86 Med J Malaysia Vol 75 No 1 January 2020
SUMMARYA 47-year-old woman with poorly controlled asthma
andallergic rhinitis presented with recurrent episodes ofbilateral
upper eyelid swelling associated with fornicealconjunctival mass
for the past 10 years. Routine bloodinvestigations showed raised
IgE levels and raisedeosinophil counts. The diagnosis of
Churg-Strausssyndrome (CSS) was made following biopsy of
theconjunctival mass. The symptoms responded well to oralsteroid
treatment but recurred following cessation of thetherapy. The
patient was co-managed with a rheumatologistand the patient
currently remains stable and is on oralMethotrexate and low dose
oral steroids. Ocularinvolvement in CSS is unusual but this unique
presentationof CSS was successfully managed, and the patient
remainsin remission.
INTRODUCTIONChurg-Strauss syndrome (CSS) is also known as
Eosinophilicgranulomatosis with polyangiitis (EGPA). It is a rare
systemicvasculitis which affects the small and
medium-sized-vesselsand cause fibrinoid necrosis. CSS usually
involves the heart,skin, lung and gastrointestinal tract. It very
rarely involvesthe orbital tissue. Here we describe a patient who
presentedwith bilateral recurrent upper eyelid swelling
andconjunctival mass for the past 10 years which responded wellto
steroid treatment. The diagnosis of CSS was madefollowing
histopathological examination of the conjunctivalbiopsy.
CASE REPORTA 47-year-old woman with poorly controlled
bronchialasthma and allergic rhinitis since 2004, first presented
to oureye clinic in October 2017 with right eye painless upper
lidswelling. She had four previous episodes of bilateral
eyelidswelling associated with a conjunctival mass over the past
10years, which resolved after oral high dose steroid treatment.In
2011 and 2017, biopsies were taken from her left eyeconjunctival
mass and histopathology was consistent withChurg-Strauss syndrome.
At presentation, both eyes Snellenvisual acuity were 6/9 with no
relative afferent pupillarydefect (RAPD). On examination, the right
upper eyelidappeared swollen with a diffused, mildly injected
bulbarconjunctival mass extending from the superonasal to the
superotemporal area measuring 3.5mm x 2mm (Figure 1).The left
eye also has a similar conjunctival mass at thesuperotemporal area.
Extraocular muscle movements in botheyes were full, and no
proptosis was present. Fundusexamination of both eyes were
unremarkable. Ourdifferential diagnosis at this point included
conjunctivalgranuloma, conjunctival lymphoma or idiopathic
orbitalinflammatory disease.
Laboratory investigation of the blood of the patient wasnegative
for perinuclear anti-neutrophil cytoplasmicantibody (p-ANCA),
cytoplasmic anti-neutrophil cytoplasmicantibody (c-ANCA) and
anti-double stranded DNA antibody(dsDNA). Titres of antinuclear
antibody (ANA) andcomplement C3/4 were within normal range, as were
theerythrocyte sedimentation rate (ESR). However, eosinophilcount
and IgE was elevated at two hundred four. A repeatbiopsy was done
under local anaesthesia for the rightconjunctival mass. The
histopathology report diagnosed thecondition as granulomatous
vasculitis. Microscopicexamination showed fibrocollagenous tissue
whichcontained multiple large granulomas with centralnecrobiotic
collagenous core surrounded by palisade ofhistiocytes and
macrophages, lymphocytes, multinucleatedgiant cells and
eosinophils. The interstitium showed infiltrateof eosinophils.
There was no fibrinoid necrosis (Figure 2).These histological
features were compatible with that seen inCSS as she had four of
the six criteria to the diagnosis of CSS.1
The patient was treated with oral Prednisolone 20mg OD
andtopical tobradex QID. The patient was co-managed with
therheumatologist. The symptoms improved, and lesion reducedin size
after oral and topical steroid treatment which wasslowly tapered
over a 1-year period. The lesion recurredhowever while tapering the
oral steroid after one year. Thus,oral Methrotrexate was commenced
to control theinflammation and as a steroid sparing agent. The
patientwas last seen in February 2019 and the conjunctival masshad
resolved completely. She remains stable. Her asthmawas also well
controlled after starting on oral steroid andmethotrexate.
DISCUSSIONChurg-Strauss syndrome, eosinophilic granulomatosis
withpolyangiitis, is a rare systemic autoimmune vasculitis
which
Recurrent bilateral eyelid and conjunctival granulomatosisin
Churg-Strauss syndrome
Nurul Faaiqah Jainuddin, MBBS1, Aliff Irwan Cheong, MOphthal1,
Chiew Seow Fan, MPatho2, Norlina Ramli,MOphthal1
1Department of Ophthalmology, Faculty of Medicine, University
Malaya, Kuala Lumpur, Malaysia, 2Department of Pathology,Faculty of
Medicine, University Malaya, Kuala Lumpur, Malaysia
CASE REPORT
This article was accepted: 17 November 2019Corresponding Author:
Dr. Nurul Faaiqah JainuddinEmail: [email protected]
19-Recurrent00110_3-PRIMARY.qxd 1/29/20 6:02 PM Page 86
Recurrent bilateral eyelid and conjunctival granulomatosis in
Churg-Strauss syndrome
Med J Malaysia Vol 75 No 1 January 2020 87
affects the small and medium-sized blood vessels. It was
firstdescribed by Churg and Strauss in 1951. The criteria
todiagnose CSS according to the American College ofRheumatology
include asthma, eosinophilia of more than10% in peripheral blood,
paranasal sinusitis, pulmonaryinfiltrates, histological proof of
vasculitis with extravasculareosinophils and mononeuritis multiplex
or polyneuropathy.1
The presence of four or more criteria yields a sensitivity of85%
and a specificity of 99.7%.2 Our patient had four of
thesefeatures.
CSS classically evolves in three stages which starts with
aprodromal asthma and allergy phase, followed by peripheralblood
eosinophilia and infiltration phase and finally with avasculitis
phase.3 This phase can occur up to 8-10 years afterthe asthma phase
and is often associated with extravasculargranulomas.
CSS usually affects the skin, heart, lung and
gastrointestinaltract. Ocular involvement in CSS is unusual. It can
manifestas conjunctival nodules, orbital myositis,
orbitalinflammatory syndrome, dacryoadenitis and cranial
nervepalsy.3-5
Management of CSS requires a team effort, usually with
arheumatologist and a general physician. Primary therapy forCSS is
systemic prednisolone for 6 to 12 weeks until remissionand then the
dose is tapered gradually. The majority of casesrespond well to
systemic prednisolone alone, but isolatedcases may need
immunosuppression to induce remission. Inour case, systemic
methotrexate was required to maintainremission as her symptoms
would recur once the oralcorticosteroids were stopped. Although
ocular involvement israre, the ophthalmologist must have high index
of suspicionto diagnose CSS from its ophthalmic manifestations. The
clueto this in our patient was her history of rapid response to
oralsteroids and then recurrence once oral steroid is stopped. If
indoubt, a biopsy of the lesion is essential for diagnosis. This
iscrucial as untreated CSS has 50% risk of death within threemonths
of vasculitis onset. With treatment, survival rateimproves to 70%
to 90% at 5 years.4
In conclusion, although CSS is rarely encountered in ourclinical
practice, it is important to have a high index ofsuspicion
especially in patients with systemic illness andrecurrent disease.
Prompt medical work up and biopsy of thelesion will help to
diagnose CSS which is a potentially life-threatening illness.
ACKNOWLEDGEMENTThe authors would like to acknowledge the patient
for theinformation and description.
CONFLICT OF INTERESTAll authors declare no conflicts of
interest.
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LH, et al. The American College of Rheumatology 1990 criteria
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1094-100.
2. Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross
WL, et al.Nomencalture of systemic vasculitides. Proposal of an
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187-92.
3. Akella SS, Schlachter DM, Black EH, Barmettler A.
OphthalmicEosinophilic Granulomatosis with Polyangiitis
(Churg–Strauss Syndrome)A systemic review of Literature. Ophthalmic
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4. Messmer EM, Miller CV, Kampik A. Conjunctival Granulomatosis
inChurg-Strauss Syndrome. Arch Ophthalmol 2012; 130(9):1228-9.
5. Ameli F, Phang KS, Masir N. Churg-Strauss syndrome presenting
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2011; 66(5):517-9.
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