Case Presentation

Rafid Asfar, MD

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

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

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

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

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

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

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

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

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

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

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

Autoimmune serologies

• ANA negative

• C-ANCA 1:80 (normal <1:20)

• EIA positive for PR3

• P-ANCA <1:20

• RF 51 (normal <15)

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

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

Clinical course

• CT chest was ordered as part of evaluation for possible systemic vasculitis

• CT showed multiple cavitary and non cavitary nodules

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”

Treatment

• Pulsed steroid therapy

• Oral steroid tapered down slowly

• Rituximab (RAVE study protocol)

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

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

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

Acknowledgements

• Henry Ford Hospital, Department of Internal Medicine, Division of Rheumatology:

Kathleen McKinnon, D.O.

Donard Haggins, M.D.