Orbital and adnexal sarcoidosis: Clinical presentations and management outcomes

Abstract

Purpose:

To present the clinical features and management outcomes in a series of patients with orbital and adnexal sarcoidosis.

Methods:

This was a retrospective analysis of 19 histopathologically proven cases of orbital and adnexal sarcoidosis over the past ten years. The data analyzed included demographic details, clinical and imaging features, and management outcomes. The response to treatment was categorized based on clinical improvement at the last follow-up as complete resolution, partial resolution, or no resolution.

Results:

There were 15 (78.9%) females and four (21%) males. The mean age at presentation was 51.05 ± 14.35 years. Upper lid swelling was the most common sign (n = 6), followed by lacrimal gland enlargement (n = 5). The specific locations of involvement were orbital soft tissues (n = 8), lid (n = 5), lacrimal gland alone (n = 3), lacrimal sac (n = 1), extra ocular muscles (n = 1), and conjunctiva (n = 1). An incisional biopsy was performed on 16 patients, and an excisional biopsy on three. Of those undergoing excisional biopsy, the lesion was located in the superonasal extraconal space in one and in the superotemporal lid in two. Twelve patients (63.2%) had systemic involvement at presentation. No further treatment was necessary for patients who underwent excisional biopsy. Additional treatments included oral steroids (n = 10) and systemic immunosuppressants (n = 5). Six patients were lost to follow-up. Complete resolution of signs and symptoms was noted in 11 patients (84.6%), while partial resolution was seen in two patients. The mean duration of follow-up was 7.56 ± 12.63 months.

Conclusion:

Sarcoidosis can affect any part of the orbit or adnexa, with varied presentations depending on the involvement. Excisional or incisional biopsies, along with systemic steroids and immunosuppressants in select cases, remain the mainstay of treatment.

Sarcoidosis is a multisystem granulomatous disease of unknown etiology. The incidence is more common in females compared to males. Although the lungs are the most commonly affected, sarcodosis can affect any organ of the body. Ophthalmic involvement has been reported in 10%–50% of cases, with uveitis being the most common ocular manifestation.[1] Orbital and adnexal involvement is rare, occurring in 8%–28% of systemic sarcoidosis cases.[2,3,4] Among the periocular tissues, the lacrimal gland is most commonly involved, followed by the orbit, eyelids, and rarely, the lacrimal sac.[2,3,4,5,6,7] To date, only six large case series on orbital and adnexal sarcoidosis have been published.[2,3,4,5,6,7] The remaining literature consists of isolated case reports or small case series. The present study is the first from the Indian subcontinent to describe the clinical presentations, radiological features, and management outcomes in a series of patients with orbital and adnexal sarcoidosis.

Methods

The study adhered to the tenets of the Declaration of Helsinki and was approved by the institutional review board. A retrospective analysis of 19 histopathologically proven cases of orbital and adnexal sarcoidosis over the past ten years (January 2010 to December 2020) at two tertiary eye care centers was conducted. All cases with noncaseating epithelioid granuloma on histopathological examination were included in the study.

The data analyzed included demographic details, clinical and imaging features, previous diagnosis, and management outcomes. Orbital soft tissue involvement was classified as discrete (involving 1–2 quadrants) or diffuse (involving more than 2 quadrants). The response to treatment was categorized based on clinical improvement at the last follow-up as complete resolution, partial resolution, or no resolution. Patient consent was obtained for the use of clinical and radiological photographs.

Results

A total of 19 patients with a confirmed diagnosis of orbital or adnexal sarcoidosis were analyzed. Of these, 15 (79%) were females and four (21.1%) were males. The mean age at presentation was 51.05 ± 14.35 years (range: 17–73 years; median: 52 years). Bilateral involvement was seen in five patients (26.3%). The right eye was affected in eight patients (42.1%), and the left eye in six (31.6%). The demographic details, clinical features, initial diagnosis, and management outcomes are summarized in Table 1.

Table 1.

Demographic details, clinical features, and management outcome

Parameters		Number and Percentage
Total number of patients		19
Mean age		51.05+/-14.35 years (Range: 17-73 years, Median: 52 years)
Laterality		Right eye -8 (42.1)
		Left eye -6 (31.6)
		Bilateral- 5 (26.3)
Sex		M: F=15: 4
Mean duration of symptoms (months)		3.03+/–1.95 months (Median: 3 months, Range: 0.5 – 8 months)
Signs		Upper lid swelling- 6 (31.6)
		Lacrimal gland enlargement -5 (26.3)
		Palpable orbital mass-6 (31.6)
		Proptosis-5 (26.3)
		Lower lid swelling-4 (21.1)
		Conjunctival congestion-3 (15.8)
		Ocular movement restriction -3 (15.8)
		Induration-2 (10.5)
		Yellowish palpebral conjunctival nodule-1 (5.3)
Initial Diagnosis		Idiopathic Orbital Inflammation (IOID)-4 (21.1)
		Lymphoproliferative disorder – 3 (15.8)
		Amyloidosis -1 (5.3)
		Thyroid Eye Disease – 1 (5.3)
		Sarcoidosis -1 (5.3)
		Parasitic cyst-1 (5.3)
		Neurofibroma-1 (5.3)
		Nodular scleritis-1 (5.3)
		Primary nasolacrimal duct obstruction-1 (5.3)
		None – 5 (26.3)
Location		Isolated lacrimal gland		3 (15.8)
		Orbital soft tissue		8 (42.4)
		Lids		5 (26.3)
		Lacrimal sac		1 (5.3)
		Extroacular muscle		1 (5.3)
		Conjuncitva		1 (5.30
HRCT chest findings		Mediastinal Lymphadenopathy-10 (52.6)
		Reticular pulmonary interstitial thickening- 1 (5.3)
		Lung nodules- 1 (5.3)
		Normal- 7 (36.8)
Modalities of management		Incisional biopsy-16 (84.2)
		Excisional biopsy-3 (15.8) (superonasal extraconal space -1, upper lid -2)
		Biopsy + oralcorticosteroids-5 (26.3)
		Biopsy + oralcorticosteroids + oral methotrexate- 5 (26.3)
Outcome		Complete resolution-11 (84.6)
		Partial resolution-2 (15.4)
Lost to follow -up		6 (31.6)

The most common presenting complaint was periocular swelling, observed in 15 patients (79%) [Fig. 1a – f], followed by pain in four patients (21.1%). Other presenting symptoms included double vision, drooping of the lids [Fig. 1d], watering, redness, and discharge (n = 2 each, 10.5%). The mean duration of symptoms was 3.03 ± 1.95 months (median: 3 months, range: 0.5–8 months). Upper lid swelling [Fig. 2a] was the most common clinical sign (n = 6, 31.6%), followed by lacrimal gland enlargement (n = 5, 26.3%) [Fig. 2b], palpable orbital mass (n = 6, 31.6%), and proptosis (n = 5, 26.3%). The remaining clinical signs are listed in Table 1. All patients presented with one or more clinical features of inflammation. Bilateral lacrimal gland enlargement was noted in two patients [Fig. 2]. No intraocular involvement was observed in any of the patients, and there was no history of tuberculosis in any of the patients.

Figure 1.

(a) External color photograph of a 17-year-old girl showing bilateral lower lid swelling (b) Magnetic resonance imaging (MRI) orbit, coronal T2-weighted sections, showing homogenous, isointense, symmetrical soft tissue lesions involving bilateral lower lids (c) Intraoperative appearance displaying multiple yellowish nodular masses in the lower lid (d) External color photograph of a 60-year-old male presenting with wing bilateral periocular swelling (e) Subconjunctival yellowish nodules on the lower palpebral conjunctiva, visible upon retraction of the lower lid; (f) MRI orbit, coronal cuts, T2-weighted images, showing bilateral isointense, ill-defined soft tissue lesions enveloping the globe

Figure 2.

(a) External color photograph of a 52-year-old lady showing bilateral upper lid swelling (b and c) Enlarged and slightly congested palpebral lobe of the lacrimal gland bilaterally (d) Computed tomography (CT) scan, axial cuts, showing bilateral lacrimal gland enlargement with maintained shape of the gland

Orbital imaging was performed in all cases except for two. One of these cases involved presumed nasolacrimal duct obstruction (NLDO), while the other had a bulbar conjunctival nodule without any orbital involvement. Magnetic resonance imaging (MRI) was conducted in 16 cases (84.2%), and computed tomography (CT) was performed in one (5.3%) patient. Nonspecific orbital soft tissue involvement was seen in eight (42.1%) patients, while three patients (15.8%) had pure lacrimal gland involvement. Among the eight patients with orbital soft tissue involvement, diffuse lesions were noted in three patients (15.8%), while discrete lesions were seen in five patients (26.5%). Of the three patients with diffuse involvement, the lesions enveloped the globe [Fig. 1f] and were associated lacrimal gland infiltration in two patients and lid infiltration in one [Fig. 1e]. Imaging features were suggestive of an inflammatory etiology in most cases, except for three in which the imaging suggested well-defined mass lesions. The lesions were located in the upper lid in two patients and in the superonasal extraconal space in one.

The shape of the gland was maintained in cases of isolated lacrimal gland involvement, and the enlargement was symmetrical in bilateral cases [Fig. 2d]. Both the orbital and palpebral lobes were affected. On MRI, the enlarged lacrimal gland appeared isointense on T1-weighted sequences and showed an intermediate signal on T2-weighted sequences, with mild contrast enhancement. Mild enlargement of the extraocular muscles due to contiguous inflammation was observed in three cases. Bilateral symmetrical enlargement of the extraocular muscles, with tendon involvement but without adjacent soft tissue involvement, was seen in one patient. The muscles exhibited uniform hyperintense signals on T2-weighted images.

Eyelid involvement was seen in five (26.3%) patients, with two patients each having upper and lower eyelid involvement, and one having medial canthal involvement [Fig. 3a]. Of the two patients with lower lid lesions, one had bilateral involvement [Fig. 1b]. Imaging was performed in these cases as the involvement was often diffuse, and the posterior extent could not be assessed clinically. None of the patients had optic nerve or orbital apex involvement on imaging. The sites of involvement have been summarized in Table 1.

Figure 3.

(a) External color photograph of a 57-year-old lady showing yellowish conjunctival nodules (arrow) on the nasal bulbar conjunctiva (b) Histopathology of the conjunctival biopsy showing multiple discrete granulomas in the conjunctival stroma, composed of epithelioid cells and multinucleated giant cells with minimal surrounding lymphocytic infiltrate (stain: eosin and hematoxylin, magnification- 400×); (c) Post-contrast CT scan of the chest, thin axial sections, showing mildly enhancing symmetrical hilar and mediastinal lymphadenopathy (d) External photograph at 4-month follow-up showing complete resolution of the conjunctival nodules

An incisional biopsy was performed in 16 (84.2%) patients [Fig. 1c] and an excisional biopsy in three patients (15.8%). Of these, one biopsy was located in the superonasal extraconal space, while two were in the superotemporal lid. Histopathological examination showed multiple foci of discrete noncaseating granulomas comprising epithelioid histiocytes, a few Langhans giant cells, and scattered lymphoplasmacytic infiltrates, all suggestive of granulomatous inflammation [Fig. 3b]. Gomori methenamine silver (GMS) staining was done in all cases and was negative for fungus.

HRCT of the chest was performed in all cases except for two. Bilateral mediastinal lymphadenopathy was noted in 10 patients (52.6%) [Fig. 3c], of which one case had additional bilateral reticular pulmonary interstitial thickening and another had lung nodules. Normal pulmonary imaging was noted in seven patients (36.8%). Although two cases exhibited lung involvement, only one patient reported a history of cough when asked retrospectively. She also had bilateral mediastinal lymphadenopathy. None of the cases reported any history of pulmonary sarcoidosis.

Serum angiotensin-converting enzyme levels were raised in eight patients (42.1%), normal in six patients (33.3%), and was not assessed in five (26.3%) patients. Of the eight patients with elevated serum ACE, five had systemic involvement in the form of mediastinal lymphadenopathy. Both serum calcium and lysozyme levels were normal in nine (47.4%) patients and not assessed in ten patients (52.6%).

Six cases (31.6%) were lost to follow-up after surgery. The remaining 13 patients were reviewed by a rheumatologist and pulmonologist. Three patients did not receive any further medical treatment owing to the complete removal of the lesions. Ten patients (52.6%) were started on oral steroids at a dose of 1 mg/kg/day, with a slow tapering schedule depending on the severity and treatment response. Five (26.3%) of these 10 patients received subsequent concurrent immunosuppression in the form of oral methotrexate due to inadequate response to oral steroids. Patients with lacrimal sac and conjunctival sarcoidosis [Fig. 3a, d] were additionally treated with a course of topical fluorometholone eye drops, tapered over 4 to 6 weeks. The duration of steroid treatment ranged from 1.5 to 8 months (mean: 3.55 ± 2.35 months). The duration of oral methotrexate ranged from 2 to 3 months. The dose and duration of methotrexate were determined by the rheumatologist based on the patient’s response to treatment. The mean duration of follow-up was 7.56 ± 12.63 months (median: 3 months, range: 0.25– 48 months).

Complete resolution of signs and symptoms was noted in 11 patients (84.6%), while partial resolution was noted in two patients (15.4%). Of the two patients with partial resolution, one had lesions in the superior orbit with infiltration of the adjacent superior rectus and LPS complex, and the second had lesions in the inferior extraconal space. No further treatment was administered in these case, as there was significant resolution of clinical features on subsequent follow-up. No recurrences or complications were reported by any of the patients at the last follow-up.

Discussion

The present study comprises of 19 biopsy-proven cases of orbital and adnexal sarcoidosis. The most common presentation was periocular swelling. Nonspecific orbital soft tissue lesions were found to be the most common type of involvement (42.1%), compared to lacrimal gland enlargement (15.7%). Initial misdiagnosis occurred in 68.4% of patients, most commonly as idiopathic orbital inflammatory disease or lymphoproliferative lesions. Oral corticosteroids were the first line of treatment, with complete resolution noted in 57.9% of patients without any recurrence.

The criteria for the diagnosis of orbital and adnexal sarcoidosis have been debatable. A few studies suggest that the diagnosis of orbital and ocular adnexal sarcoidosis can be made only with supporting evidence of systemic disease.[3,8] In contrast, a few other authors believe that histopathologically proven orbital and adnexal sarcoid, even in the absence of systemic involvement, qualifies for the diagnosis of sarcoidosis.[2,4,5] The term “sarcoid-like granulomatous orbitopathy” (SLGO) has been used by some authors to describe cases where the patient had solely orbital disease or concurrent systemic involvement.[7] Concurrent systemic sarcoidosis may be present at the time of diagnosis of orbital sarcoidosis or may develop many years later.[5] The disease was found in eight cases during systemic investigations, while it developed in another two during follow-up.[5] The present study has considered all biopsy-proven cases of orbital and adnexal sarcoidosis, even in the absence of supporting systemic findings. The differential diagnosis of granulomatous orbital inflammation includes sarcoidosis, tuberculosis, orbital fungal infections, granulomatosis with polyangiitis, parasitic infections, or rarely, a ruptured dermoid cyst. All other possible causes of noncaseating granuloma were ruled out by appropriate investigations (granulomatous workup), including GMS staining for fungus, polymerase chain reaction (PCR) for Mycobacterium tuberculosis, QuantiFERON-TB Gold, or cartridge-based nucleic acid amplification test (CBNAAT) of the sputum to rule out tuberculosis. The tuberculosis work up was conducted in these patients for two key reasons: first, to rule out latent tuberculosis, which can reactivate after corticosteroid treatment for sarcoidosis, and second, to address the possibility of tuberculosis associated with noncaseating granulomas.

There is an increased risk of neoplasia in organs affected by sarcoidosis, especially lymphoma, which has an incidence 11 times higher in these cases compared to the normal population.[9] Hence, a biopsy is warranted in all cases, regardless of the presence of systemic sarcoidosis. A review of the literature on previously reported studies of orbital and adnexal sarcoidosis is summarized in Table 2.

Table 2.

Review of literature of cases presenting with orbital and adnexal sarcoidosis

Study		Numbr of subjects		Most common presentation		Location		Systemic involvement		Raised Serum ACE level		Treatment modalities		Mean Follow up (month)		Outcome
Collison et al.[2] (1986)		15		NA		Lacrimal gland -60% Orbital fat -40%		Present in 93.33%		NA		NA		NA		NA
Prabhakaran et al.[3] (2007)		26		Mass/swelling - 88.5%		Lacrimal gland - 42.3% Orbit-38.5% Eyelid - 11.5% Lacrimal sac - 7.7%		Hilar lymphadenopathy - 65.4%		57.69%		Oral steroid - 73.1% Surgical Debulking- 38.5% Methotrexate- 15.4% Intra-orbital steroid injection - 3.8%		18.75		Complete response-84.6% Partial- 7.69% Progression-7.69
Mavrikakis and Rootman[4] (2007)		20		Palpable mass -65%		Lacrimal gland - 55% Orbit- 20% Optic nerve sheath and dura - 20% Extraocular muscle- 5%		Lung – 45% skin and skeletal muscle – 5%		20%		Oral steroid- 45% Oral and intralesional steroid – 15% Intralesional steroid alone- 40%		NA		Recurrence – 30%
Demirci and Christianson[5] (2011)		30		Eye lid swelling -63%		Lacrimal gland -63% Eyelid - 17% Orbit - 13% Lacrimal sac - 7%		Lung - 67% Skin -33% Gastrointestinal tract -8%		NA		Oral steroid- 50% Excision - 27% Observation- 23%		44		Regression - 70% Stable - 27%
Vahdani and Rose[6] (2020) (included only patients with sarcoidal myopathy)		20		Eye lid swelling -95%		Superior rectus/LPS complex-50% Lateral rectus-40% Inferior rectus-25% Medial rectus-20% Inferior oblique-20% Superior oblique-10%		Upper airway -15% Lower airway-55% Lung, skin, nodes-55%		29%		Observation-20% Oral steroids-80% Second- line immunosuppressants-25%		56		Improvement -65% Recurrence 30%
Vahdani and Rose[7] (2021) (excluded patients with isolated lid and lacrimal drainage system)		61		Eye lid swelling -87%		Lacrimal gland- 52% Diffuse orbital fat -20% Lacrimal gland and fat -28%		Upper airway-16% Lower airway -64% Elsewhere -33%		48% (of 48 patients with available titers)		Observation -38% Oral steroids -62% Second- line immunosuppressants – 15%		53.4		Improved -87% No change -5% Incomplete records-8%
Present study (2021)		19		Periocular swelling -79%		Orbital soft tissue -42.1% Isolated Lacrimal gland – 15.8% Lid –26.3% Lacrimal sac- 5.3% Extraocular muscle- 5.3% Conjunctiva –5.26%		Bilateral mediastinal lymphadenopathy -52.6% Lung –10.5%		42.1%		Excision biopsy -15.8% Incisional biopsy- 84.2% Biopsy + corticosteroids- 26.3% Biopsy+corticosteroids+oral methotrexate- 26.3%		7.56		Complete resolution – 57.9% Partial resolution- 10.5% No follow up – 31.6%

NA – not available :LPS – levator palpebrae superioris

The most common presentation noted in the present study was periocular swelling, consistent with existing literature.[3,5,7] None of our patients reported any symptoms of dry eye, although five patients had lacrimal gland involvement. According to the literature, associated pain was seen in very few cases (21.05%).[3] This distinction is important for clinically distinguishing these lesions from idiopathic orbital inflammation, where pain is an important clinical finding.

Sarcoidosis can affect any tissue in the orbit and adnexa, including the lacrimal gland, orbital soft tissues, eyelids, lacrimal sac, extraocular muscle, optic nerve, and conjunctiva.[2,3,4,5,6,7] Although the lacrimal gland is the most commonly involved site reported in the literature, orbital soft tissue involvement (n = 8, 42.11%) was more frequent in the present study.[2,3,4,5,6,7] In the case of nonspecific orbital soft tissue involvement, a predilection for the antero-inferior orbit has been noted in the literature, although we did not observe this preference.[3] In all three cases of diffuse orbital soft tissue sarcoidosis, imaging showed characteristic molding around the globe, a feature typically associated with lymphoproliferative lesions.[10]

Lacrimal sac sarcoidosis is very rare and has been reported to be associated with upper respiratory system sarcoidosis.[11] It may be accompanied by nasal congestion and/or medial canthal lesion.[11,12] In cases of sarcoid involvement, the lacrimal sac and nasal mucosa are often found to be abnormally thickened, yellowish, crusty, edematous, friable, and sometimes nodular.[11,13] Studies indicate an increased risk of dacryocystorhinostomy failure in these patients.[12], likely due to ongoing inflammation at the sight of nasolacrimal anastomosis. A large rhinostomy ostium and the intraoperative placement of a stent have been suggested to increase the success rate.[13] In the present study, there was only one case of lacrimal sac sarcoidosis, which was initially misdiagnosed as primary acquired nasolacrimal duct obstruction. This patient underwent external dacryocystorhinostomy with silicone intubation. Intraoperatively, the lacrimal sac appeared abnormally thickened, prompting the decision to send a specimen for histopathological examination. The patient had no history of nasal symptoms. Nasolacrimal duct obstruction secondary to sarcoidosis is often misdiagnosed as PANDO, and a high index of suspicion is required both preoperatively and intraoperatively to accurately diagnose these cases.[12]

A total of five patients had isolated eyelid involvement, all of whom presented with progressive, painless swelling of the affected lids. In sarcoidosis, full-thickness eyelid involvement can distort the lid architecture. Posterior lamellar involvement may result in complication such as entropion, forniceal shortening, and symblepharon formation.[14] Mukherjee et al.[15] reported a case of eyelid sarcoidosis extending to the medial canthus, accompanied by erythema. Initially, the case was misdiagnosed as dacryocystitis but was later confirmed as eyelid sarcoidosis through biopsy. The patient also had associated hilar lymphadenopathy.

Conjunctival sarcoidosis usually presents with yellowish sub-conjunctival nodules, which can resemble chronic follicular conjunctivitis. Other presentations include conjunctival deposits, large conjunctival tumors, multilobular limbal nodules, and limbal conjunctival hypertrophy.[16,17] In the present study, there was one case of conjunctival involvement, which presented as a bulbar conjunctival nodule. This case was initially misdiagnosed as nodular scleritis. A biopsy was considered after the patient showed no response to standard therapy.

A single case with bilateral extraocular muscle sarcoidosis was initially diagnosed and treated as thyroid ophthalmopathy before presenting to us. However, upon further investigations, the thyroid function test results were within normal limits, and imaging showed diffuse extraocular muscle enlargement, including tendon involvement. A biopsy of the inferior oblique muscle confirmed the diagnosis of sarcoidosis. The superior rectus -levator palpebrae superioris muscle has been reported as the most commonly involved extraocular muscle in sarcoid orbital myopathy.[6]

Further disease progression was not seen in any of our patients, although the literature documents cases of subsequent bilateral lacrimal gland enlargement and swollen optic discs at 3-month follow-up in a case of eyelid sarcoidosis treated with debulking.[3] Unilateral to bilateral progression of the disease has also been reported.[3] Recurrence can occur in approximately 30% of cases.[4] The incidence of intraocular disease in association with orbital and adnexal sarcoidosis ranges from 3%–15%.[3,5] There was no associated intraocular sarcoidosis in any of our study subjects. None of our study participants developed pulmonary or other organ recurrence during the follow-up period. Nevertheless, Kaplan-Meier survival analysis in a study of 19 patients with orbital and adnexal sarcoidosis, with no evident systemic sarcoidosis at presentation, showed an 8% risk of developing systemic disease sarcoidosis over the next 5 years.[5] This underlines the importance of long-term follow-up in patients with isolated orbital sarcoidosis.

The management of orbital and adnexal sarcoidosis varies based on the tissue involved, extent of the disease, and presence of associated systemic involvement. Treatment options include observation, oral corticosteroids, surgical debulking, complete excision, and systemic immunosuppressants.[2,3,4,5,6,7] Among these, oral corticosteroids remain the mainstay of treatment.[3]

Literature indicates that 95% of patients with orbital and adnexal sarcoidosis show a complete to partial response to systemic steroids alone.[3] For cases without active systemic disease, a standard course of oral steroids can be initiated at a dose of 1 mg/kg/dose, with gradual tapering over 2–3 months. In case where the response is inadequate or there is no response or if patients are intolerant to oral steroids, additional immunosuppressants may be added to the therapy. Methotrexate is the most commonly used disease-modifying antirheumatic drug in these instances.[18] However, it can take up to 6 months to exhibit therapeutic effects, and the treatment duration can vary depending on the response.[18] Other less commonly used agents include azathioprine, mycophenolate mofetil, and cyclosporine.[19] For patients intolerant to oral steroids, intra-orbital steroid injection offer an alternative.[3,5] Additionally, isolated conjunctival sarcoidosis has been shown to respond well to topical cyclosporine (0.05%).[20] Oral minocycline has also been tried successfully for treating sarcoidosis in a patient with cutaneous, conjunctival, and choroidal lesions.[21] The mechanism of action is thought to be inhibition of T-lymphocyte activation and proliferation.[22] All patients with systemic involvement should be managed by a pulmonologist and rheumatologist for comprehensive care.

The retrospective design, absence of follow-up imaging to assess objective resolution of the lesion, a relatively short follow-up period, and the lack of follow-up in some patients are some of the drawbacks of the present study.

Conclusion

Similar to systemic sarcoidosis, orbital and adnexal sarcoidosis can mimic various other orbital pathologies as it involves different periocular soft tissues and present with a range of signs and symptoms. Diagnosis is confirmed by identifying noncaseating granuloma on histopathology, after excluding other possible etiologies. Elevated serum angiotensin-converting enzyme levels and pulmonary involvement seen on HRCT chest scans serve as supportive evidence. The primary treatment includes systemic steroids, with immunosuppressants used in select cases. A high index of suspicion, early diagnosis, and a multidisciplinary approach involving an ophthalmic plastic surgeon and pulmonologist are crucial for achieving complete resolution of the disease.

Conflicts of interest:

There are no conflicts of interest.

Funding Statement

Nil.