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MORNING REPORT
Aaron McCoy, MD, PGY3
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HPI
Otherwise healthy 12 y.o. that presented to Nephrology clinic
with microscopic hematuria.
Around 5 mo. prior to referral to clinic he developed fever and
pharyngitis.
This was felt to be a viral syndrome but per mom, he never
bounced back.
A couple months later, initial work up showed positive IgG, IgM
for CMV and mild elevation in
transaminases.
Around that time, he was also noted to have some iron deficiency
with serum iron level of 15 and5% saturation. No definitive
diagnosis was made.
A few weeks later, he presented with acute appendicitis and was
admitted for treatment.
At that time, he was first noted to have hematuria but was
discharged without diagnosis. Post-
discharge, after otherwise typical appendicitis treatment, he
continued to have intermittent fevers
resulting in an abdominal CT to look for post-op infections.
This was unremarkable.
A few weeks later, he developed bloody diarrhea and was treated
for C Diff colitis- lab work wasnegative for C Diff toxin.
However, after treatment, he continued to have low grade fevers
in the 100-101 range, and
recently developed poor appetite, intermittent severe wrist and
neck pain, persistent microscopic
hematuria, and facial and arm rash (resolved shortly
thereafter), bilateral eye erythema and
swelling (also resolved), weight loss (about 1 kg), and anemia
(HCT 29.6).
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HPI cont.
PMH: Refractive error- eye glasses;Tonsilectomy 2007
FH: 20 yo sister with SLE, MGF c melanoma,
no family hx of other autoimmune orrheumatological
conditions
SH: Lives in with his parents. They have a cat,dog and lizard at
home. He attends 7th grade.
There is no recent travel or unusual exposures Medications: PRN
Tylenol and Ibuprofen
Allergies: None
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Physical Exam
Vitals: T 37.0 HR 103 RR 20 BP 103/67
HT 153cm WT 35.7kg
General: Thin and mildly ill appearing, no acute distress
Extremities: No clubbing, cyanosis, edema, joints with normal
ROM andnon-tender
HEENT: Normocephalic, EOMI, PERRLA, clear conjunctiva and
sclerae, nooropharyngeal lesions, neck with shotty posterior
cervical LAD
CV: RRR, PMI normal, no murmur, no gallop, pulses and perfusion
normaland symmetric
Resp: CTAB
Abd: Soft, non-tender, no masses or organomegally, no ascites,
normal
bowel sounds, small well healed surgical incisions x 3 Skin: No
rash
Neuro: Normal cranial nerves, motor, sensory, and reflexes
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Imaging/Labs
POC UA: Moderate Hgb with numerous RBC on microscopy
ANA IgG: positive (Titer 1:160)
RF: 10
C3: 60 (L)
C4: 4 (L)
Smith (ENA) Ab: 5SSA (Ro) Ab: 4
SSB (La) Ab: 0
DNAse B Ab: 271 (H)
Cardiolipin Ab IgG 17 (H), IgM 14 (H)
dsDNA IgG: positive (Titer 1:2560)
CBC: WBC 3.1, Hgb 11.6, HCT 35.7, MCV 76.4, AbsL 1.2 (L)CMP: WNL
except Alk Phos 112
CRP 0.7
ESR 75
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SLE
+ ANA, + dsDNA and anticardiolipin
antibodies, lymphopenia, malar rash and
arthritis
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American College of Rheumatology Criteria
Need
4 of 11
Criteria
1. Malar rash
2. Discoid rash
3. Photosensitivity
4. Oral Ulcers
5. Arthritis- 2 or more joints6. Serositis- pleuritis or
pericarditis
7. Renal disorder- persistent proteinuria or cellular casts
8. Neurological disorder- seizures or psychosis
9. Hematolgical disorder- hemolytic anemia,
leukopenia,lymphopenia, or thrombocytopenia
10. Immunological disorder- anti-DNA, anti-Smith, falsepositive
syphilis test, positive Lupus Erythematous cellprep (neutrophil or
macrophage phagocytosingdenatured cell material)
11. ANA +
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SLE in children/adolescents
- Usually more severe than adult SLE
- 20% of SLE cases are dx during first 2
decades
- Average age of onset in Pediatric SLE is 12
- Pre-puberty M:F ratio is 1:3, after puberty it is
1:9
- Incidence is much higher in non-caucasians
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Presentation Clinical
Common signs/symptoms- fever, weight loss, LAD, and HSM
More specific findings are malar rash and arthritis
Rash- malar, discoid, vasculitic (palmar erythema, tender skin
nodules, purpura,ulcerations), Raynaud phenomenon, nail fold
capillary changes, livedo reticularis,alopecia
Musculoskeletal- Non-erosive arthritis, arthralgia, Jacoud
arthropathy (ulnar deviation
of 2-5 fingers and subluxation of metacarpophalangeal joints),
myalgia, myositis Renal- HTN
Neuropsych- decreased concentration, cognitive dysfunction,
psychsosi, seizures,transverse myelitis, CNS vasculitis, stroke
Pulmunary- pleuritis, pneumonitis, pleural effusion, hemorrhage,
pulmonary HTN,restrictive lung disease
Cardiac- pericarditis, pericardial effusion, myocarditis,
endocarditis (bacterial v non-bacterial valvitis),
atherosclerosis
GI- pancreatitis, enteritis, vasculitis, functional asplenia
Endocrine- Hypothyroidism
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Malar Rash
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Discoid Rash
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Nail Capillary Changes
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Livedo Reticularis
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Jacoud Arthropathy
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Presentation Lab
Renal-proteinuria, microscopic hematuria, elevatedBUN or Cr
level, immune complex deposits lead tolow C3, C4
Hematologic- leukopenia (lymphopenia), anemia
(hemolytic is classic, anemia of chronic
disease),thrombocytopenia, anticardiolipin ab, antiphospholipidab,
lupus anticoagulant ab, prolonged PTT, elevatedESR, normal CRP
FEN/GI- transaminitis, hypoalbuminemia, elevated Cr Rheum- ANA,
anti-dsDNA (very specific), anti-Smith
(very specific), anti-ribonucleoprotein, anti-Ro, anti-La,
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Neonatal Lupus Erythematous
1% of infants who experience transplacentalpassage of maternal
SSA or SSB Abs
Can present with rash (erythematous, raisedborder, prominent on
sun exposed areas and
around eyes), cytopenia, hepatitis, hepatomegalyBUT worry about
congenital heart block from Abmediated damage to conducting
system
Start evaluating for bradycardia at 16weeks inmothers with SLE
or known SSA or SSB
Sx other than heart block will self-resolve around6 mo age with
dissipation ofAbs. Heart block isoften permanent and requires
pacemaker.
