Case Presentation · Scleritis/episcleritis • Keratitis • Uveitis/retinal disease (rare) •...
Transcript of Case Presentation · Scleritis/episcleritis • Keratitis • Uveitis/retinal disease (rare) •...
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Case Presentation
Rafid Asfar, MD
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Introduction
• ANCA associated vasculitis may be localized or systemic, and can involve the eyes
• Ocular manifestations can occur in the absence of systemic disease in persons of any age
• Ocular disease may be a rapidly progressive and serious manifestation
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Introduction
Manifestations of ocular disease may include:• Eyelid swelling or periorbital edema• Lacrimal gland involvement (including vasculitis and dacrocystitis)• Scleritis/episcleritis• Keratitis• Uveitis/retinal disease (rare)• Orbital pseudotumor • Bone erosion, destruction and invasion of the orbitANCA may be negative in patients with isolated ocular involvement
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Introduction
Management of ocular manifestations is based on type and degree of involvement and may include:• Local steroid therapy• Systemic glucocorticoids• Systemic steroid sparing immunosuppressive agents,
including cyclophosphamide and rituximab, as induction therapy
• Surgical intervention
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History of present illness
• 18 year old Lebanese female presented with 3 weeks of left periorbital, facial, and ear pain
• She was treated with oral antibiotics by PCP for possible otitis media
• Symptoms continued to progress and patient presented to ER shortly thereafter
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Review of systems
• Constitutional: fatigue
• HEENT: pain at left maxillary sinus and left periorbital area. Negative for hearing loss, sore throat
• Respiratory: Negative for cough, sputum production, hemoptysis or shortness of breath
• CVS: Negative for chest pain. No lower extremity edema
• GI: Negative for nausea, vomiting, diarrhea or abdominal pain. No hematemesis. No bleeding per rectum
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Review of systems
• Urinary system: no hematuria, no dysuria, no urgency, no increase in frequency of urination
• Neurological system: Negative for confusion, dizziness, numbness and headaches. No weakness in upper or lower extremities
• RES: Negative for lymphadenopathy, no hepatosplenomegaly
• Skin: no rash, no ulcers, no bruises
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Background history
• Past medical history
None
• Past surgical history
None
• Medications Recent antibiotics as noted
• Allergies NKDA
• Social history Non smoker
No alcohol use
No illicit drug use
Not sexually active
• Family history
No autoimmune disease
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Emergency room course
• CT scan at that time demonstrated pansinusitis with left orbital extension
• The patient was prescribed levofloxacin and discharged home with a tapering dose of steroids
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Clinical course
• Three days later she presented to ENT and ophthalmology with worsening symptoms of left orbital pain, proptosis, swelling of left face contour and left ear pain
• Left myringotomy and tympanostomy tube placement were done
• The same day she was assessed by neuroophthalmologywith a normal visual examination
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Clinical course
• Three days after that she woke up suddenly with left sided facial paralysis, headaches and worsening of ocular proptosis
• She was admitted to the hospital
• CT scan showed worsening of the orbital mass
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Initial work up
CBCHb 12.4; WBC 10.4; Plt 361
CMPNa 138; K 3.9; Urea 11; Cr 0.88; HCO3 99; Cl 27; AG 12
LFT
ALT 18; AST 10; Alk P 91; T.Bili 0.5; Pr 8.4; Alb 3.8
UA
RBC: negative
Protein: negative
No cellular casts
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Autoimmune serologies
• ANA negative
• C-ANCA 1:80 (normal <1:20)
• EIA positive for PR3
• P-ANCA <1:20
• RF 51 (normal <15)
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Surgical intervention
A combined surgery by ophthalmology and ENT was done this included:
• Left orbitotomy with drainage of left orbital/subperiosteal abscess and left orbital medial wall decompression
• Left anterior ethmoidectomy, left sphenoidectomy and left maxillary anterostomy
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Clinical course
• The pathology report of the sinuses and periorbita showed necrotizing acute and chronic inflammation
• We recommended lung biopsy to confirm the diagnosis of suspected GPA
• Meanwhile she was started on IV Solumedrol
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Clinical course
• CT chest was ordered as part of evaluation for possible systemic vasculitis
• CT showed multiple cavitary and non cavitary nodules
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Lung biopsy
Pathology:
• necrotizing granulomatosis with eosinophils
• geographic necrosis with eosinophils, surrounded by microabscesses, palisading histiocytes and giant cells
• vasculitis present in vessels at the periphery of the zones of necrosis.
• GMS negative for fungal organisms; AFB stain negative
• “The differential includes the clinical impression of Wegener's granulomatosis”
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Treatment
• Pulsed steroid therapy
• Oral steroid tapered down slowly
• Rituximab (RAVE study protocol)
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Follow up
• Patient is asymptomatic and doing well off of all steroids
• Resolution of the sinusitis, orbital mass and lung lesions
• C-ANCA undetectable
• CD19 & CD20 B-lymphocytes undetectable
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Discussion
• Young patient with less typical presentation
• Main presentation in our patient was orbital mass (abscess) beside the sinusitis
• Aggressive, rapidly progressive and destructive disease
• Rituximab can be efficacious in the treatment of aggressive ocular manifestations
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References
• Ocular disease in patients with ANCA-positive vasculitis J Ocul Biol Dis Infor. 2010 Mar; 3(1): 12–19
• Ocular Manifestations of Wegener's Granulomatosis HazlitaIsa; Susan Lightman; Charles D Pusey; Simon RJ Taylor DisclosuresExpert Rev Ophthalmol. 2011;6(5):541-555
• Semin Arthritis Rheum. 2006 Apr;35(5):284-92. Wegener's granulomatosis: ophthalmic manifestations and management. akrou N1, Selva D, Leibovitch I
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Acknowledgements
• Henry Ford Hospital, Department of Internal Medicine, Division of Rheumatology:
Kathleen McKinnon, D.O.
Donard Haggins, M.D.