$1 Million $500,000 $250,000 $125,000 $64,000 $32,000 $16,000 $8,000 $4,000 $2,000 $1,000 $500 $300 $200 $100 Welcome to Who Wants to be a Millionaire 50:50

© Mark E. Damon - All Rights Reserved Another Presentation

© Mark E. Damon - All Rights Reserved

$1 Million $500,000 $250,000 $125,000 $64,000 $32,000 $16,000 $8,000 $4,000 $2,000 $1,000 $500 $300 $200 $100

© Mark E. Damon - All Rights Reserved A: + ANA C: thrombocytopenia B: elevated ESR D: proteinuria 50: $1 Million $500,000 $250,000 $125,000 $64,000 $32,000 $16,000 $8,000 $4,000 $2,000 $1,000 $500 $300 $200 $100 What is a common lab finding in Kawasaki disease?

© Mark E. Damon - All Rights Reserved Typical findings in Kawasaki Disease Fever persisting at least 5 days (> 38.5/ 101) Changes in extremities Erythema or edema of hands and feet Desquamation of hands and feet (periungal) Conjunctivitis Bilateral bulbar erythema with limbic sparing Oral Mucus Membrane Changes (mucositis) Dry, cracked lips Strawberry tongue Polymorphous rash Perineal  rash of trunk/extremities Cervical lymphadenopathy (> 1.5 cm)

© Mark E. Damon - All Rights Reserved Kawasaki Labs Acute phase reactants (CRP and ESR) –CRP >3 –ESR >40 Complete blood count (CBC) –WBC >15 –normocytic anemia –possibly thrombocytosis Urinalysis (U/A), preferably clean catch –sterile pyuria Serum alanine aminotransferase level Serum albumin –> 3

© Mark E. Damon - All Rights Reserved $1 Million $500,000 $250,000 $125,000 $64,000 $32,000 $16,000 $8,000 $4,000 $2,000 $1,000 $500 $300 $200 $100

© Mark E. Damon - All Rights Reserved A: SLE C: Pauciarticular JRA B: Transient synovitis D: Lyme disease 50: $1 Million $500,000 $250,000 $125,000 $64,000 $32,000 $16,000 $8,000 $4,000 $2,000 $1,000 $500 $300 $200 $100 3 yo girl with swollen left knee and + ANA. What is the likely diagnosis?

© Mark E. Damon - All Rights Reserved JRA Symptoms at least 6 weeks & at least one joint Morning stiffness Polyarthritis –5 or more joints affected –ANA is positive Pauciarticular –4 or fewer joint affected –ANA positive with negative RF –At risk for UVEITIS

© Mark E. Damon - All Rights Reserved $1 Million $500,000 $250,000 $125,000 $64,000 $32,000 $16,000 $8,000 $4,000 $2,000 $1,000 $500 $300 $200 $100

© Mark E. Damon - All Rights Reserved A: HLA B27 +, RF - C: HLA B27 +, RF + B: HLA B27 -, RF - D: HLA B27 -, RF + 50: $1 Million $500,000 $250,000 $125,000 $64,000 $32,000 $16,000 $8,000 $4,000 $2,000 $1,000 $500 $300 $200 $100 Ankylosing spondylitis is associated with what lab finding?

© Mark E. Damon - All Rights Reserved Ankylosing Spondylitis Chronic, painful, degenerative arthritis of spine and SI joints –Complete fusion is known as bamboo spine It is a rheum disease with a big genetic component –HLA B27 is positive in >95% of patients –However, RF is usually negative NSAIDS are first line treatment –Don’t forget physical therapy too!

© Mark E. Damon - All Rights Reserved $1 Million $500,000 $250,000 $125,000 $64,000 $32,000 $16,000 $8,000 $4,000 $2,000 $1,000 $500 $300 $200 $100

© Mark E. Damon - All Rights Reserved A: Urticaria C: Erythema multiforme B: Meningococcemia D: HSP 50: $1 Million $500,000 $250,000 $125,000 $64,000 $32,000 $16,000 $8,000 $4,000 $2,000 $1,000 $500 $300 $200 $100 What is this?

© Mark E. Damon - All Rights Reserved A note about HSP… Self-Limited Vasculitis –Most common in children aged 5-7 years –Most common systemic vasculitis in kids Tetrad –Palpable Purpura (100%) –Arthritis / Arthralgia (45-80%) –Abdominal Pain (50-65%) –Glomerular Disease (20-55%)

© Mark E. Damon - All Rights Reserved $1 Million $500,000 $250,000 $125,000 $64,000 $32,000 $16,000 $8,000 $4,000 $2,000 $1,000 $500 $300 $200 $100

© Mark E. Damon - All Rights Reserved A: Hepatomegaly C: Lymphadenopathy B: Pain awakens child D: Morning stiffness 50: $1 Million $500,000 $250,000 $125,000 $64,000 $32,000 $16,000 $8,000 $4,000 $2,000 $1,000 $500 $300 $200 $100 A patient presents with diffuse pain. What makes you think ALL instead of JRA?

© Mark E. Damon - All Rights Reserved Congratulations! You’ve Reached the $1,000 Milestone! Congratulations! C o n g r a t u l a t i o n s !

© Mark E. Damon - All Rights Reserved $1 Million $500,000 $250,000 $125,000 $64,000 $32,000 $16,000 $8,000 $4,000 $2,000 $1,000 $500 $300 $200 $100

© Mark E. Damon - All Rights Reserved A: Oral ulcers C: Genital ulcers B: Pressure ulcers D: GI ulcers 50: $1 Million $500,000 $250,000 $125,000 $64,000 $32,000 $16,000 $8,000 $4,000 $2,000 $1,000 $500 $300 $200 $100 Which of these findings is not seen in Behcet’s syndrome?

© Mark E. Damon - All Rights Reserved Behcet Syndrome Described by Hippocrates in the 5 th century Syndrome of recurrent aphthous ulcers, genital ulcers and uveitis. –Uveitis can lead to blindness! –Arthritis is also a common manefestation. ANA and RH are normal Treatment includes steroids, anti-gout medication and immunosuppresive agents

© Mark E. Damon - All Rights Reserved $1 Million $500,000 $250,000 $125,000 $64,000 $32,000 $16,000 $8,000 $4,000 $2,000 $1,000 $500 $300 $200 $100

© Mark E. Damon - All Rights Reserved A: Pauciarticular arthritis C: Carditis B: Bells palsy D: Strep pyogenes 50: $1 Million $500,000 $250,000 $125,000 $64,000 $32,000 $16,000 $8,000 $4,000 $2,000 $1,000 $500 $300 $200 $100 This disease is associated with all of the following except?

© Mark E. Damon - All Rights Reserved Not a lemon but a lyme! The rash shown is erythema chronicum migrans from Lyme disease The causative organism is Borrelia burgdorferi. Acute rheumatic fever and lyme disease can look similar –ARF has migratory polyarthritis and erythema marginatum rash; –Carditis can be part of both ARF and Lyme disease.

© Mark E. Damon - All Rights Reserved $1 Million $500,000 $250,000 $125,000 $64,000 $32,000 $16,000 $8,000 $4,000 $2,000 $1,000 $500 $300 $200 $100

© Mark E. Damon - All Rights Reserved A: Acyclovir C: Ceftriaxone B: IVIG D: Levofloxacin 50: $1 Million $500,000 $250,000 $125,000 $64,000 $32,000 $16,000 $8,000 $4,000 $2,000 $1,000 $500 $300 $200 $100 Lyme disease is treated with what?

© Mark E. Damon - All Rights Reserved More on Lyme Treatment –doxycycline, –erythromycin, –azithromycin –ceftriaxone Treat for minimum 14 days

© Mark E. Damon - All Rights Reserved $1 Million $500,000 $250,000 $125,000 $64,000 $32,000 $16,000 $8,000 $4,000 $2,000 $1,000 $500 $300 $200 $100

© Mark E. Damon - All Rights Reserved A: NSAIDS C: Send ANA B: Aspirate joint D: Ultrasound of hip 50: $1 Million $500,000 $250,000 $125,000 $64,000 $32,000 $16,000 $8,000 $4,000 $2,000 $1,000 $500 $300 $200 $100 2 yo male presents with a limp. He has a URI 2 wks ago & AGE 1 month ago. PE elicits pain on rotation of L hip. Management?

© Mark E. Damon - All Rights Reserved A bum knee Based on history, this is probably post infectious or toxic synovitis. Treat with NSAIDs and supportive care and follow-up. If the limp/pain does not resolve in a week or two, then a further work-up is warranted.

© Mark E. Damon - All Rights Reserved $1 Million $500,000 $250,000 $125,000 $64,000 $32,000 $16,000 $8,000 $4,000 $2,000 $1,000 $500 $300 $200 $100

© Mark E. Damon - All Rights Reserved A: Carditis C: Subcutaneous nodules B: Arthralgia D: Shortened PR interval 50: $1 Million $500,000 $250,000 $125,000 $64,000 $32,000 $16,000 $8,000 $4,000 $2,000 $1,000 $500 $300 $200 $100 Which of the following is not seen in Acute Rheumatic Fever?

© Mark E. Damon - All Rights Reserved Congratulations! You’ve Reached the $32,000 Milestone! Congratulations! C o n g r a t u l a t i o n s !

© Mark E. Damon - All Rights Reserved $1 Million $500,000 $250,000 $125,000 $64,000 $32,000 $16,000 $8,000 $4,000 $2,000 $1,000 $500 $300 $200 $100

© Mark E. Damon - All Rights Reserved A: E coli UTI C: Salmonella poisoning B: Chlamydia D: Gonorrhea 50: $1 Million $500,000 $250,000 $125,000 $64,000 $32,000 $16,000 $8,000 $4,000 $2,000 $1,000 $500 $300 $200 $ yo male c/o of red eyes and dysuria. Also has low grade fever & a swollen knee and ankle. What is cause of his dysuria?

© Mark E. Damon - All Rights Reserved Reiter syndrome Can’t pee, see or climb a tree remember? –Urethritis –Arthritis –Iritis Negative ANA, RF Most cases in children are post-enteric: Yersinia, Shigella, Salmonella. Most cases in sexually active teenagers are chlamydial---NONgonococcal.

© Mark E. Damon - All Rights Reserved $1 Million $500,000 $250,000 $125,000 $64,000 $32,000 $16,000 $8,000 $4,000 $2,000 $1,000 $500 $300 $200 $100

© Mark E. Damon - All Rights Reserved A: Supraclavicular LAD C: + RF B: Caseating granulomas D: Hypercalcemia 50: $1 Million $500,000 $250,000 $125,000 $64,000 $32,000 $16,000 $8,000 $4,000 $2,000 $1,000 $500 $300 $200 $ yo female has cough x 6 mo, 10 lb wt loss & dropped out of dance team b/c of fatigue. PPD is neg. Which finding raises your suspicion for sarcoidosis?

© Mark E. Damon - All Rights Reserved Sarcoidosis facts NON caseating granulomas secrete vitamin D Hypercalcemia and hypercalcuria Renal disease and eye disease (may also see hilar adenopathy)

© Mark E. Damon - All Rights Reserved $1 Million $500,000 $250,000 $125,000 $64,000 $32,000 $16,000 $8,000 $4,000 $2,000 $1,000 $500 $300 $200 $100

© Mark E. Damon - All Rights Reserved A: Low C3, Normal C4 C: Low C3 and C4 B: Normal C3, Low C4 D: Elevated C3 and C4 50: $1 Million $500,000 $250,000 $125,000 $64,000 $32,000 $16,000 $8,000 $4,000 $2,000 $1,000 $500 $300 $200 $100 You are evaluating a patient with pleural effusions, confusion, calf tenderness, hematuria and HTN. You expect to see:

© Mark E. Damon - All Rights Reserved Get low! Low C3 and C4 seen in lupus nephritis. –This is a common board question –This is also a common Dr. V question! This patient also has serositis and thromboembolus and lupus cerebritis (confusion) –DVT from lupus anticoagulant She’s in bad shape!

© Mark E. Damon - All Rights Reserved $1 Million $500,000 $250,000 $125,000 $64,000 $32,000 $16,000 $8,000 $4,000 $2,000 $1,000 $500 $300 $200 $100

© Mark E. Damon - All Rights Reserved A: Nevus sebaceous C: Ash leaf spot B: Neurofibromatosis D: Scleroderma 50: $1 Million $500,000 $250,000 $125,000 $64,000 $32,000 $16,000 $8,000 $4,000 $2,000 $1,000 $500 $300 $200 $100 What is the diagnosis?

© Mark E. Damon - All Rights Reserved Scleroderma Excessive collagen is deposited in the skin and other organs Localized disease –Disabling and disfiguring –Not lethal Systemic disease –Can be fatal –Almost all patients have Raynaud’s phenomenon –Effects heart, lung, kidney, GI

© Mark E. Damon - All Rights Reserved Scleroderma Labs –+ANA –+ anti-centromere More common in limited form –+anti- scl70 More common in diffuse disease Types –Diffuse Rapid onset –CREST/ limited Calcinosis Raynaud’s Esophageal dysmotility Sclerosactyly Telangiectasia –Linear Aka Morphea Limited to skin

© Mark E. Damon - All Rights Reserved $1 Million $500,000 $250,000 $125,000 $64,000 $32,000 $16,000 $8,000 $4,000 $2,000 $1,000 $500 $300 $200 $100

© Mark E. Damon - All Rights Reserved A: Non-hodgkin lymphoma C: Hairy cell leukemia B: Multiple myeloma D: Constipation 50: $1 Million $500,000 $250,000 $125,000 $64,000 $32,000 $16,000 $8,000 $4,000 $2,000 $1,000 $500 $300 $200 $100 Your patient complains of dry mouth and has been using Visine because her eyes are always dry. She is at increased risk to develop:

© Mark E. Damon - All Rights Reserved Sjogren’s Syndrome Autoimmune attack on exocrine glands 5-10% of patients with Sjogren syndrome progess to NHL –Due to lymphocytic infiltration of exocrine glands May occur by itself or in conjunction with other autoimmune diseases

© Mark E. Damon - All Rights Reserved YOU WIN $1 MILLION DOLLARS!