Pediatric Rheumatologic Disorders

Question 1

Juvenile Dermatomyositis (JDMS)

Answer 1

Bimodal age of onset in kids: 3-7y/o & Early teens.

HALLMARKS: Rash with proximal & muscle weakness

Question 2

Pathophysiology of Juvenile Dermatomyositis (JDMS)

Answer 2

xx

Question 3

Clinical Manifestations of Juvenile Dermatomyositis (JDMS)

Answer 3

HALLMARKS: Rash with proximal & muscle weakness

Question 4

Dx of Juvenile Dermatomyositis (JDMS)

Answer 4

xx

Question 5

Labs/Tests for Juvenile Dermatomyositis (JDMS)

Answer 5

Calcinosis and Myositis on MRI;

Increased Muscle Enzymes: (CPK, AST/ALT, LDH, Aldolase)
Increased Inflammatory Markers: ESR
Auto-AB: [ANA may be + (low titer)]; RF negative (even if arthritis)
Other Labs: NORMAL (CBC, U/A (no myogobin or Ca)
CRP, Calcium(even if calcinosis), Other LFTs&Coags)

Question 6

Management for Juvenile Dermatomyositis (JDMS)

Answer 6

Steroids, IVIG, Methotrexate, Biologics, PT/OT, Sunscreen/protection

Question 7

Skin Findings in JDMS

Answer 7

Heliotrope (purple eyelids); Gottrons (papules on knuckles, elbows, knees); Shawl sign (erythroderma in V-neck distribution); Calcinosis (late complication); nailbed telangectasias (vasculitis)

Question 8

MSK Findings in JDMS

Answer 8

Proximal Muscle Weakness: Difficulty: Getting OOB/chair, Lifting neck, Lifting legs, Raise arms above head, Sitting up, Stairs, Crossing legs…………………Trendelenburg gait
(waddle when walk); Gower’s sign- Use hands to get up off floor; Difficulty swallowing (Dysphagia, Odynophagia); Arthritis.

Question 9

Pediatric SLE

Answer 9

20% of SLE in diagnosed in kids < 18y/o; Rare before 5y/o; Commonly present w/ puberty or pregnancy [F>M (4:1)]; More common in: AA, Asians, Hispanics

Question 10

Typical Scenario of Pediatric SLE

Answer 10

Ethnic female teenager with mutlisytem c/o: fever, fatigue, weight loss, hair loss (constitutional); joint symptoms; butterfly rash……..Other: seizure, abn U/A, pleurisy, coomb’s + hemolytic anemia

Question 11

1997 ACR Classification Criteria of Pediatric SLE

Answer 11

4 skin: Malar rash, Discoid rash, Photosensitivity, Oral/nasal ulcers………………………3 lab: Cytopenias, Serologies, Antinuclear antibody…………………………“4 –itis”: Arthritis, Serositis, Nephritis, Cerebritis/CNS disease…………………..4/11 criteria: 96% sens/spec for Dx of SLE

Question 12

Laboratory Findings for Pediatric SLE

Answer 12

AutoAB: ANA almost always +, Anti-ds-DNA + 60-70% (Pathognomonic& reflects renal dz), Anti-smith + 30% (specific)………APLA common: PT/PTT b/c of LAC & thrombocytopenia, Associated w/ miscarriages, clots, &raynauds (whitebluered color change)……..Anti-histoneAb: Drug induced lupus (Anticonvulsants) ……. Hypocomplementemia (C3, C4) …… Cytopenias: Coombs+ AIHA, Thrombocytopenia (ITP), Leukopenia and lymphopenia……….Inflammatory markers: ESR high w/ flare, CRP normal (rise w/infection)

Question 13

Clinical Manifestations of Pediatric SLE

Answer 13

Constitutional: Fever, weight loss, lethargy………..Skin: 70-80% (Malar, Discoid, Photosensitive, Oral ulcers, Alopecia) ….. Arhtitis: 80-90% : (NON-erosive, Polyarticular (sm&lg)) …….. Renal: 50-60% : (HTN, Abn U/A w/casts ->Bx crucial; If DPGN -> steroids/CP) ………. CNS: 10-30% : (SZ common, Psychosis (10-20%),CN>Peripheral neuropathy, Retinal vasculitis, Headache, Mood disorders, Poor school performance) ………. Cardiac: 25-30% : (Pericarditis, Myocarditis, Libman sacks (sterile) endocarditis, APLA associated)

Question 14

Malar Rash with Pediatric SLE:

Answer 14

“Butterfly” : Spares N/L folds, Crosses nasal Bridge

Question 15

Discoid Rash with Pediatric SLE:

Answer 15

Circular & Raised

Question 16

Nasal and Oral Ulcers with Pediatric SLE

Answer 16

Nasal and Oral ulcers: Usually painless; Location: HARD palate & nasal SEPTUM

Question 17

Treatment of Pediatric SLE:

Answer 17

Treatment: Immunosuppression [[Steroids, Steroid-sparing agents:(Plaquenil (skin), Immuran (cytopenias), Methotrexate (joints), Cellcept(Nephritis),Cytoxan(Nephritis, carditis, Cerebritis)]]

Question 18

S/E of steroids:

Answer 18

AVN *** (groin pain), Osteoporosis, Growth failure, Glaucoma/cataracts, DM, HTN, Accelerated atherosclerosis, Infection

Question 19

Neonatal Lupus:

Answer 19

Syndrome in neonates: Skin rash, 3rd ° CHB (destroys AVN) -> requires pacing, Liver disease, Cytopenias ………… Due to maternal auto-ab: Ro and La (SSA/SSB), attack fetal heart tissue)

Question 20

Common Presentations of Neonatal Lupus

Answer 20

phototherapy->rash; bradycardia from CHB ->mistaken as fetal distress during labor

Question 21

Treatment Principles of Rheumatic Disease

Answer 21

Medical: NSAIDS (PMD can start); Steroids: (IA, PO, IV); DMARDS: Disease-Modifying Anti-Rheumatic Drug (Methotrexate & Cyclosporine (MAS)); Cytotoxic-cytoxan, Biologics…………….Non-Medical: Treating extra-articular findings (skin, GI, Eyes): Rehab (PT/OT, Splinting, Pool therapy), Optho screening, Counseling, Pain management

Question 22

“Biologic agents” Tx of Rheumatic Disease

Answer 22

Drugs which mimic substances already present in the body: Antibody-like molecules (monoclonal antibodies, fusion proteins (receptor + IgG-Fc), receptor antagonists); “Designer drugs” -> Very costly

Question 23

Functions of Biologic Agents

Answer 23

Block different steps of the immune signaling and inflammatory response………… Some Meds Block Downstream- “like an umbrella in the rain (cytokine storm)”………. Some Meds Block Upstream- “decrease the storm clouds”

Question 24

How do Treatments Vary

Answer 24

Treatments will vary “depending on the storm” ; …which is difficult to predict and control, everyone has their own “weather pattern” and response……………..Combination therapies make sense

Question 25

Henoch-SchonleinPurpura (HSP): Clinical Features

Answer 25

Most common vasculitis in kids with a mean age 4y/o (3/4 < 7 y/o); M: F (2:1).

Question 26

Pathophysiology of HSP

Answer 26

Seasonal: winter and spring. ½ with preceding URI; Possible other triggers: GABHS, mycoplasma, MMR, cephalosporins, PNC, insect bites; IgA mediated

Question 27

HSP Physical Features

Answer 27

``` Skin Lesions (100%): ½ = presenting finding LE/buttocks [Younger kids (face)], Palpable purpura (May also see: Petechiae, Macular-papular).........Lasts 1 to 4 wks ```

Question 28

Other Physical Features of HSP: Joints

Answer 28

2nd most common; ¼ get arthritis 1st, Very tender | Peri-articular swelling, edema around joint (Knees, ankles)

Question 29

Other Physical Features of HSP: GI

Answer 29

3rd most common; Abdominal pain with N,V, Occult blood common (1/3 melena), Thickened GB (hydrops), IC intuscussecption (up to 15%), (Intermittent fatigue w/ abd pain, Currant jelly stools)

Question 30

Other Physical Features of HSP: Renal

Answer 30

Renal: 10-50%; Can be mild, transient or chronic. Isolated microscopic hematuria/proteinuria. May occur up to 3 months after onset.

Question 31

Other Physical Features of HSP: GU

Answer 31

Orchitis: Can be hallmark of presentation

Question 32

Other Physical Features of HSP: Pulmonary

Answer 32

Pulmonary hemorrhage

Question 33

HSP Labs and Tests

Answer 33

LABS: Increased WBC, ESR and IgA. NORMAL platelets, coags!!! ........ Other: Hydrops of GB, Intussuception

Question 34

HSP Treatment

Answer 34

Supportive: (No data to support steroids: Does not prevent nephritis but may be used for symptoms (GI or MSK)); Most are self-limited (w/in 4wks)............ Recurrences are common! (40%): Extremes of age, W/in 6 wks to 2 years after initial episode, Usually milder, Provide reassurance

Question 35

Kawasaki Disease (KD)

Answer 35

Typical Clinical Scenario: 15 m/o infant male with prolonged fever for >7 days associated with irritability, rash, and diffuse swelling of hands..........1st day of fever=1st day of illness

Question 36

PE of Kawasaki Disease (KD)

Answer 36

Unilateral cervical LAD, B/L conjunctivitis, Cracked and dry lips

Question 37

Dx of Kawasaki Disease (KD)

Answer 37

NO DIAGNOSTIC TEST EXISTS; DX based on recognition of the clinical features-> Diagnostic Criteria: ≥ 5 days of fever(High-spiking (≥104) and remittent) (MANDATORY), Plus 4 of the following 5 Signs: [Mucous membrane changes (90%), Bilateral conjunctival injection (85%), Rash (80%), Changes of Extremities (70%), Cervical Lymphadenopathy (70%)

Question 38

Mucous Membrane Changes of Kawasaki Disease (KD)

Answer 38

Red strawberry-like tongue & Red, dry, cracked lips

Question 39

What is not observed in the Mucous Membranes with KD?

Answer 39

[Not Observed: Oral ulcers, Exudates, Koplik spots]

Question 40

Eye problems with KD

Answer 40

Bulbar Conjunctival Injection: May develop anterior uveitis. Spares the Limbus and is non-exudative.

Question 41

Rash with KD

Answer 41

Most common: Maculopapular, Erythematous, Morbiliform ....... Desquamation of the groin (50%), Acute phase; Not seen: Vesicles, Bullae.

Question 42

Changes in the extremities with KD

Answer 42

Acute Phase: Abrupt erythema (confined to palms and soles), Swelling......... Subacute Phase: Desquamation

Question 43

LN Changes with KD

Answer 43

Unilateral Cervical Lymphadenopathy: ≥ 1.5cm in diameter, non-fluctuant

Question 44

KD: Associated Features

Answer 44

``` Cardiac Involvement (25%), Extreme irritability (90%), Sterile Pyuria (60%), Mild Hepatic Dysfunction (40%), Hydropsof gallbladder (10%), Arthralgias and arthritis (30%), Aseptic meningitis (25%), Diarrhea & Abdominal Pain (20%), Pneumonitis ```

Question 45

KD Laboratory Findings

Answer 45

Acute Phase: Inflammatory state (Elevated ESR, CRP, WBC), Anemia ................ Subacute phase:Thrombocytosis (>1,000K) in 2nd or 3rd week; Greatest risk of sudden death ........... Convalescent phase: Persistence of elevated acute phase reactants for weeks

Question 46

Atypical "Incomplete" KD

Answer 46

Fever and <4 criteria, Diagnosis based on coronary abnormalities. More common in young infants (especially males): -More severe illness, -Increased Cardiac abnormalities, -High mortality, -Dx often missed

Question 47

Risk Factors for CAA

Answer 47

1. Fever: Persistent, Recurrent 2. Laboratory: Low platelet count, CRP 3. Demographic:Age < 1 year (½ atypical KD), Male gender 4. Other:Late administration of treatment

Question 48

KD Tx

Answer 48

1. Conventional Therapy: Aspirin (ASA), High Dose: Anti-inflammatory effect, Low Dose: Anti-thrombotic effect; 2. Intravenous Immunoglobulin (IVIG): Reduces fever, Reduces risk of CAA (if given within 10 days), Repeat if fever recurs. 3. Other (if resistant to multiple courses IVIG): Higher prevalence of CAA, No guidelines for treatment. Some studies suggest benefit of steroids.

Question 49

Hypermobility Syndrome

Answer 49

Common in kids (15%); Primarily females .........If found, r/o inherited dz (Marfan/Ehlers-Danlos- (less common)) ........[If pain associated: Rx: PT (swimming), orthodics (flat feet), NSAIDS]

Question 50

Growing Pains

Answer 50

Most Common cause of joint paint in kids; Benign (growth or weight not affected), Bilateral (If not think of something else), Deep ache, Not articular (thigh or calf), No AM stiffness, no limping, does not interfere w/ADLs, Nighttime: wakes up ........... Massage or tylenol helps ........... Rx: Reassurance, Disappear by teenage years.

Question 51

Reactive Arthritis

Answer 51

NOT SEPTIC JOINT!!! Arthritis = 2° IR to an infection elsewhere in the body: GI: (Yesinia, Salmonella, Campylobacter) GU: (Chlamydia) dysuria URI/LRT: (Mycoplasma)

Question 52

Reactive Arthritis Clinical Scenario

Answer 52

Arthritis occurs 1-4 weeks after infection; Lasts 4-8 weeks (transient; NOT chronic) .......... Other Associations: Conjunctivitis, Skin (oral/GU ulcers) or rash, Fever