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Grand Rounds

Grand Rounds. Eddie Apenbrinck M.D . University of Louisville School of Medicine Department of Ophthalmology & Visual Sciences 3/7/2014. Subjective. CC: Left inferotemporal periorbital tenderness and swelling

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Grand Rounds

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  1. Grand Rounds Eddie Apenbrinck M.D. University of Louisville School of Medicine Department of Ophthalmology & Visual Sciences 3/7/2014

  2. Subjective CC: Left inferotemporal periorbital tenderness and swelling HPI: 17 year old white female with a 2 ½ month history of chronic sinus congestion presents to Kosair ED with 1 day of tenderness and swelling of the left inferotemporal orbital rim

  3. Treatment prior to ED • Diagnosed with pansinusitis 2 week prior to ED visit • Started on clindamycin and prednisone PO • Minimal improvement on antibiotics and steroids • CT sinuses: erosion of nasal septum • Nasal Mucosa Biopsy: • Initially read as likely granulomatosis with polyangiitis (GPA) • Second opinion read biopsy as possible Epstein Bar Virus (EBV) lymphoma • Treated with pulse steroids and scheduled for pediatric rheumatology appointment

  4. POH: Myopic PMHx: saddle nose deformity FHx: seasonal allergies ROS: intermittent headaches, epistaxis, myalgiaand fatigue (~2½ months) Allergies: cefprozil (hives) Meds: none

  5. Exam OD OS VA(sc,n): 20/20OU Pupils: 3 2 OU No RAPD IOP: 12 OU EOM: Full OU slight inferior periorbital tenderness on upgaze Orbits: No proptosis, No lid retraction, No ecchymosis

  6. Exam OD OS Anterior Segment L/L: WNL slight swelling of left inferotemporal periorbital area C/S: WNL OU K: WNL OU AC: No cell or flare OU I/L: WNL OU Vitreous: WNL OU Dilated Fundus Exam: WNL OU

  7. MRI T2 Coronal MRI of orbits shows enhancement of ethmoid sinus with erosion into left medial orbit

  8. MRI T1 Axial with gadolinium shows enhancement of ethmoid sinus with erosion into left medial orbit

  9. Assessment • Assessment: 17yo white female with chronic sinus congestion found to have an ethmoid sinus lesion with extension into the left orbit. • DDx • Granulomatosis with polyangiitis (GPA) • EBV lymphoma • Chronic Sinusitis

  10. Plan • Plan • Admission for further workup and treatment • Started on IV ampicillin-sulbactam(Unasyn) 3,000mg q6h • Repeat biopsy of nasal mucosa • Biopsy of left inferior nasal septum and left inferior turbinate obtained and sent to pathology • Ophthalmology follow-up as outpatient following discharge

  11. Hospital Course Labs Immunoglobulin Panel: (all WNL) C3 149 C4 25 IgA 165 IgG 949 IgM 67 CBC, CMP and UA: wnl Gram Stain: positive for gram negative bacillus Nasal Mucosa Culture: Klebsella Pneumonia ESR: 65 (0-25) CRP: 166.7 (1.0-9.0) Proteinase-3 Auto Antibodies: positive c-ANCA: negative p-ANCA: negative Myeloperoxidase Auto antibodies: negative TSH: wnl Free T4: wnl

  12. Hospital Course Imaging • CXR, MRI/MRA brain: WNL Flow Cytometry • normal percentages and absolute numbers of T-cell, B-cell and natural killer cell populations. No evidence of B-cell monoclonality or increased blast population Anatomic Pathology • Both biopsy samples showed necrotizing granulomatous inflammation consist with GPA

  13. Pathology Necrotizing granulomatous inflammation 10x magnification 20x magnification eosinophiles Granuloma with necrotic center granuloma Multinucleated giant cell

  14. Hospital Course • Started on Solumedrol 1g once followed by oral taper as outpatient • Started on rituximab (15mg/kg) and cyclophosphamide (15mg/kg) once • Patient discharged with plan for readmission in 2 weeks for second dose of rituximab and cyclophosphamide

  15. Ophthalmology 1 week follow up OD OS VA(cc): 20/20 OU Pupils: 4 3 OU; No RAPD IOP: 13 14 EOM Full OU without pain Ant Segment: WNL OU DFE: WNL OU Assessment: Patient with GPA currently asymptomatic Plan: Observe with follow-up in 6 months

  16. Clinical Course • Second dose of rituximab and cyclophosphamide 2 weeks after discharge • Patient with increased energy, no epistaxis and improved nasal congestion • CBC & CMP: WNL • CRP = 15.8 (<10.0) • Patient was started on maintenance therapy of Azathioprine, Bactrim, and continued prednisone taper

  17. Granulomatosis with Polyangiitis • GPA is a multisystem autoimmune disorder characterized by the classic triad of necrotizing granulomatous vasculitis of the upper and lower respiratory tract, focal segmental glomerulonephritis, and necrotizing vasculitis of small vessels • Incidence: 3 per 100,000 reported in United States • Differential Diagnosis: • Clinical: Orbital pseudotumor, Sarcoidosis, fungal infections, HistiocytosisX, IGG4-related disease, , Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy), • Pathology: polyarteritisnodosa, microscopic polyangiitis, Churg-Strauss syndrome

  18. Granulomatosis with Polyangiitis • Anti-cytoplasmic Nuclear Antibodies (ANCA): staining occurs in two patterns • Cytoplasmic staining pattern (C-ANCA), which is specific for Proteinase-3 (PR3) also called myeloblastin • Perinuclear staining pattern (P-ANCA), which is specific for myeloperoxidase • 32% to >95% of patients with GPA are positive C-ANCA depending on disease activity 7 • 91% sensitivity and 99% specificity in active disease 8 • Less than 5% of patient with GPA are positive for P-ANCA

  19. Clinical Features • Patient typically have flu-like symptoms lasting several days or weeks including fever, polymyalgia, polyarthralgia, headache, malaise, anorexia, unintended weight loss • 90% of patients report ear, nose, or throat problems • 75% of patients seek care because of upper and lower respiratory complaints  • Ocular or orbital involvement is seen in 15% of patients at presentation and up to 50% during the course of the disease.

  20. Clinical Features Chronic Complications: Orbital socket contracture, enophthalmos, restrictive ophthalmopathy, chronic orbital pain Foster CS, Yang J. Wegener’s Granulomatosis. Albert & Jakobiec's Principles and Practice of Ophthalmology. Pages 4447- 4460. 2008.

  21. Treatment • There is no standardized treatment regimen for granulomatosis with polyangiitis • Treatment is aimed at inducing remission • General approach includes high-dose steroids and cyclophosphamide • Rituximab-based regimen plus steroids may be considered as alternative in patients with relapsing or refractory disease • Remission maintained with cyclophosphamide, methotrexate or azathioprine • Plasma exchange may be beneficial in patients with severe renal disease

  22. Literature Search Aug 2013 Randomized double blind clinical trial included 197 patients, compared rituximab (once a week for 4 weeks) followed by placebo to cyclophosphamide administered for 3 to 6 months followed by azathioprine for 12 to 15 months. Both groups showed comparable rates of remission at 6, 12 and 18 months Conclusion: a single course of rituximab was as effective as continuous conventional immunosuppressive therapy for the induction and maintenance of remission over the course of 18 months.

  23. Literature Search Ophthalmology. 2014 Feb • Retrospective noninterventional comparative case series including 247 patients with orbital inflammation, compared GPA with other causes of orbital inflammation to identify the presenting clinical and radiographic features most likely to predict GPA • Features highly suggestive of GPA: sinonasal symptoms, sinonasal changes, or paranasal bone erosion on imaging. • 22% of patients (8/37) with GPA had evidence of systemic involvement at presentation, and no patient presenting with only orbital GPA developed later systemic disease over a median follow-up of 2.7 years.

  24. Thank You

  25. References 1. BCSC: Intraocular Inflammation and Uveitis. Pgs :145-146 2. BCSC: Update on General Medicine. Optic Neuritis. Pgs 177-179 3. Pakrou N, Selva D, Leibovitch I. Wegener;s Granulomatosis: ophthalmic manifestations and management. Semin Arthritis Rheum. 2006;35(5):284-292 4. Berden A, Göçeroglu A, Jayne D, et al. Diagnosis and management of ANCA associated vasculitis. BMJ. 2012 Jan 16;344:e26. 5. Schilder AM. Wegener's Granulomatosis vasculitis and granuloma. Autoimmun Rev. 2010 May;9(7):483-7. 6. Tan LT, Davagnanam I, et.al. Clinical and Imaging Features Predictive of Orbital Granulomatosis with Polyangiitis and the Risk of Systemic Involvement. Ophthalmology. 2014 Feb 20. pii: S0161-6420(13)01175-5. doi: 10.1016/j.ophtha.2013.12.003. [Epub ahead of print] 7. Foster CS, Yang J. Wegener’s Granulomatosis. Albert & Jakobiec's Principles and Practice of Ophthalmology. Pages 4447- 4460. 2008. 8. SchonermarckU, Lamprecht P, Csernok E, Gross WL. Prevalence and spectrum of rheumatic diseases associated with proteinase 3-antineutrophil cytoplasmic antibodies (ANCA) and myeloperoxidases- ANCA. Rheumatology 2001; 40; 178-84 9. Specks U, Merkel PA, Seo P, Spiera R et al. RAVE-ITN Research Group. Efficacy of remission-induction regimens for ANCA-associated vasculitis. N Engl J Med. 2013 Aug 1;369(5):417-27.

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