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Aaron McCoy, MD, PGY3


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 and 5% 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. Postdischarge, 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 was negative 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).

HPI cont.

PMH: Refractive error- eye glasses; Tonsilectomy 2007 FH: 20 yo sister with SLE, MGF c melanoma, no family hx of other autoimmune or rheumatological 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

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 and non-tender HEENT: Normocephalic, EOMI, PERRLA, clear conjunctiva and sclerae, no oropharyngeal lesions, neck with shotty posterior cervical LAD CV: RRR, PMI normal, no murmur, no gallop, pulses and perfusion normal and 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

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: 5 SSA (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


+ ANA, + dsDNA and anticardiolipin antibodies, lymphopenia, malar rash and arthritis

American College of Rheumatology Criteria


Need 4 of 11 Criteria

2. 3. 4. 5. 6.

8. 9.



Malar rash Discoid rash Photosensitivity Oral Ulcers Arthritis- 2 or more joints Serositis- pleuritis or pericarditis Renal disorder- persistent proteinuria or cellular casts Neurological disorder- seizures or psychosis Hematolgical disorder- hemolytic anemia, leukopenia, lymphopenia, or thrombocytopenia Immunological disorder- anti-DNA, anti-Smith, false positive syphilis test, positive Lupus Erythematous cell prep (neutrophil or macrophage phagocytosing denatured cell material) ANA +

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

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 nonbacterial valvitis), atherosclerosis GI- pancreatitis, enteritis, vasculitis, functional asplenia Endocrine- Hypothyroidism

Malar Rash

Discoid Rash

Nail Capillary Changes

Livedo Reticularis

Jacoud Arthropathy

Presentation Lab

Renal-proteinuria, microscopic hematuria, elevated BUN or Cr level, immune complex deposits lead to low C3, C4 Hematologic- leukopenia (lymphopenia), anemia (hemolytic is classic, anemia of chronic disease), thrombocytopenia, anticardiolipin ab, antiphospholipid ab, lupus anticoagulant ab, prolonged PTT, elevated ESR, normal CRP FEN/GI- transaminitis, hypoalbuminemia, elevated Cr Rheum- ANA, anti-dsDNA (very specific), anti-Smith (very specific), anti-ribonucleoprotein, anti-Ro, anti-La,

Neonatal Lupus Erythematous

1% of infants who experience transplacental passage of maternal SSA or SSB Abs Can present with rash (erythematous, raised border, prominent on sun exposed areas and around eyes), cytopenia, hepatitis, hepatomegaly BUT worry about congenital heart block from Ab mediated damage to conducting system Start evaluating for bradycardia at 16weeks in mothers with SLE or known SSA or SSB Sx other than heart block will self-resolve around 6 mo age with dissipation of Abs. Heart block is often permanent and requires pacemaker.