CATEGORY : UVEA

PRESENTING AUTHOR: Dr.Vaidehi Jande

CO AUTHORS: 1.Dr.Nirmala Sarpotdar 2.Dr.Aratee Palsule

INSTITUTION: Deenanath Mangeshkar Hospital and Research Centre, Pune.

* Aggressive form of necrotising scleritis: first presentation of Wegener`s Granulomatosis.

*A 39 year old apparently healthy female presented to us with C/O redness, pain in left eye H/O treatment with topical and oral steroids in tapering doses for the same since 1 month.*Other complaints - Hoarseness of voice and Stridor, ear discharge.*No H/O any systemic illness, joint pains, Recurrent respiratory infections in the past.*Previously done investigations: negative ANA and Anti dsDNA, borderline RA factor.*Right eye-WNL, Left eye-Congestion, Chemosis, Perilimbal swelling, Scleral nodular elevation with Brownish Discolouration

*History:

* Course in hospital:*Investigations done:1. Flexible stroboscopy revealed severe subglottic oedema.2. HRCT WNL,OCT WNL3. Conjunctival swab sent for Gram staining, Zn staining and Aerobic

culture, results later on came to be NEGATIVE.4. Serology investigations sent *C-Reactive Protein* c ANCA *RA Factor *ESR *Anti CCP

*Treatment:Patient was symptomatically treated with *I.V Antibiotics *Inj Dexamethasone 4mg BD*Topically with Fortified Cephazolin e/d , Fortified Amikacin e/d and Tropicacyl

Plus.Patient improved symptomaticallyPatient was discharged on Oral Steroids 1mg/kg dose and Topical Moxifloxacin eye drops and Prednisolone acetate eye drops.Results of Serology Showed-*C-Reactive Protein- Positive 83.45* c ANCA –POSITIVE 313 U/ml*RA Factor POSITIVE 61.82 IU/ml*ESR 105mm/hr*Anti CCP 1.21 u/ml DIAGNOSIS - c ANCA positive Necrotising scleritis Wegener`s Granulomatosis.

Subsequent Follow Up Visits within 1 month:

• Even though visual acuity remained the same , clinical picture worsened with infiltration over the protrusion

• Patient was given Inj Methyl Prednisolone 1g i.v x 3 days with monitoring of vitals.

• Despite of this , further deterioration occurred and there was further thinning with bare sclera seen.

• With consultation of Rheumatologist and Physician, patient was started on Inj Cyclophosphamide i.v with Mesna and adequate hydration.

• Patient tolerated the first dose well.

• Further thinning and uveal show , Bare sclera temporally and extending inferiorly also.

• Consequently she received 2nd and 3rd doses of Cyclophosphamide on 26.07.2014 & 29.08.2014 respectively.

• c ANCA levels > 200After 1 MONTH of Injection Cyclophosphamide

*After 2 months she presented with episodes of haemoptysis and cough for which she was referred to Chest Physician and investigations were done.*Bronchoscopy and HRCT - Tree in Bud Appearance s/o Bronchial

Tuberculosis, Inflammatory pathology.*2 of 3 sputum samples showed AFB on ZN staining, RNTCP Grade I.*Sputum culture showed various organisms such as Gram +ve cocci,

budding yeast, Morxella Catarrhalis,Candida Albicans.*Cyclophosphamide was put on hold. Oral steroids(40mg/kg/day) and

Antitubercular Treatment were started.*Patient developed Drug Related ADR:1. Acute psychosis ?INH induced2. Steroid induced Depression and Myopathy.3. AKT induced hepatitis.*AKT was temporarily stopped and then started after LFTs returned to

normal levels.

*Follow up after 3 months:c ANCA 219.9 U/mlHRCT shows resolution of Tuberculosis.2D ECHO : Ejection Fraction 30%,LV Thrombosis

Right eye also showed superficial + Deep congestion s/o Scleritis Left eye showed Scleromalacia with uveal tissue ectasia 5mm temporally & inferiorly.Rituximab was suggested treatment but could not be initiated due to Koch`s so she was started on Methotrexate injections weekly.

PROGRESSION OVER NEXT 2 MONTHS:

Despite treatment with various drugs,unfortunately patient succumbed to cardiac complications.

*DISCUSSION:Granulomatosis with Polyangitis (Wegener's Granulomatosis) is an uncommon multisystem autoimmune disorder having significant mortality and morbidity.Ophthalmic manifestations of the disease are seen in 28-45 % of the patients.In 16 % patients ocular manifestation is the presenting feature of the disease.Ophthalmic manifestation of Wegener's granulomatosis is potentially sight threatening.Scleritis is one of the manifestation of Wegener's granulomatosis seen in 16-38% patients. The underlying pathology is primary granulomatous vasculitis and subsequent ischaemia. The presence and severity of necrotising scleritis reflects the systemic involvement.Serum Antibody against c ANCA is sensitive for diagnosis and for monitoring remission of the disease.Fulminant nature of the disease requires prompt treatment with immunosuppressants.

Ophthalmologist may be the first to diagnose Wegener's Granulomatosis if Necrotising scleritis is the presenting feature as in our patient.Nature and severity of the disease warrants referral and close coordination with Internist for aggressive immunosuppression to achieve remission as well as to keep watch on the adverse effects of the treatment.Cyclophosphamide is the most commonly used immunosuppressant in the treatment of the disease.In some cases the scleritis may be refractory to conventional therapy, biological agents targeting lymphocytes, TNF α should be considered.In our patient, there was constant progression of the disease despite continual treatment.

Thus our case denotes the aggressive nature and magnitude of morbidity attributed to Necrotising scleritis due to Wegener's Granulomatosis(Granulomatosis with polyangitis) despite maximal immunosuppressive therapy.

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