JANUARY 7, 2019

KATIE MAILEY, PGY2

Morning Report
9 year old girl presents to PCH ED from her pediatrician for
facial swelling, fatigue, and pallor.

Case Presentation
 History
HPI Past Medical History
• 3-4 days swollen cheeks, took Benadryl didn’t help • Type 1 Diabetes
• Fatigue and pallor for 2 weeks Home Meds
• Glucoses have been high, 300-400s • Lantus 13 QHS, Novolog CR 1:10, CF 1: 50/150/200
• Abdominal pain, nausea/vomiting for last 3 days Family History
• No polyuria, polydipsia. Reports decreased appetite • Father with Hodgkin Lymphoma
• Some “brown” urine, urinating less frequently • Mother, Maternal Grandmother, Maternal Aunt- Type 1 diabetes
• Headache for 2 weeks • Maternal grandmother with thyroid disease
• couple of nose bleeds in last 2 weeks • Paternal Grandfather: Rheumatoid arthritis
• cough, congestion, rhinorrhea • Paternal Aunt- MS
• fever last week, 101.7 F. Dx with AOM started on Amoxicillin Social History
• no recent sore throat • Dogs in house. Lives on land, neighboring farm with sheep and
livestock
• Presented to PCP – Hgb 4.3. Negative strep test
Physical Exam
T: 37.7 C, HR 136, RR: 36, BP 142/102, SpO2 93%, Wt 30 Kg
General: Ill appearing, alert, oriented, pale
HEENT: +facial and periorbital edema, PERRL, EOMI, no cervical LAD, normal
thyroid
Cardiac: tachycardic, regular rhythm, normal S1, S2, strong symmetric
peripheral pulses. +pedal non pitting edema
Respiratory: good air movement, symmetric, no adventitious sounds, no
retractions, no distress
GI: distended, soft, mild TTP, tympanic to percussion, no fluid wave, normal
bowel sounds
Neuro: cranial nerves intact, normal strength, tone, normal gait.
Skin: no rash, no petechiae
 Initial Labs
Cap Gas: Urinalysis
7.41/25/33/15.7/8 Color: normal, Specific Gravity: 1.015, pH: 6
CBC Glucose > 1000, 3+, Ketones: negative
WBC: 13.7 (Diff: 73% segs, 22% lymphs, 1% eos, 3% Nitrite: Negative, Leuk esterase: negative, Bacteria negative
monos, 1% baso)
Hgb: Large, Protein: >300, 3+
Hgb: 4.2 , Hct 13.7
WBC: 8, RBC: > 30
Plts 411
+Granular cast
CMP:
Inflammatory Markers
Na: 139, K 4.8, Cl 105, CO2 16, Glu 327
ESR 136 , CRP 7.4
BUN 85, Cr 5. 48, Ca 8.3, Total protein 6.5, Albumin
2.9, Tbili 0.5, Alk Phos 95, AST 13, ALT 6
Differential
 9 yo girl with T1DM presents with anemia, edema, proteinuria,
hematuria, hypertension and acute kidney injury.
Cardiac Heme/Onc Rheum
• Heart failure •Hemolytic anemia • Vasculitis ( Granulomatosis
• Endocarditis •Intrinsic anemia (sickle cell) with polyangitis, microscopic
polyangitis, IgA vasculitis,
GI •Leukemia, Lymphoma Kawasaki, PAN)
• IBD
•Abdominal mass: • Good pastures syndrome (Anti
Renal neuroblastoma GBM)
•PSGN Infectious •Antiphospholipid syndrome
• IgA nephropathy •bacteremia (Meningococcal,
GAS) •Lupus
•C3 glomerulonepathies
•Viral ( Hepatitis, Parvo B19, HIV) Endo
• SLE nephritis
•TB DKA
• HUS
•MGPN
More work-up
•Reticulocyte %: 1.99% •ANA IgG – Detected 1:80
•Retic, Abs: 34 K/mcl •C3 153 (High)
•PT 13.6 seconds, INR 1 •C4 25 (normal)
•PTT 35 seconds •dsDNA – Negative
•LDH 273 u/L (normal)
•Haptoglobin 359 mg/dL (high)
•Peripheral smear: hypochromic, normocytic
RBCs
At sign out you get a page
But actually:
FYI Patient with increased WOB, now on 4 L NC
Imaging
• Chest Xray
• Diffuse reticulonodular pulmonary opacities are
scattered throughout the lung with most
confluent area of opacification being the lingual
and left lower lobe
• No pneumothorax, no pleural effusion
• Cardiac silhouette is mildly enlarged
• Impression: opacities most likely represent
mixed interstitial and alveolar pulmonary edema
•Echo
• Left ventricular hypertrophy
• Mildly dilated left atrium
• Normal systolic function
•CT Chest w/o contrast
• Extensive patchy airspace opacities bilaterally, L
> R. Alveolar hemorrhage or pneumonia could
have a similar appearance. Clinical correlation
recommended.
• Tiny bilateral pleural effusions
• Abnormal enlarged low-attenuation kidneys,
suggesting renal edema
•CT Sinus w/o contrast
• No abnormality noted
Bronchoscopy
Trachea normal
Carina normal, sharp
Right main stem bronchus: normal
Left main stem bronchus: abnormal, blood
seen oozing out. Did not proceed
Secretions: normal amount, thin, clear
Airway mucosa: healthy appearing on R
Left Renal Biopsy
Pauci-immune crescentic glomerulonephritis
Light microscopy
• 18/19 glomeruli show crescents
• Interstitium is edematous, mild fibrosis
• Tubules with red cell, granular, and protein casts
Immunofluorescence
• No staining for IgA, IgG, or C3
• Only Trap-like staining with IgM and C1q
Electron Microscopy
• Segmental effacement of podocyte foot processes
Work-up
continued:
•ANCA panel
• ANCA IgG 1:160
• MPO Ab 110 AU/ml (High)
• PR3 AB 4 AU/ml

•Antiphospholipid panel
(anticardiolipin IgG/IgM, anti-
beta2 glycoprotein IgG/IgM, lupus
anticoagulant): Negative
MPO-ANCA Vasculitis:
Microscopic polyangiitis
FINAL DIAGNOSIS
 Pediatric Vasculitis
Primary Causes
• Incidence: 12-53 cases per 100,000
• IgA Vasculitis
•Primary v. Secondary Process • Kawasaki disease
• Secondary causes:
• Polyarteritis nodosa (small/medium artery)
• Infectious: Parvo, Hepatitis, HIV, varicella, • Skin involvement, myalgias, HTN, peripheral neuropathy, renal
rickettsia involvement
• Medication: anti-thyroid, TNF inhibitors
• Takayasu arteritis (large vessel)
• systemic disease: SLE, JIA, • Affects aorta and major branches
dermatomyositis, IBD, sarcoidosis
• ANCA
•Varied symptoms based on type and • GPA
body system involvement • Microscopic polyangiitis
• Churg strauss syndrome (eosinophilic GPA)
•Classification criteria • Asthma and eosinophilia
• rarely do children fill these criteria
• Bechet syndrome
Vasculitis in children: Evaluation overview
visit UpToDate
Due to copyright restriction images have been removed
Pauci-immune ANCA vasculitis
MICROSCOPIC POLYANGIITIS
•Incidence less than GPA
•No granuloma formation
•Lower respiratory tract – usually pulmonary
capillaries  hemorrhage
•Renal – extensive glomerular
•pANCA (perinuclear), anti-myeloperoxidase
(MPO) antibodies
GRANULOMATOSIS WITH POLYANGIITIS
(FORMERLY WEGENER’S) AKA GPA
•Incidence in kids: < 1/1,000,000
•Granulomatous inflammation of small and
medium sized arteries
•Upper and Lower Respiratory tract
•Laryngotracheobronchial stenosis
•Renal Involvement
•cANCA (cytoplasmic), anti- proteinase 3
antibodies (PR3)
 •Differentiating vasculitis from other diseases that can either
mimic vasculitis or cause secondary vasculitis
•Defining disease activity and severity
• Induction
Management • Combination of glucocorticoids or cyclophosphamide/rituximab
• Methotrexate
Goals • Maintenance
• Glucocorticoids +
• azathioprine (AZA), rituximab, methotrexate or mycophenolate mofetil.
• Relapse

•Weighing the benefits and risks of therapy

 Management
Vasculitis AKI
• Fluid restriction
1. High dose pulse steroids (5 days total) • Renal diet + phosphate binders
30 mg/kg IV methlypred x 3days
• Diuretics
15 mg/kg x1 day
• Avoided dialysis
7.5 mg/kg x1 day
2. Maintenance steroids
2 mg/kg/day divided BID maintenance HTN
3. Plasmapheresis Nifedipine, amlodipine, labetalol, Lisinopril
5 days total (QOD schedule)
4. Rituximab
350 mg/m2 (weekly x 4 doses)
5. Azathioprine 50 mg po daily