|Year : 2022 | Volume
| Issue : 2 | Page : 191-194
Concurrent occurrence of thyroid-associated orbitopathy and idiopathic orbital inflammatory diseases
Sarala Sankar, Bhagwati Wadwekar
Department of Ophthalmology, Pondicherry Institute of Medical Sciences, Kalapet, Puducherry, India
|Date of Submission||07-Oct-2021|
|Date of Decision||05-Feb-2022|
|Date of Acceptance||09-Feb-2022|
|Date of Web Publication||30-Jun-2022|
Department of Ophthalmology, Pondicherry Institute of Medical Sciences, Kalapet, Puducherry - 605 014
Source of Support: None, Conflict of Interest: None
Background: The coexistence of thyroid-associated orbitopathy (TAO) and idiopathic orbital inflammatory diseases (IOID) is rare. They mimic each other clinically, and it is challenging to differentiate the two. We are presenting a case of bilateral proptosis resulting from concomitant TAO and IOID. Case Report: A 44-year-old female presented with swelling, pain, and protrusion of both eyes for one month. She had a past history of trauma in the right eye (RE) 15 years ago. Her best-corrected visual acuity in RE was hand movements and 20/30 in the left eye (LE). There was lid edema, conjunctival congestion, chemosis with axial proptosis, and restricted extraocular movements in both eyes (BE). The anterior segment of RE revealed sequels of old anterior traumatic uveitis and it was normal in the LE. The B scan done in BE showed enlargement of all extraocular (EOM). CT scan orbit showed enlargement of all the EOM belly and tendon insertions. Thyroid profile found to have high anti-TPO (thyroid peroxidase) suggestive of Hashimoto's thyroiditis. The patient was started on intravenous methylprednisolone and showed significant improvement. Conclusion: TAO and IOID can present together. This case report highlights the need for clinicians to be aware of this condition.
Keywords: Hashimoto's thyroiditis, orbital pseudotumor, steroid response, thyroid associated orbitopathy
|How to cite this article:|
Sankar S, Wadwekar B. Concurrent occurrence of thyroid-associated orbitopathy and idiopathic orbital inflammatory diseases. TNOA J Ophthalmic Sci Res 2022;60:191-4
|How to cite this URL:|
Sankar S, Wadwekar B. Concurrent occurrence of thyroid-associated orbitopathy and idiopathic orbital inflammatory diseases. TNOA J Ophthalmic Sci Res [serial online] 2022 [cited 2022 Aug 12];60:191-4. Available from: https://www.tnoajosr.com/text.asp?2022/60/2/191/349509
| Introduction|| |
Grave's orbitopathy is the most common orbital disease with unilateral or bilateral proptosis. It is commonly associated with hyperthyroidism in 90%. It may be seen in hypothyroid autoimmune thyroiditis in 5% cases and euthyroid status in 1 2%.
It must be differentiated from other orbital tissue inflammatory conditions, mimicking Grave's orbitopathy. The most common differential diagnosis is idiopathic orbital inflammation (IOID). Other differential diagnoses include orbital infections, orbital manifestations of systemic diseases, primary and secondary orbital neoplasms, and orbital vascular anomalies.
IOID is a benign inflammatory process of the orbit with a large polymorphous lymphoid infiltrate, associated with fibrosis in variable amounts. The localized inflammation occurs within the extraocular muscles (orbital myositis), lacrimal gland (dacryoadenitis), sclera (scleritis), uvea (uveitis), also the superior orbital fissure, and the cavernous sinus (Tolosa-Hunt syndrome).
The coexistence of thyroid-associated orbitopathy (TAO) and IOID is rare and one may hide the presence of the other. Clinically, TAO and IOID mimic each other, and it is challenging to differentiate the two. We are presenting a case of bilateral axial proptosis resulting from concomitant TAO and orbital pseudotumor.
| Case Report|| |
A 44-year-old female presented to the ophthalmology outpatient department, with complaints of swelling around both eyes for the past one month which was aggravated in the last one week. It was sudden in onset, progressive, and associated with watering, eye pain, and redness. There were no known co-morbidities. She had a history of trauma with stick injury to the right eye 15 years back, following which she lost vision in her right eye. On examination, her best-corrected visual acuity was hand movements in the right eye (RE) and 20/30 in the left eye (LE). She had lid edema, conjunctival congestion, and chemosis with axial proptosis (RE - 23 mm LE - 24 mm using Hertel's exophthalmometer) and restriction of extraocular movements in both eyes (BE) [Figure 1]a. The anterior segment of RE showed adherent leucoma with irregular anterior chamber depth with an eccentric pupil and it was normal in the LE. The RE fundus could not be viewed and LE had normal fundus. B scan ultrasonography for BE showed enlargement of all extraocular muscles [Figure 2]. The CT scan of the orbit showed diffuse enlargement of all the extraocular muscle belly as well as tendon insertions [Figure 3]. MRI scan of the orbit was performed; it also showed a similar picture as that of CT diffuse enlargement of all the extraocular muscle belly as well as tendon insertions (Ethics committee is obtained and date of the approval 20-01-2021).
|Figure 1: (a) Lid edema, conjunctival congestion and axial proptosis. (b) After IVMP showed clinical improvement|
Click here to view
|Figure 3: CT scan of the orbit showed diffuse enlargement of all the extraocular muscle belly as well as tendon insertions|
Click here to view
The routine blood investigations revealed that there was an increase in C-reactive protein (CRP) and Erythrocyte segmentation rate (ESR) which confirmed the active inflammation [Table 1]. Thyroid profile reports were found to have high TSH (Thyroid stimulation hormone) and high Anti TPO (Thyroid peroxidase) [Table 2]. The physician consultation was done and the patient was diagnosed to have Hashimoto's thyroiditis. An ophthalmological diagnosis of TAO (Clinical activity score 7) and IOID, in both eyes, with old traumatic uveitis sequelae RE was made. The patient was admitted and was started on Intravenous methylprednisolone (IVMP) 1mg/kg for 3 days followed by oral steroids.
The patient was reviewed on day 3 after IVMP and showed clinical improvement [Figure 1]b with a decrease in proptosis (RE - 20 mm LE - 21mm using Hertel's exophthalmometer) and was advised for oral steroids in tapering doses. Patient was followed up for one year, and there was no recurrence.
| Discussion|| |
Thyroid-associated orbitopathy and IOID are two common and distinct orbital inflammatory disorders. It is rare to see the presentation of two together. Clinically both simulate each other. We have reported a case of concurrent occurrence of TAO and IOID.
Other causes of orbital inflammation are IOID, Cushing's syndrome, obesity, idiopathic myositis and cellulitis, granulomatous disorders, primary or metastatic tumors, fistulas in the cavernous portion of the carotid artery, and other vascular conditions. Few cases have been reported of the coexistence of TAO and IOID.,
Thyroid-associated orbitopathy is an autoimmune disorder of the orbit and periocular soft tissue associated with dysfunction of the thyroid status. Even though it is highly prevalent in a hyperthyroid state, it can occur in euthyroid and hypothyroid patients (Hashimoto's thyroiditis). Our patient was diagnosed to have Hashimoto's disease. The patient had high TPO and TSH levels.
Usually, Hashimoto's thyroiditis presents as subclinical hypothyroidism as in our case. And rarely, develop hyperthyroidism at the initial presentation, which resolves over a few weeks to months. This phase of hyperthyroid is followed by the euthyroid or hypothyroid state.
The prevalence of TAO varied in different ethnic groups and was found to be higher among Europeans (42%) than Asians (7.7%). Another study found the prevalence of TAO 40% among Indians. The incidence rate documented in previous studies was 16 females and 3 males per 1 lakh in the general population per year. Although it is common in females, the severity increases in male proportion. Tijang et al. studied a group of 20 patients with Hashimoto's thyroiditis and found that 34% of the patients had TAO. Whereas IOID accounts for 40% of cases present with proptosis.
IOID is a benign, non-infectious, inflammatory process of the orbit. It was first described by Birch-Hirshfeld as an orbital syndrome clinically resembling a neoplasm but surgical pathology showing only inflammation. It is characterized by a polymorphous lymphoid infiltrate with varying degrees of fibrosis, without a known local or systemic cause. It is diagnosed by excluding other possible causes of proptosis. IOID occurs more often in the third to sixth decades with no strong sex predilection.
Risk factors of TAO include tobacco use, genetics, TSH receptor antibody level, advanced age, smoking and stress. However, severity and prognosis worsen when it is associated with diabetes mellitus causing a higher incidence of dysthyroid optic neuropathy.
The most accepted mechanism causing TAO is that autoantibodies are formed against thyrotropin receptors present in both the thyroid gland and orbit fibroblasts, which causes activation of T lymphocytes with subsequent release of inflammatory mediators. This infiltrates into the muscles and hyaluronic causing enlargement of the EOMs and an increase in the orbital fat volume. The involvement is frequently bilateral and symmetric. The pathogenesis of IOID is not known. Various immune-mediated processes are considered as a possible ocular mechanism. Various possibilities put forward are trauma (aberrant wound healing) and surgical inflammation, infectious diseases (Epstein-Barr virus) autoimmune disorders, carcinoma (usual fibrosarcoma), IgG4-related disease, and infections. The coexistence of two conditions is considered, as a tendency for concurrent occurrences of autoimmune diseases. The patient was diagnosed with Hashimoto's thyroiditis which is an autoimmune disease.
In both TAO and IOID, they have a similar clinical picture of proptosis, lid edema, conjunctival congestion and chemosis, reduction of ocular motility and diplopia, and in the most severe cases, compression of the optic nerves at the orbital apex, with reduction of visual acuity. In TAO, involvement is symmetrical and bilateral, whereas IOID is usually unilateral but occasionally bilateral. In our case, the patient had proptosis, lid edema, conjunctival congestion, and chemosis, with restriction of extraocular movements in all directions as shown in [Figure 1]a. TAO has a clinical presentation similar to other orbital inflammatory conditions.
Hashimoto's thyroiditis is was confirmed by a deranged thyroid profile. The presence of high serum concentrations of antibodies to thyroid peroxidase confirms the condition in 90% of the cases.
The diagnostic evaluation to differentiate TAO and IOID includes computed tomography (CT)/magnetic resonance imaging (MRI) of the orbit. Observation of muscle shape, size, borders, the pattern of involvement, surrounding soft tissues, and bone involvement can help in the differential diagnosis. In TAO, there will be an enlargement of EOMs and an increase in retrobulbar fat volume is noted. Classical criteria is a spindle-shaped enlargement of EOMs >4 mm without the involvement of the tendon on both MRI and CT. The most common muscle involvement is the inferior rectus, followed by the medial rectus, then the superior rectus, and finally, it affects the lateral rectus. In IOID, single muscle involvement is more common with characteristic diffuse irregular enlargement of muscles with tendons in most cases. Orbital fat may be involved, suggestive of inflammation. In the present case, an MRI scan of the orbit showed diffuse enlargement of all extra-ocular muscles including the tendon, and inflammation of orbital fat. As there was the involvement of both the belly and tendon, we made a probable diagnosis of IOID. As the disease involved all the EOM and fat with raised TPO, we considered TAO as a differential diagnosis. Hence our patient had both the IOID and TAO coexisting together.
The biopsy is indicated, particularly in patients with persistent or recurrent IOID. Our patient responded well to IVMP and did not show recurrence hence biopsy was not done in our case. Moreover, taking a biopsy from muscle may lead to complications such as scar formation. The mainstay of treating both the condition is reducing the inflammatory process. Therefore, Corticosteroids remains the first-line therapy in IOID whereas, in TAO, mild cases are treated with lubricants, and moderate to severe cases started with intravenous steroid therapy. Patients with recurrent or persistent IOID are treated with low dose radiotherapy, chemotherapy, or immunosuppressive therapy. Our case had active TAO with a CAS score of 7 and was started on IVMP. Our patient responded to IVMP with a resolution of clinical signs and symptoms.
| Conclusions|| |
Thyroid-associated orbitopathy and IOID can present together. This case report highlights the need for clinicians to be aware of this condition.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Bartalena L, Baldeschi L, Boboridis K, Eckstein A, Kahaly GJ, Marcocci C, et al.
European Group on Graves' Orbitopathy (EUGOGO). The 2016 European Thyroid association/European Group on Graves' Orbitopathy Guidelines for the Management of Graves' Orbitopathy. Eur Thyroid J. 2016;5:9-26.
Stanciu M, Popa FL, Totoian IG, Bera LG. Orbital pseudotumor can mimic Graves'ophthalmopathy. Acta Endocrinol 2016;12:344-8.
Boddu N, Jumani M, Wadhwa V, Bajaj G, Faas F. Not all orbitopathy is Graves': Discussion of cases and review of literature. Front Endocrinol (Lausanne) 2017;8:184. doi: 10.3389/fendo.2017.00184.
Bijlsma WR, Kalmann R. Idiopathic orbital inflammation and Graves' ophthalmopathy. Arch Ophthalmol 2010;128:131-2.
Tachibana S, Yokoi T, Sato S, Oda Y, Yanase T, Yamashita H. Idiopathic orbital myositis associated with Graves' disease. Intern Med 2013;52:787-90.
Ponto KA, Binder H, Diana T, Matheis N, Otto AF, Pitz S, et al
. Prevalence, phenotype, and psychosocial well-being in Euthyroid/hypothyroid thyroid associated orbitopathy. Thyroid 2015;25:942-8.
Shahbaz A, Aziz K, Umair M, Sachmechi I. Prolonged duration of hashitoxicosis in a patient with Hashimoto's thyroiditis: A case report and review of literature. Cureus 2018;10:e2804. doi: 10.7759/cureus. 2804.
Tellez M, Cooper J, Edmonds C. Graves' ophthalmopathy in relation to cigarette smoking and ethnic origin. Clin Endocrinol (Oxf) 1992;36:291-4.
Lim SL, Lim AK, Mumtaz M, Hussein E, Wan Bebakar WM, Khir AS. Prevalence, risk factors, and clinical features of thyroid-associated ophthalmopathy in multiethnic Malaysian patients with Graves' disease. Thyroid 2008;18:1297-30.
Bartley GB, Fatourechi V, Kadrmas EF, Jacobsen SJ, Ilstrup DM, Garrity JA, et al
. The incidence of Graves' ophthalmopathy in Olmsted County, Minnesota. Am J Ophthalmol 1995;120:511-7.
Tjiang H, Lahooti H, McCorquodale T, Parmar KR, Wall JR. Eye and eyelid abnormalities are common in patients with Hashimoto's thyroiditis. Thyroid 2010;20:287-90.
Espinoza GM. Orbital inflammatory pseudotumors: Etiology, differential, diagnosis, and management. Curr Rheumatol Rep 2010;12:443-4.
Shan SJ, Douglas RS. The pathophysiology of thyroid eye disease. J Neuroophthalmol 2014;34:177-85.
Jin R, Zhao P, Ma X, Ma J, Wu Y, Yang X, et al
. Quantification of Epstein–Barr virus DNA in patients with idiopathic orbital inflammatory pseudotumor. PLoS One 2013;8:e50812. doi: 10.1371/journal.pone. 0050812.
Shen T, Chen J, Lin J, Liu R, Yan J. Concomitant idiopathic orbital inflammatory pseudotumor and thyroid-associated ophthalmopathy. J Craniofac Surg 2015;26:e479-81.
Muller-Forell W, Kahaly GJ. Neuroimaging of Graves' orbitopathy. Best Pract Res Clin Endocrinol Metab 2012;26:259-71.
Ruchala M, Sawicka-Gutaj N. Advances in the pharmacological treatment of Graves' orbitopathy. Expert Rev Clin Pharmacol 2016;9:981-9.
[Figure 1], [Figure 2], [Figure 3]
[Table 1], [Table 2]