Dr.Kiran Chandra Kedarisetti, K20816, Dr.Usha Singh
Abstract
Purpose: To analyse non-tumor dacryoadenitis that presented to a tertiary care centre in North India and its management options.
Methods: A retrospective case series of 13 patients diagnosed as chronic dacryoadenitis.
Results: Thirteen patients presented with Dry Eyes (11), Proptosis (5), S- Shaped deformity (5), Lid Swelling (2), Ptosis (2) and discharging Sinuses (1). All underwent a complete biochemical, radiological and disease specific tests. Tissue biopsy was sought in 10. The final diagnosis included presumed chronic tubercular dacryoadenitis (5), sarcoidosis (2), reactive lymphoid hyperplasia (5) and IgG4 disease (1). Treatment included ATT, steroids, anti-metabolities, surgical excision and radiotherapy.
Conclusions: In order to avoid misdiagnosis, mistreatment, and morbidity, complete systemic evaluation is needed. In an endemic region like ours, there should be high index of suspicion for tuberculosis.
Introduction
Dacryoadenitis is the inflammation of the lacrimal gland, with many different potential etiological possibilities which could be extensive. Aetiology is either inflammatory or infective.Typically, these patients initially present with discomfort, mild pain and dryness. However, presentation of dacryoadenitis may be quite variable.(1)
Acute cases usually present with more severe symptoms (red and swollen eyelids, warm, tender and enlarged glands, conjunctival congestion, chemosis and lymphadenopathy).Chronic dacryoadenitis has a less severe presentation than acute dacryoadenitis and is easily mistaken to be a benign tumour.(1)The tumours of the lacrimal gland also present with dry eye, palpable mass over lateral eye lid, ptosis and proptosis. A thorough systemic work-up and radiologic investigations can lead to differentiation between the two. Dacryoadenitis is associated with raised inflammatory mediators and a more defined enlargement of gland not invading into surrounding tissues. Computed Tomography scan of the orbits with contrast enhancement (CECT)of a lacrimal gland tumour usually reveals solid enlarged gland with occasional heterogenous calcification, bony remodelling, irregular margins, nodularity or bony destruction. A biopsy provides the definitive diagnosis.
The challenge in identifying the aetiology of dacryoadenitisis because it is a rare disease and may be the initial and only manifestation of a systemic disease. The underlying systemic illness needs to be treated, usually under the cover of steroids.
Materials and methods
The patients who presented to a tertiary care referral Institute in North India were studied from a time period between January 1998 – June 2018. A retrospective study of patients diagnosed as dacryoadenitis was done.
All these patients, at presentation, underwent a complete ophthalmic evaluation that included visual acuity, intra-ocular pressure, extra-ocular movements, complete anterior and posterior segment evaluation, Schirmer’s test and exophthalmometry. Radiological investigations, CEMRI/CECT of head and neck were done. A thorough general physical examination involving vitals, lymph nodes, respiratory, cardiovascular and gastro-intestinal system was done. Systemic work-up of the patients involvedHaematological tests: complete hemogram, differential leukocyte count, renal function test, liver function test, thyroid function test; Tuberculosis (TB)panel: Mantoux test, chest X-ray/ High- resolution computed tomography of chest (HRCT chest), TB QuantiFERON gold; Rheumatoid Work-up: Serum ACE (angiotensin converting enzyme) levels, Serum calcium, erythrocyte sedimentation rate andc-reactive protein. In patients with an equivocal diagnosis or poor resolution to just steroids a histopathological evaluation was done.
Results
Nineteen eyes of 13 patients were studied. The median age of presentation was 40 years (range: 14 – 56)with2 (17%) males and 10 (83%) females. The medianduration of symptoms was 3 months (range 1 day–1year). Unilateral involvement was seen in 7 patients while the other 6 were bilateral.
The presenting complaints were dry eyes(11), proptosis (5),S-shaped deformity (4), lid swelling (2), ptosis (2), discharging sinuses (1).CECT/ CEMRI was done in all patients that showed a heterogenous enhancement of lacrimal gland with no bone remodelling or invasion in surrounding tissue confirming the diagnosis of dacryoadenitis.
A systemic evaluation of all the patients was done. One patient a known case of Adult onset Still’s disease, was treated with steroids, considering dacryoadenitis a part of the spectrum of AOSD.
The rest of the patients underwent a complete TB work-up along with haematological and rheumatological examination. Those patients who were Mantoux positive (5), went through a pulmonary evaluation, were diagnosed either tuberculosis (1) or equivocal (4). A patient with enlarged mediastinal lymphadenopathy post HRCT was diagnosed sarcoidosis post pulmonology evaluation and was treated with steroids. Ten patients (equivocal or negative on the TB panel) underwent a histopathological evaluation. On biopsy, the final diagnosis involved tubercular dacryoadenitis (4), sarcoidosis (1), reactive lymphoid hyperplasia (3), Juvenile Xanthogranuloma(1) and IgG4 disease (1).
All were treated with steroids. Those diagnosed withTB dacryoadenitis were given anti-tubercular treatment, followed by steroids after 2 weeks. Two patients who required long term steroids were given immunosuppressants. The patient diagnosed IgG4 disease was given T. Methotrexate 2 weeklyand Still’s disease IV Tocilizumab (8mg/kg per month). Juvenile Xanthogranuloma patient was given radiotherapy post excision biopsy.
Discussion
TB Dacryoadenitis is 10% amongst orbital TB. (2) In a country like India, it is essential to keep Tuberculosis as a differential diagnosis in mind due to the high prevalence of the disease in our country.(3)Acid fast bacilli (AFB) staining and culture for Mycobacterium tuberculosis (MTb) are negative(4)possibly due to the paucibacillary nature of the disease. On starting ATT alone, there is worsening of the disease due to paradoxical increase in inflammation. Hence steroids are given to the patient.
Adult-onset Still’s disease (AOSD) is a systemic inflammatory condition that typically presents with a recurring spiking fever with a concomitant salmon pink rash and arthralgia. Orbital inflammation and dacryoadenitis are a rare complication associated with this disease.(5)
IgG4 related disease are syndromes characterized by elevated serum IgG4 (>135 mg/
dL), IgG4-positive lymphoplasmacytic infiltrative lesions in the body on histopathology (ratio of IgG4/IgG-positive cells >40% and >10 IgG4-positive plasma cells/HPF) and fibrosis of the affected organ. (6) IgG4-dacryoadenitis a diagnosis that was recently found to account for 23%–36% of idiopathic orbital inflammation and is confirmed by IgG4 immunostaining. IgG4 usually presents as bilateral lacrimal gland inflammatory conditions and a normal serum IgG4 level does not rule out the diagnosis. (7)
Juvenile Xanthogranuloma (JXG) is generally a rare inflammatory/ nonspecific injury disorder that occurs in the skin of infants or in early childhood as nodules. Extacutaneous JXGs in the central nervous system, lungs, heart, liver, testicles, bone marrow, and blood cells are rare. Ocular involvement of JXG includes the iris(most common), orbit, eyelid, conjunctiva, uvea, retina, and optic disc. (8) A histopathological examination reveals features of reactive granuloma containing foamy histiocytes, foreign body giant cells, Touton giant cells, with a surrounding wreath of nuclei, histiocytes, lymphocytes; and is necessary to make an accurate diagnosis of JXG. The disease responds well to steroids and excision in resistant cases.
Careful monitoring of response is needed as steroids maybe required over a long period. This is done through exophthalmometry, Schirmer’s test, serial photographs and haematological tests to rule out steroid induced complications. Male sex and inflammation
extending to extraocular muscle have been reported to have a poor prognostic outcome.(9, 10)
Conclusion
Careful history, examination, and focused investigations are warranted to diagnose dacryoadenitis and regular monitoring of response to corticosteroids is required to appropriately manage it.Dacryoadenitis is a highly misdiagnosed entity if treated empirically without appropriate tissue diagnosis. Biopsy remains the gold-standard for diagnosis of dacryoadenitis.The underlying systemic illness needs to be treated, usually under the cover of steroids.
References
- Witmer M, Tampa.Unravelling the Difficult Diagnosis of Dacryoadenitis. Review of Ophthalmology; 2009
- Madge SN, Prabhakaran VC, Shome D, Kim U, Honavar S, Selva D; Orbital tuberculosis: a review of the literature. Orbit. 2008;27(4):267-77.
- BabuK,Mukhopadhyay M, Bhat S, Chinmayee JT; Orbital and adnexal tuberculosis: a case series from a South Indian population. Journal of Ophthalmic Inflammation and Infection 2014, 4 – 12.
- Marta Ruman-ColombierM,Crisinel P, Cohen-Dumani N,et al; Bilateral Dacryoadenitis: Dont foget tuberculosis! The Pediatric Infectious Disease Journal,2017, 36 (1).
- Bannai E, Yamashita H, Takahashi Y, et al; Two Cases of Adult-onset Still’s Disease with Orbital Inflammatory Lesions Originating from the Lacrimal Gland. Intern Med 2015, 54: 2671-2674.
- Sato Y, Notohara K, Kojima M, Takata K, Masaki Y, Yoshino T; IgG4-related disease: historical overview and pathology of hematological disorders. Pathol Int 2010,60:247.
- Pei-Yuan Su ,Chien-Sheng Wu, Chang S, et al; IgG4-related dacryoadenitis Taiwan Journal of Ophthalmology 3 (2013) 116 – 119
- Samara W.A. Khoo C.T.L, Say E.A Saktanasate J. et; Juvenile Xanthogranuloma Involving the Eye and Ocular Adnexa. American Academy of Ophthalmology. 2015
- Andrew N, Kearney D, Sladden N et al; Idiopathic Dacryoadenitis: Clinical Features, Histopathology, and Treatment Outcomes. Am J Ophthalmol 2016;163
- Yeşiltaş Y and Gündüz A; Idiopathic Orbital Inflammation: Review of Literature and New Advances. Middle East Afr J Ophthalmol 2018 Apr-Jun; 25(2): 71–80.
Leave a Comment