (role in mucosal immunity) aggregates with IgG to form complexes that deposit in tissue → SV LCV

58
Q

HSP labs

A

Elevated serum IgA
-Normal complements
-Proteinuria

59
Q

** HSP Tx **

A

Tx infxn
-Most = supportive care
-Purpura: dapsone
-Arthritis: Tylenol > NSAID if GIB

-Abdo pain or bleeding: prednisone 1mg/kg

-Nephritis:
– GC (Pulsed if proteinuria >1g/d, nephrotic syndrome or >50% crescents) w/ MMF, Ritux, CNI
– ACEi
–+/-PLEX

-Recurrent hospitalizations: B cell depletion (ritux)

60
Q

HSP dx

A

Skin bx shows LCV w/ predominantly IgA vessel wall deposition

61
Q

HSP poor prog features

A

-Melena
-Persistent rash
-Hematuria w/ proteinuria >1g/d
-Nephrosis / renal insuff

62
Q

Enzyme deficiency in childhood PAN

A

DADA2 (adenosine deaminase 2 deficiency)

63
Q

DADA2 manifestations

A

Vasculitis, ie:

-Fever
-Livedo reticularis
-hemorragic/ischemic strokes
-PNS involvement eg neuropathy

-HSM,LN
-immunodeficiency

64
Q

DADA2 labs

A

Hypogammablobulin
-Cytopenias

65
Q

DADA2 pathophys

A

ADA2 = growth factor in endothelial and hematopoeitic cell development,

-Induces monocyte prolif and macrophage differentiation (more M1 proinflammatory macrophages)

66
Q

DADA2 Tx

A

Anti TNF

-GC,AZA, CNI, CYC, MTX, MMF variable success.
-Hematopoeitic stem cell transplant if treatment resistant

67
Q

JDM manifestations

A

Muscle weakness: proximal, palate, swallowing → choking/cough/aspiration
-Rash: heliotrope, gottrons, telangectasia, capillary dropout/dilatation
-Abdo pain, GIB, intestinal vasculitis/bowel perf
-Arthralgias/arthritis
-Decreased DLCO

68
Q

JDM biopsy results

A

Inflammation and/or fiber necrosis
-Perifascicular atrophy
-SVV

69
Q

JDM EMG

A

Spontaneous fibrillation
-Increased insertional activity
-Small muscle unit action potential

70
Q

JDM diff from adult DM/PM

A

PM rare in childhood
-Rare malignancy in children

71
Q

Chronic sequelae JDM

A

NXP2: Subcutaneous Calcinosis
-TIF1: Lipodystrophy → metabolic syndrome, insulin resistance, hirsutism, clitoroemgaly, acanthosis nigricans

72
Q

Child CTD infxn mimicks

A

Acute rheumatic fever
-Parvovirus, EBV
-Humoral/combined immunodeficiency
-Lyme
-HIV

73
Q

Kawasaki Disease (KD) diagnostic criteria

A

-1. Conjunctivitis (bilateral, bulbar, nonexudative)
-2. Oral changes (erythema, fissuring, cracking, bleeding, oropharyngeal erythema w/o exudate, strawberry tongue)
-3. Cervical LN (anterior cervical triangle, unilateral)
-4. Rash (multiforme, diffuse maculopapular, erythoderma, urticarial,
-5. Extremity changes (erythema & desquamation palms/soles)

74
Q

KD investigations

A

Elevated ESR/CRP, WBC >15, Plt >450
-Anemia
-Alb <3
-ALT up
-Urine WBC >10/HPF
-Echo

75
Q

KD organ systems

A

MSK
-Cardio
-Ocular
-Resp
-Gi
-Nervous system
-Skin
-Heme

76
Q

KD MSK manifestations

A

Oligoarthritis

77
Q

KD Cardiac manifestations

A

Myopericarditis
-Aortic root enlargement
-Medium artery aneurysm
-Shock

78
Q

KD Resp manifestations

A

Cough
-Hoarseness

79
Q

KD GI manifestations

A

N/V/D/Abdo pain
-Pancreatitis
-Hepatitis
-Gallbladder hydrops

80
Q

KD GU manifestations

A

Urethritis
-Sterile pyuria

81
Q

KD Ocular manifestations

A

Anterior uveitis

82
Q

KD Skin manifestations

A

Inflamm at previous BCG immunization site
Erythematous mouth and pharynx, strawberry tongue or red, cracked lips
Polymorphous exanthem (morbilliform, maculopapular, or scarlatiniform)
Swelling of hands and feet with erythema of the palms and soles

83
Q

KD Heme manifestations

A

MAS

84
Q

KD Echo findings, what is considered positive

A

-Positive if Z score >2.5 OR
-3 of the following:
– LV dysfunction
– Mitral regurg
– Pericardial effusion
– Z score between 2-2.5

85
Q

KD DDx

A

-Staph/strep toxin mediated dz (scarlet fever, toxic shock)
– Viral: measles, adeno/enterovirus, EBV
– D hypersensitivity: SJS
– Rheum: sJIA
– Other: ricketts, leptospirosis, mercury poison

86
Q

KD Tx

A

IVIG 2g/kg (w/i 10d onset prevents aneurysm)
-+ASA (30-50 or 80-100mg/kg in 4 divided doses)

-ASA→ 3-5mg/kg once fever resolved; discontinue after 6 wks when labs normal

-Refractory = add infliximab, or steroids

87
Q

Refractory KD Tx

A

2nd dose IVIG
-Infliximab (5mg/kg)
-High dose pulsed steroids (20-30mg/kg)
-Anakinra, Cyclosporine, PLEX

88
Q

KD FU

A

No coronary artery lesion:
-Echo @ 1-2wks, 6wks (can DC ASA if normal), and 1 yr after DC
-Then q5y stress test w/ lipid profile

89
Q

** 3 ways in which pediatric patients with APL differ from adult patients with APLAS.**

A

Children
– Higher rates of LAC and anticardiolipin IgM
– More peripheral DVT and brain clots (CVST, stroke) bc Higher assoc w/ SLE
– Less RF like DLPD, HTN, smoking, E2
– CAPS as 1st presentation is more common
– More likely infxn as trigger
– Avoid ASA given risk of bleeding and Reyes syndrome

90
Q

**14-year-old boy presents with a 2 week history of fever, weight loss, ankle swelling, tender nodules on calves, and a cough.
-What is the most likely diagnosis?
-List 4 tests to confirm your diagnosis.
-

A

Sarcoid
-CBC, CXR, ESR/CRP, ANA, Blood Cx, ASOT, Throat culture, Biopsy, Ca

91
Q

** List 6 other diagnoses for hypermobility?**

A

-EDS (classic, hypermobile, vascular, kyphoscoliosis, arthrochalasia, dermatosparaxis types)
–Loeys-Dietz Syndrome (Aortic aneurysm with arterial tortuosity, hypertelorism, bifid uvula or cleft palate)
–Marfan syndrome (marfanoid habitus, arachnodactyly, arm span/heigh ratio >1.05, ectopia lentis, aortic dilatation or aneurysm, pectus)]\
–Osteogenesis imperfecta (blue sclera, bone fragility, SNHL) – can have overlap with EDS
–Downs
–Turners

–Arterial Tortuosity Syndrome (tortuosity and elongation of large and medium-size steries with propensity towards aneurysm formation, soft/velvety/hyperextensible skin, dysmorphic facial features, abdominal hernias)
–Lateral Meningocele Syndrome (widespread spinal lateral meningoceles, facial dysmorphism, MSK pain)

-

92
Q

**A 6 year old boy is referred to you because of hepatosplenomegaly and lymphadenopathy. He has had these present since age 3 years. He has had splenectomy for persistent thrombocytopenia. Several female family members have had splenectomies.
-a) What is the diagnosis: **

A

ALPS – autoimmune lymphoproliferative syndrome (Canale–Smith syndrome) -
-Lymphadenopathy,
-Hepatosplenomegaly,
-Autoimmune hemolytic anemia
-Thrombocytopenia

93
Q

**A 6 year old boy is referred to you because of hepatosplenomegaly and lymphadenopathy. He has had these present since age 3 years. He has had splenectomy for persistent thrombocytopenia. Several female family members have had splenectomies.
-b) Name 3 characteristic genetic features **

A

Fas germline (autosomal dominant) or somatic mutation
-Fas ligand germline mutation
-Caspase germline mutation
-

94
Q

**A 6 year old boy is referred to you because of hepatosplenomegaly and lymphadenopathy. He has had these present since age 3 years. He has had splenectomy for persistent thrombocytopenia. Several female family members have had splenectomies.
-c) Name 3 clinical features apart from thrombocytopenia and mechanism for each **

A

Lymphadenopathy and HSM: lymphoid cell proliferation due to Fas abnormality.

-Autoimmune hemolytic anemia and neutropenia: (+DAT )

-HL, NHL: overactive lymphoid cells.

95
Q

Gaucher clinical features

A

Lysosomal storage disease causing infiltration and accumulation of lipid laden macrophages in bone, lung, organs →
-AVN
-ILD
-HSM

96
Q

**What is the differential diagnosis of Stroke in young patient **

A

SLE, APS
-Takayasu’s arteritis, PACNS, PAN, GPA

-TB, syphilis, VZV, fungal, meningitis
-IE, PFO

-Sickle cell, Prot C/S deficiency, Factor V leiden

-Moya moya, FMD, Dissection (EDS, Marfan)

-CADASIL, DADA2

97
Q

**Marfan clinical characteristics **

A

-Lens dislocation, Myopia
-Aortic root dilatation, MV prolapse
-Pectus carinatum/excavatum
-Pneumothorax

-Wrist/thumb sign
-Hindfoot deformity
-Protrusio acetabuli
-Scoliosis, thoracolumbar kyphoscoliosis

-Skin striae

98
Q

** 6 systemic physical signs of Marfan syndrome?**

A

Tall, long extremities
-Lower upper/lower body ratio
-Low fat
-Pectus carinatum/excavatum
-Scoliosis
-Pes planus (flat feet
-Long narrow face
-Retrognathia
-Striae

1. Rheum RC (23 decks)

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