Silent tears and the loud mimic.

Abstract

A 57-year-old gentleman presented with a bilateral (OU) upper eyelid swelling since a month along with gritty sensation in the eyes. There was no history of systemic manifestations or similar family history. On examination, best corrected visual acuity (BCVA) in OU was 20/20 and N6 with Schirmer’s of 5 mm in the right eye (OD) and 3 mm in the left eye (OS), and ocular surface staining OU indicating aqueous deficiency dry eyes. Bilaterally, upper eyelid fullness was seen temporally along with S-shaped drooping of the eyelids [Fig. 1]. The superior orbital margin was palpable in OU along with mild tenderness. The palpebral lobe of the lacrimal gland was prolapsed and congested with surrounding chemosis. There was mild restriction of ocular motility on abduction. There was no evidence of proptosis. Intraocular pressure was 16 mmHg in OU. Posterior segment examination was within normal limits.Figure 1: (a) Shows bilateral upper eyelid fullness laterally and S-shaped lateral droop; (b) shows bilaterally prolapsed and congested palpebral lobes of the lacrimal gland, Figure 1c shows axial cut of the CT orbit showing a diffusely enlarged isodense lacrimal gland bilaterally, with no additional bony changesWhat is the next step? Radiological investigations Multilevel incisional biopsy with histopathologic confirmation Collagen vascular profile All of the above Correct Answer D. All of the above Diagnosis and Management Table 1 shows the clinical characteristics of common lacrimal gland disorders. Considering the possibility of subacute dacryoadenitis involving the orbital and palpebral lobes of the lacrimal gland, radiological investigation was done in the form of computed tomography (CT), which showed bilaterally bulky lacrimal glands abutting the lateral rectus on both the sides along with slightly bulky appearance of the lateral recti. A multilevel incisional biopsy was performed for the patient, and histopathology and immunohistochemistry confirmed the diagnosis of IgG4-related dacryoadenitis. A detailed collagen vascular profile was done to evaluate all underlying collagen vascular diseases. Out of the panel, serum IgG4 was found to be raised, therefore correlating with the histopathology. The patient was treated with intravenous methyl prednisolone 500 mg pulse therapy for six pulses along with topical lubricants. He started responding by the fourth cycle and at the latest follow-up of 2 years and 8 months, he is doing extremely well with normalized serum IgG4 levels and resolution of ocular symptoms with Schirmer’s improved to 10 mm in OU.Table 1: Clinical characteristics of lacrimal gland disordersDiagnosis OU IgG4-related dacryoadenitis with severe dry eyes. Discussion Over the last two decades, the unidentified zone of IgG4-related disease has been duly explored. The disease mimics a broad spectrum of clinical entities, ranging from neoplastic to inflammatory and even infectious conditions, which makes it mandatory for the clinicians to understand the clinical presentation, possible differentials, management, and prognosis of IgG4-related disease. Ophthalmologically, the patients present with diplopia, ptosis, visual acuity, disturbance, ocular motility restriction, dry eyes, corneal ulcer, or epiphora.[1] Baseline radiological imaging followed by surgical biopsy would be appropriate.[2] Systemic correlation rules out its presentation as sialadenitis, pancreatitis, and lymphadenopathy as well.[1] Serum IgG4 levels are confirmatory as well as prognostic. The disease responds well to treatment with steroids, but has follows a chronic course with systemic morbidity if left undiagnosed and untreated as it causes end-stage organ failure.[3] Therefore, this possible differential diagnoses and the low remission rate of 23.8% must be kept in mind in a patient with dacryoadenitis presenting with dry eyes.[4] Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his names and initials will not be published and due efforts will be made to conceal his identity, but anonymity cannot be guaranteed.