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  5. FP379 : Orbital Inflammatory Diseases- Clinical Features, Investigations and Treatment Outcomes.

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Dr. Teena Elizabeth George, T19793, Dr. Parvathi T H, Dr. Anjali Kiran, Dr. Gupta Roshmi

Introduction :

Orbital inflammatory disease(OID)commonly include the non-infectious conditions, ie, thyroid related ophthalmopathy, idiopathic orbital inflammatory diseases and systemic causes of inflammatory mass lesions. Malignancies, congenital mass lesions, infectious diseases, and trauma can mimic inflammatory orbital conditions. Orbital inflammatory disease is diagnosed after simulating conditions are ruled out. Orbital inflammatory diseases comprise non-specific orbital inflammatory disease(NSOID) and specific orbital inflammatory disease. A thorough history, clinical examination, and radiological examination will confirm diagnosis of OID and its location. Laboratory investigations and biopsy are required  to rule out less common causes of inflammatory conditions 1such assarcoidosis, Wegener’s granulomatosis, Crohn’s disease, systemic lupus erythematosis, and other connective tissue diseases, Churg–Strauss syndrome, Erdheim–Chester, histiocytosis X, and giant cell arteritis.

Even though NSOID shows good sensitivity to systemic corticosteroids, some forms of OID are poor responders and require other therapeutic modalities including radiation therapy or immune modulation.It is important to rule out underlying systemic diseases by laboratory tests and biopsy before starting empirical treatment of systemic steroids, as specific OID too show good response to steroids. Also specific OID can be isolated pathological lesions with no systemic involvement2. Hence it is important to understand the various etiopathological presentations of OIDs, its frequencies in our populationand its response to the conventional therapy.

Aim of the study:

  1. To understand the various conditions and clinical features of orbital inflammatory diseases.
  2. To evaluate various diagnostic modalities- including imaging, blood tests and biopsy in the management of orbital inflammatory diseases.
  3. To evaluate various treatment modalities used and their outcomes.

Methods and Materials:

A retrospective chart review of patients who had presented to the Orbit-Oculoplasty clinic in atertiary care centre with complaints suggestive of inflammatory orbital diseases from May 2014 to April 2017.

Inclusion criteria:

All patients with features suggestive of orbital inflammatory diseases were included.

Mass lesions with biopsy proven inflammation were also included.

Exclusion criteria:

  1. Patients with infective diseases like orbital cellulitis, orbital abscess.
  2. Patient with thyroid eye disease.
  3. Biopsy showing orbital malignancies.

Data collected on patients with orbital inflammatory diseases  included history for previous episodes, symptoms and any previous treatment taken,  physical examination , laboratory examination to rule for collagen vascular and autoimmune diseases, chest x ray and imaging of orbit. In selected cases tissue for diagnostic studies were taken. Biopsy was performed in included cases with atypical presentation, suspected systemic condition, recurrence and nonresponders to steroids.

Results:

85 eyes of 80 patients with non-infective orbital inflammation had presented from May 2014 to April 2017. Fifty patients(62.5%) were female and 30(37.5%) were male. Male: female ratio was 3:5.

Mean age was 42.8 years. Age ranges from 9-78yrs. Mean interval from symptoms to presentation at our clinic was 8.6 weeks.

Specific orbital inflammation was present in 16/80 (18.8%),whereas rest 64/80 (81.2%) had non-specific orbital inflammatory disease.

49/80 (62%) of the cases received naïvetreatment prior to referral.

62/80(77.5%) patients presented with inflammatory signs and the rest 18/80 (22.5%) had presented as painless mass.

28/80 (35 %) patientshad presented as recurrence.

Localisation sites were analysed: commonest was dacryoadenitis in 45/80 (57.5%); others included myositis 17/80 (21.25%),5/80(6.3%) as anterior OI,3/80 (3.75%) as diffuse OI.Perineuritis was associated with 3 cases along with myositis/diffuse OID.6 cases had both involvement of muscles and lacrimal gland(combined myositis and dacryoadenitis). Associated dry eye was seen in 12 (15%) patients and compressive optic neuropathy in 5 (6.25%) cases, scleritis or uveitis in 8 cases (10%).

27/80 (33.8%) required biopsy.Biopsy showed specific inflammatory disease features in 16/27 (59%) of biopsied cases-6 cases of sclerosing inflammation, 4 cases of sarcoidosis, 2 cases of IgG disease, 3 cases of kimura’s diseases and 1 case of granulomatosis with polyangiitis.

Thorax imaging was positive in 4/80,all of which had a granulomatous inflammation on biopsy. Blood tests for autoimmune/collagen vascular disease was positive in 13/42 (31%).Treatment was started with oral steroids in 54/80 (67.5%) cases. A recurrence was noted in 15/80 (19%) cases with initial oral steroids only.Additional immunosuppressant were given in 23/80 (29%)either initially for specific inflammation or on recurrence with steroids. 19 cases were lost to follow up.At the last visit,in patients with 2 or more visits and known outcome (61 cases), no recurrence was note in 46/61 (75%) patients. A residual inactive mass lesion was seen in7/61 (11.5%)cases.

Discussion:

Orbital inflammatory disease accounts for up to 6% of orbital diseases.3Orbital inflammation can be the presenting face for a vast array of conditions either primary inflammation or secondarily induced by other lesions and both will commonly show a corticosteroid response.The spectrum of orbital inflammatory disease ranges broadly from specific diseases like Wegener’s granulomatosis or sarcoidosis, to nonspecific inflammation which may involve one or multiple structures of the orbit. These conditions can be roughly grouped into two categories, specific orbital inflammatory disease (SOIS) and nonspecific orbital inflammatory disease (NSOIS). Orbital inflammatory disease may be secondary to an underlying systemic inflammatory disease, which must be diagnosed in order to develop a comprehensive therapeutic plan, or may represent localized pathologic processes without systemic involvement. Non-specific orbital inflammation is a diagnosis of exclusion,hence is critical for ruling out other conditions prior to a definitive diagnosis of IOID. It is the third most common orbital disease after Grave’s orbitopathy and lymphoproliferative diseases.3 Twenty percent of non-thyroid orbital masses are idiopathic. 9

In this study, the male: female ratio in the study is 3:5and the mean age is 42.8 years. The peak incidence of non-specific OI is in the 4th to 5th decades of life and seems to have no gender predilection.3But according to Gordon et al Women appear to be affected more often than men in non-specific OID. 3IgG4-RD is more common in men and in patients older than 50years.12In Specific inflammatory diseases like sarcoidosis onset is typically between the ages of 20 and 40 years.4

Inflammatory signs were the most common presentation in this study even though ¼ th of the cases had presented as painless mass. A swelling/mass was the most common presentation followed by proptosis, pain, extraocular muscle restriction, diplopia, ptosis and decreased visionin Idiopathic OID5as well as specific orbital inflammation like sarcoidosis.6,18

Lacrimal gland was the most commonly involved gland in this group of orbital inflammations.Any orbital structure can be involved either focally or in a diffuse manner, with orbital fat beingthe common site of involvementin non-specific orbital inflammations.5Sarcoidosis is more likely to involve the lacrimal glands bilaterally.17

Systemic involvement was high in cases with biopsy proven granulomatous OID.

In review of cases of sarcoidosis a prior history of sarcoidosis is rare, but the chest imaging revealedhilarlymphadenopathy..7,8

The laboratory investigations for autoimmune/collagen vascular diseases were negative in 84% of orbital inflammatory cases in this study. 3 out of 16 cases of biopsy proven cases of specific orbital inflammatory cases were showed negative results on blood investigations.

The positive predictive value of serum angiotensin converting enzyme (ACE) or lysozyme in sarcoidosis or serum IgG4 titres in orbital IgG4-RD, is generally too low for routine use.11,12

Even though Anti neutrophil cytoplasmic antibody reactive to proteinase 3 (PR3-ANCA) is suggestive of GPA, and anti-SSA (Ro) or anti-SSB (La) of Sjögren syndrome, they remain undetectable in asignificant proportion of patients with solely orbital disease.13,14

1/3 rd cases of orbital inflammatory diseases had undergone biopsy. Of cases undergone biopsy, half of the cases showed specific inflammatory patterns on histopathology. The rest were non-specific orbital inflammation. The indications for biopsy were atypical presentation, bilateral presentation, recurrence and non responders to steroids.

Oral steroids were found to be effective as initial line of treatment with a recurrence in only 19 %. This was in contrast to a study by Mombaerts I et al who reported a cure and relapse rates at 37 and 52%,respectively with oral steroids.15,16

Conclusion:

Selective biopsy in orbital inflammation yielded significant specific inflammation. Blood tests for autoimmune disease and chest imaging are best advised in a targeted fashion, rather than in all patients. Initial treatment with oral steroids is effective. Immunosuppressants are useful adjuvant therapy in specific inflammation and in recurrent disease.

References:

  1. McCluskey P, Powell RJ. The eye in systemic inflammatory diseases. Lancet 2004; 364: 2125–2133.
  2. Mombaerts, I. The many facets of dacryoadenitis. CurrOpinOphthalmol 2015;26:399-407.
  3. Gordon LK. Orbital inflammatory disease: a diagnostic and therapeutic challenge. Eye 2006;20:1196-206.
  4. Thomas KW, Hunninghake GW. Sarcoidosis. JAMA 2003;289: 3300-3.
  5. B N Swamy, P McCluskey, A Nemet, R Crouch, P Martin, R Benger, R Ghabriel, D WakefieldIdiopathic orbital inflammatory syndrome: Clinical features and treatment outcomes Br J Ophthalmol 2007;91:1667–1670.
  6. Venkatesh C. Prabhakaran, PeroozSaeedOrbital and Adnexal SarcoidosisArch Ophthalmol. 2007;125(12):1657-1662
  7. King MJ. Ocular lesions of Boeck’ssarcoid. Trans Am Ophthalmol Soc. 1939;37: 422-458
  8. Kaplan M. Boeck’ssarcoid: report of a case with an unusual precipitating factor.Am J Ophthalmol. 1948;31:83-85.
  9. Ben Simon GJ, Yoon MK, Atul J, et al. Clinical manifestations of orbital mass lesions atthe Jules Stein Eye Institute, 1999-2003. Ophthalmic Surg Lasers Imaging 2006;37:25-32.
  10. Plaza JA, Garrity JA, Dogan A, et al. Orbital inflammation with IgG4-positive plasma cells: manifestation of IgG4 systemic disease. Arch Ophthalmol 2011;129:421-8.
  11. Radice A, Bianchi L, Sinico RA. Anti-neutrophil cytoplasmic autoantibodies: methodological aspects and clinical significance in systemic vasculitis. Autoimmun Rev 2013;12:487-95
  12. Wallace ZS, Deshpande V, Mattoo H, et al. IgG4-related disease: Clinical and laboratory features in 125 patients. Arthritis Rheumatol 2015;67:2466-75.
  13. Fechner FP, Faquin WC, Pilch BZ. Wegener’s granulomatosis of the orbit: a clinicopathological study of 15 patients. Laryngoscope 2002;112:1945-50.
  14. Parkin B, Chew JB, White VA, Garcia-Briones G, Chhanabhai M, Rootman J. Lymphocytic infiltration and enlargement of the lacrimal glands: a new subtype of primary Sjögren’s syndrome? Ophthalmology 2005;112:2040-7.
  15. Mombaerts I, Koornneef L. Current status in the treatment of orbital myositis. Ophthalmology 1997;104:402-8.
  16. Mombaerts I, Schlingemann RO, Goldschmeding R, Koornneef L. Are systemic corticosteroids useful in the management of orbital pseudotumors? Ophthalmology 1996;103:521-8.
  17. Carmody RF, Mafee MF, Goodwin JA, Small K, Haery C. Orbital and optic pathway sarcoidosis: MR findings. AJNR Am J Neuroradiol 1994;15:775-83.
  18. Yuen SJ, Rubin PA. Idiopathic orbital inflammation: distribution,clinical features, and treatment outcome. Arch Ophthalmol 2003;121:491-9.

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