Abstract

Sarcoidosis is a multisystem granulomatous disease with an unknown aetiology. It has a worldwide prevalence, but variable incidence among different geographical regions. The disease affects adults between 20 to 40 years of age and it is slightly more common in women than men. Eyes are affected only in one fourth of cases and they are asymptomatic. There is increased production of TH1 cytokinesis. The TNF is important in propagating inflammation in sarcoidosis. Gallium 67 citrate scan will reveal the chronic course of the disease even if X-ray chest is normal. Local steroid injection or surgical excision of the affected areas may be useful in selected patients.

Introduction

Sarcoidosis, an multisystemic disease, may affect the entire visual apparatus and its adnexa. The eyes are affected only in one fourth of cases and majority of the lesions are asysmptomatic, requiring appropriate examination technique to be detected.1 Sarcoidosis is a systemic chronic granulomatous disease of unknown aetiology characterised by non-caseating granulomatous inflammation of various organs with protean clinical manifestation. Sarcoidosis commonly involves the eye causing uveitis, lacrimal gland and cranial nerves including the optic nerve itself.

Case Report

We present a case of Sarcoidosis outside the lungs presenting as lacrimal gland with swelling on right eye since 2 months. Pt XY Male Age 55 years coming from Gujarat (Fig. 1).

H/O Diabetes mellitus since 4 years.

H/O Essential Hypertension since 10 years.

Fig. 1 :Showing lacrimal gland swelling.

H/O Dryness of effected Eye.

No H/O fever, Arthalgia, dermatology lesion, Anorexia neurological deficit, fungal, viral, Chlamydia infection.

Cardiovascular and Respiratory status normal.

Lacrimal Glands swelling 1 x 1.5 cms.

Shirmer's Test : 10 mm

Tear film break up time : 10 secs.

Rose Bengal staining of conjunctiva : Negative

Occular Movements : Normal.

Investigation

• X-ray chest normal.

CBC	Hb 14.8 gms/dl, RBC 5.03 mill/cu.mm
	WBC 8,300/cu.mm N65 E02 L33.
	PCV 49 MCV 86.2 MCH 28.3 MCHC 29.4
	Platelet 2.74 lacs/cu.mm.

• ESR 70 mm at the end of one hour (Western gren)
• Angiotensin Converting Enzyme (ACE) : - 70 U/ml.
Method ELISA (N.R. - 0-52 U/ml).
• Fasting Blood Sugar 126 mg%, Post prandial blood sugar 220 mg%,
• HBA1C 8.3%.
• M.R.I. Scan : - revealed swelling of lacrimal gland measuring 2 x 3 cms (Fig. 2).
• Biopsy of mass of right eye revealed noncaseating granuloma suggestive of Sarcoidosis (Fig. 3).

Treatment with prednisolone 40 mg daily for one month the lesion resolved completely.

Discussion

Familial Sarcoidosis is rare.2 It is unusual for lacrimal gland enlargement to be the initial manifestation of sarcoidosis. Sarcoidosis is a multisystemic inflammatory disease of unknown aetiology that predominantly affects the lungs and intrathoracic lymph nodes. Since there is no possible infectious aetiology evident so far, importance of autoimmune process being involved in the pathogenesis of sarcoid should be considered

Fig. 1 :Showing lacrimal gland swelling.	Fig. 3 :H and E section showing biopsy of mass of right eye revealing noncaseating granuloma suggestive of sarcoidosis.

because there is an accumulation of CD4 cells at the site of disease activity which is manifested clinically by an inverted CD4/CD8 ratio. There is increased production of TH1 cytokines such as interferon. TNF, the importance of these cytokines in propagating inflammation is demonstrated by Anti TNF agent such as pentoxifylline and infliximab.3-5 There is increase in fibrinogenic cytokines for e.g.:- transforming growth factor beta (TGF beta) around the sarcoid lesion. The patients serological test failed to demonstrate the presence of rheumatoid factor, antinuclear and anti ds DNA antibodies. No cryglobulins were detected. There was regression of clinical signs after treatment with glucocorticoid even though the patient was diabetic which was controlled by human insulin injection Actaprid Monotard. Metabolic monitoring was done by estimation of Glycoslyated Haemoglobin (GHbA1c). Since the patient was an diabetic his immune response is sluggish ie the subsets of Thelper cells for e.g.. TH1 and TH2. TH1 cells normally boost cell mediated immunity. TH2 cells seem more important in generation of secretory immune response in the mucosa.6,7 The TH2 cells in diabetes are virtually absent which may account for the pathogenesis of sarcoidosis. T cells play a central role in the development of sarcoidosis as they likely propagate an excessive cellular immune response. There is an accumulation of CD4 cells accompanied by the release of interleukin (IL)-2 at the site of disease activity. This may be manifested clinically by an inverted CD4/CD8 ratio Study of Bronchoalveolar lavage (BAL) in occular sarcoidosis may be of value in refractory cases where the diagnosis is doubtful. In such cases BAL will have lymphocytosis with predominant T4+ helper cells.8,9 Tear specific protein would also help in refractory cases of occular sarcoidosis.10

Even though our patient was showing normal X-ray chest but still Gallium 67 citrate scan would reveal the chronic course of the disease or regression of the lymph node. However gallium scanning is a sensitive method in localising chronic occular affective in cases in which the lung changes are no longer detectable.11-13

The literature also mentions a new method in evaluating the anatomy and patency of the lacrimal drainage. Thermography is found to be safe simple technique.14 We had even evaluated the biopsy sample for mycobacterial tuberculosis, but negative because initially it was thought that the mycobacterial antigen plays a role in granuloma formation with sarcoidosis.15

Evaluating the diagnostic and therapeutic procedure can be difficult because there are no standard guidelines. A vital threat, demands rapid diagnosis and effective therapeutic evaluation of the appropriate therapy especially in systemic disease associated with sarcoidosis.16

However in diabetes with sarcoid systemic steroid could be avoided since the immune response being sluggish systemically so in such cases local steroid injection or surgical excision of the affected areas may be useful in selected patients.17

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*Occular Pathologist,***Ophthalmologist, Akshar Eye Clinic. **HOD and Professor of Pathology; ****Professor of Pathology, CMPH Medical College, Vile Parle (W), Mumbai.