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Grand Rounds Temporal tenderness, headache and loss of vision… Is it Giant Cell Arteritis?

Grand Rounds Temporal tenderness, headache and loss of vision… Is it Giant Cell Arteritis?. Raafay Sophie, MD 20 th April 2018. Patient Presentation. CC “Worst headache of life” HPI 54 yo W Male presented to the ER with “sharp” headache started 3 weeks ago Max intensity 10/10

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Grand Rounds Temporal tenderness, headache and loss of vision… Is it Giant Cell Arteritis?

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  1. Grand RoundsTemporal tenderness, headache and loss of vision…Is it Giant Cell Arteritis? Raafay Sophie, MD 20th April 2018

  2. Patient Presentation CC • “Worst headache of life” HPI 54 yo W Male presented to the ER with “sharp” headache • started 3 weeks ago • Max intensity 10/10 • Over top of head and down jawline • Forehead and jaw tender to touch, pain on biting • Photophobia • Decreased coordination with difficulty ambulating • Denied blurry or double vision

  3. History (Hx) Past Ocular Hx: small rock hitting OS in 2002 Past Medical Hx: • Colorectal cancer s/p chemoradiation2010 • no surveillance since 2013 • “Crohns Disease” • Anxiety and ADHD Meds: Pantaprazole, Seroquel, Sertraline, Sucralfate, Norco 5, Flexeril FamHx: Glaucoma, Diabetes Allergies: Codeine

  4. History (Hx) Social Hx: • ½ PPD smoker • Smoked marijuana and “spice” • Denied IV drugs or Alcohol use ROS: • 20 lbswt loss in last month • No fever, rash, joint pains, or recent travel • Last sexual activity 1.5 years ago with ex-wife

  5. External Exam Vitals: T: 36.5 °C HR: 64 RR: 18 BP: 99/71 MAP: 78 SpO2: 96% HT: 175.26 cm WT: 63.64 kg BMI: 20.7

  6. Anterior Segment Exam

  7. Posterior Segment Exam

  8. Labs Metabolic Profile • Na 133 mmol/L LOW • CL 98 mmol/L LOW • Albumin 3.3 Gram/dL LOW CBC • WBC 7.0 x10(3)/uL • Hct 37.9 % LOW • Plt 324 x10(3)/uL • SedRate Auto 59 mm/Hr HI • LFTs and bleeding profile WNL CT SCAN of HEAD normal

  9. Assessment/Plan • 54 y W M with severe headache, scalp tenderness, jaw pain, ESR 59, recent weight loss and mild papillitisOS • Get MRI brain/orbits w/ and w/o gadolinium and fat suppression • Presumed giant cell arteritis- 60mg PO prednisone, follow up with Oculoplastics for temporal artery biopsy

  10. F/up • Day 3- Oculoplastics clinic • OS: 20/25 VA, mild disc edema with few disc hemorrhages • Day 10 - Underwent temporal artery biopsy • Day 13 and 16 - ER • lower limb pain and weakness, swollen tongue and face. • Day 24- Oculoplasticsclinic • Negative biopsy- Referred to neuro for Optic neuropathy OS

  11. F/up Day 26- Neurophthalmology Clinic • Headaches milder but present • OS • now blurry with black patches • VA 20/30with mild disc swelling • No APD, 10/11 Ishihara, • Review of MRI showed concern for meningeal enhancement. • Plan • Taper off prednisone, workup for inflammatory causes of meningeal enhancement and repeat MRI in 3 months.

  12. F/up Day 56 - Neurophthalmology Clinic • Did not get any labs • Headaches gone now, but OS has been “black” for few weeks. Light Perception on exam • Has developed a new rash that started from head down and is now on arms and legs

  13. Labs

  14. Exam OS

  15. OD OS

  16. OD OS

  17. F/up Day 56 - Retina Clinic • PanuveitisOS • Pred Forte Q1-2h, CyclogelTID • Lab workup • CXR, RPR, FTA/ABS, Quantiferon, ACE, Lysozyme, ESR, ANA, CRP, ANCA

  18. F/up Day 62 - Retina Clinic • Did not use drops as he thought his facial edema was recurring • No change in exam (LP vision) • Lab workup available: CRP 1.5, ESR 38 • Refused AC/Vitreous Tap. • PredForte Q1-2h, Cyclogel TID

  19. F/up Day 71 - Retina Clinic • OS: VA Counting Fingers@4 ft Improvement in synechaie and haze • OD: 1+ cell, Trace flare • Lab workup: FTA-ABS positive, RPR Reactive (1:64) • Syphilitic panuveitis • Same day Appointment to ID • Continue Cylopentolate

  20. OCT OS

  21. ID workup • LP • Opening pressure? 14ml collected • Protein 48, WBC 14 (H), Lymph 59, Monocytes 39 • VDRL, NMDA, Paraneoplastic AB panel, Cryptococcus, Oliogoclonal bands negative • HIV negative, Hep C negative • Hep B core antigen positive, Hep B Surface Antibody negative

  22. F/up Day 80 - Retina Clinic - 9 days of IV PCN • OS: VA 20/100

  23. OCT OS

  24. OCT Enface at EZ

  25. Syphilis: “The Great Imitator” • STD • Primary – Chancre – usually 21 days after • Secondary - 4-10 weeks after the primary • Systemic symptoms (fever, sore throat, malaise, weight loss, hair loss, and headache) • Maculopapularrash on trunk and extremities • Condylomalatum (Wart-like lesions) on genitals • Latent – positive serology • Tertiary • Gummatous, Neurosyphyllis, Cardiovascular

  26. Ocular Syphilis • Resurgence in recent times • 200 cases from 20 states in 2 years were reported to CDC (March 2016 Clinical Advisory) • Highest risk in men who have sex with men (MSMs) CDC Recommendations • Reverse sequence testing • Initial treponemalantibodies (FTA-ABS) • Then quantitative non-treponemal test (VDRL/RPR) • All patients to have Lumbar puncture • 60% of patients with ocular syphilis will have abnormalities noted on CSF • All patients to have HIV testing • HIV present in 20% to 70% of patients

  27. Ocular Syphilis • Primary and secondary syphilis accounts for only 31.4% of all cases • Most common manifestation is panuveitis (40-50%) • posterior uveitis may be more common in HIV-negative person • The Great Imitator may present in a number of ways

  28. Inflammation, Gummas, Granulomas everywhere!!

  29. Pan/Posterior Uveitis • Chorioretinitis with vitritis is the most common, typically involving the posterior pole and mid-periphery. • Acute syphilitic posterior placoidchorioretinitis (ASPPC)

  30. Treatment And Prognosis • Aqueous crystalline penicillin G 18–24 million units/day for 10–14 day • British Ocular Syphilis Study (BOSS) - • 92.1% had visual acuity ≥ 0.3 logMAR (20/40 Snellen) after antibiotic therapy.

  31. Take Home Points • Be aware of the Great Imitator, Ocular Syphyllis is rare but on the rise!! • Majority of patients present in the tertiary or late phase • Most common presentation is pan or posterior uveitis, but may present anywhere in the eye • LP and HIV testing is mandatory • Treatment is IV penicillin

  32. References • Curr Infect Dis Rep. 2016 Nov;18(11):36. doi: 10.1007/s11908-016-0542-9. Ocular Syphilis: a Clinical Review. Woolston SL1, Dhanireddy S2, Marrazzo J3. • Ocular Syphilis: An Update. Dutta Majumder P, Chen EJ, Shah J, Ching Wen Ho D, Biswas J, See Yin L, Gupta V, Pavesio C, Agrawal R. OculImmunolInflamm. 2017 Oct 11:1-9. doi: 10.1080/09273948.2017.1371765. [Epub ahead of print] PMID: 29020491 • CurrOpinOphthalmol. 2014 Nov;25(6):513-8. doi: 10.1097/ICU.0000000000000099. Ocular syphilis. Davis JL1. • https://www.cdc.gov/std/syphilis/clinicaladvisoryos2015.htm • Mathew RG, Goh BT, Westcott MC. British Ocular Syphilis Study (BOSS): 2-year national surveillance study of intraocular inflammation secondary to ocular syphilis. Invest Ophthalmol Vis Sci. 2014; 55:5394-5400 • Clinical features and incidence rates of ocular complications in patients with ocular syphilis.Moradi A, Salek S, Daniel E, Gangaputra S, Ostheimer TA, Burkholder BM, Leung TG, Butler NJ, Dunn JP, Thorne JE. Am J Ophthalmol. 2015 Feb;159(2):334-43.e1. doi: 10.1016/j.ajo.2014.10.030. Epub 2014 Nov 5. • Spoor TC, Ramocki JM, Nesi FA et al. Ocular syphilis 1986. Prevalence of FTA-ABS reactivity and cerebrospinal fluid findings. J ClinNeuroophthalmol 1987;7:191-5, 196-7

  33. Acknowledgement • Dr. Harpal Sandhu • Dr. Thong Diep Pham

  34. Thank you!

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