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Morning Report

Morning Report . Lorrie Edwards 8-5-2013. The Case. “I have this rash on my legs”. What do you want to know?. History. JV is an 18yo female who presents with a rash on her lower extremities for 4 days

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Morning Report

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  1. Morning Report Lorrie Edwards 8-5-2013

  2. The Case “I have this rash on my legs”

  3. What do you want to know?

  4. History • JV is an 18yo female who presents with a rash on her lower extremities for 4 days • The rash is dark red and raised. It does not hurt or itch. It appeared suddenly and has not gotten better or worse. • She has also had pain in both hands, knees, and feet which started about 6 days ago. She feels that they are very swollen. She has been having trouble opening jars and cartons. She has taken motrin, which has given her moderate relief. • She feels like her eyes are also swollen, but only when she wakes up. • For the past 2 days she has had a bitemporal headache which is “cramping” in nature. • ROS: + fatigue, no weight loss, no fever. + cough for 2 days, no SOB. + mild nausea, no V/D/C. No sore throat. No GU symptoms. No numbness, tingling, or change in speech or mental status. LMP 10 days ago. • She has a PMH of migraines, for which she started taking verapamil 2 months ago. She had an MRI of the brain at that time which was unremarkable. • She has no significant family history.

  5. What do you want to do next?

  6. Physical Exam • VS: T99.6, P105, R19, BP147/112, SaO2100% • Gen: NAD • HEENT: NCAT, PERRL, OP clear, no oral ulcers • Neck: Supple, no adenopathy • Cardiac: RRR, S1/S2, no murmur • Respiratory: Good aeration, mild crackles on Left • Abdomen: Soft, NTND, +BS, no HSM • Ext: Swelling noted at BL hands and feet, especially in DIP and PIP joints. No associated erythema. • Skin: palpable, non-blanching purpuric rash over bilateral anterior lower extremities • Neuro: grossly intact

  7. Anything else? • UA: 1.020, pH 5.5, Prot >1000, large blood, no bili, ketones, glucose. • CBC: 5.4>8/24.4<171 • Chem: Na 138, K 5.7, Cl 109, Bicarb 20, BUN 32, Cr 1.4, Glu 95, Ca 8.6 • LFT, Coags: wnl • ESR 75, CRP 0.2 • Group A Strep: neg

  8. Hospital Course • In the ED JV received a dose of isradipine for hypertension, as well as morphine for pain and an NS bolus. • She had a head CT which was normal, and a CXR which revealed a small left pleural effusion. • She received a second dose of isradipine for persistent hypertension. • Her BPs remained highly elevated, and she was admitted to the PICU for management of hypertensive urgency.

  9. Hospital Course • In the PICU she was started on a nicardipine drip, which was gradually weaned off as she started oral amlodipine and atenolol. • The following rheum labs were sent: ANA (+), C3 <40, C4 <12, RF 46, Anti-Ro 0.7, Anti-La <0.2, Anti-Sm >8, Anti-DSDNA 169 • Her creatinine continued to increase, reaching a maximum of 3.5 before slowly improving. • Her labs were found to be consistent with SLE. She was started on burst steroids 1g solumedrol for 3 days followed by prednisone 60mg BID. • Her leg swelling improved, though her hands remained swollen and painful. She was started on hydroxychloroquine. • She developed hyperglycemia secondary to steroid use and is currently managed with insulin. • She was discharged on HD 12 and has been followed by rheumatology, endocrinology, nephrology, and neurology.

  10. SLE - epidemiology • 5000-10,000 cases in children annually • Affects girls more often than boys • 4:1 prepubertal • 8:1 post-pubertal • Asian > black > hispanic > white • Median age of onset 12-13 years

  11. Pathogenesis • Genetics • 14-57% concordace in monozygotic twins • 5-12% occurrence in relatives • Up to 45 “associated” gene loci have been identified • Clinical Manifestations • Mediated directly or indirectly by formation of immune complexes and immune complex deposition in end organs • Renal disease- due to immune complex deposition in the mesangium, supendothelial or subepithelial spaces, with subsequent activation of complement • Anti-nuclear antibodies and antibiodies which interact with cell surfce antigens can trigger cell injury or death • Antiphopholipid antibodies can diminish the anticoagulant effect of beta-2-glycoprotein 1 complex • Skin lesions • due to DNA damage from UV exposure • immune complexes can bind to UV-activated keratinocytes • cellular membrane phospholipids can be affected by autoantibodies

  12. Presentation • Nonspecific symptoms such as fever, fatigue, or weight loss • Photosensitive rash • Arthralgia or arthritis • Raynaud phenomenon (a more common presentation in adults) • Serositis (pleuritis, pericarditis, peritonitis) • Nephritis or nephroticsyndrome • Stage I – minimal mesangial • Stage II – mesangial proliferative • Stage III – focal (hematuria and proteinuria) • Stage IV – diffuse • Stage V - membranous • Stage VI - advanced sclerosing • Neurologic symptoms, such as seizures, psychosis, cognitive dysfunction, stroke, or headaches • Alopecia • Phlebitis, arterial and/or venous thromboembolic phenomenon • Recurrent miscarriage • Anemia, lymphopenia, thrombocytopenia

  13. ACR criteria for classification of SLE. Must have 4 of 11 criteria.

  14. ACR criteria for classification of SLE. Must have 4 of 11 criteria.

  15. The SLICC (Systemic Lupus International Collaborating Clinics) requires 4 of 17 criteria (basically the same criteria divided up differently) • One clinical • One immunologic • OR biopsy proven lupus nephritis

  16. Labs • CBC • Chem 20 • Erythrocyte sedimentation rate and/or C reactive protein • Urinalysis • Urine for calculation of creatinine clearance and for quantitation of proteinuria or protein/creatinineratios • Antiphospholipidantibodies • Antinuclear antibodies (ANA) – most sensitive test • Anti-Smith (Sm) antibodies - very specific test • Antibodies to double stranded DNA (dsDNA) – very specific test • Anti-Ro and Anti-La • C3 and C4 are often low

  17. Treatment • First you must know whether the lupus is active or not • Lab testing every 6-12m for patients with quiescent disease and no renal involvement • Every 4-6m for patients with mild renal disease but otherwise quiescent SLE • Every 2-3m for patients with decreased GFR that is currently stable • Every week for patients with actively flaring disease. • General therapies • Sun protection • Diet and exercise • Smoking cessation • Immunizations • OCPs with high-dose estrogens should be avoided!

  18. Treament • Symptomatic Treatment • NSAIDs for musculoskeletal complaints, headaches, mild serositis • not in patients with renal disease • Systemic glucocorticoids • Generally reserved for significant flares or severe renal or CNS involvement • Paucity of data suggesting that IV pulse steroids are superior to daily PO • Antimalarials – hydroxychloroquine • Act on Toll-like receptors to block costimulation of the B cell antigen receptor (anti-inflammatory) • Most useful for skin and musculoskeletal symptoms • Can also help prevent damage to kidneys • May reduce incidence of flares • Immunosuppresive therapies • Methotrexate, cyclophosphamide, azathioprine, mycophenolate, Rituximab, Belimumab • Failure to respond to cyclophosphamide is associated with poor prognosis

  19. Prognosis • Factors associated with poor prognosis • Poor compliance secondary to poor patient and family education • Neurologic complications • Intercurrentinfections • Renal disease* • Atypical presentation at disease onset is associated with greater cumulative organ damage • Children with kidney failure due to lupus nephritis appear to do as well after transplant as other children with ESRD from other causes.

  20. PREP SA 2010#30 • A 14yo girl presents with a 2 month history of joint pain that is responding poorly to OTC anti-inflammatory medicine. She reports some sores in her mouth and mild swelling around her eyes and ankles. On physical exam her temperature is 37°, HR is 76, RR os 14, and BP is 130/86. She has oral ulcers, mild periorbital edema, and mild swelling of her wrists and knee joints. Lab findings include: 10.1 Albumin 2.5 190 100 24 4.8 22 1.3 3.0 UA: 3+ blood, 3+ protein, 20-50 rbc/hpf ANA titer: 1: 1280, Anti dsDNA titer: 1:640

  21. Of the following, the next BEST step in management is to • A. Admit the patient for IV cyclophosphamide • B. initiate treatment with ibuprofen • C. order a 24-hr urine for protein collection • D. refer the patient for a renal biopsy • E. refer the patient for bone marrow aspiration

  22. Questions?

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