Orbital Myositis: A Comprehensive Review and Reclassification.

A 38-year-old female presented to a tertiary centre with a nine-year history of chronic recurrent isolated lateral rectus orbital myositis (OM). Initially, her condition responded to short courses of oral corticosteroids and orbital steroid injections, but it later became refractory, with an inability to taper off steroids. Concurrently, the patient developed a low-grade clinical activity score for bilateral thyroid eye disease (TED) due to Hashimoto’s thyroiditis (HT). Although the active phase of TED resolved without significant sequelae, she continued to experience unilateral pain and extraocular muscle (EOM) motility restriction consistent with lateral rectus myositis. This report describes a rare case of chronic recurrent idiopathic OM overlapping with a concomitant bilateral TED associated with HT, which was successfully managed with rituximab immunosuppression.

BACKGROUND Orbital myositis (OM) is a rare extraintestinal manifestation (EIM) of inflammatory bowel disease (IBD) in which inflamed extraocular muscles cause severe eye pain. With fewer than thirty cases of OM described in the literature, its etiology remains largely unknown. We thus describe eleven cases of OM in patients diagnosed with IBD or microscopic colitis as well as prevalent comorbidities observed within this group. METHODS Patients with known orbital myositis diagnosed by an ophthalmologist and documented IBD or microscopic colitis were included at this single center institution from 2012 to 2022. RESULTS Of the eleven patients included in this study, six patients (55%) had Crohn’s disease, three patients (27%) had ulcerative colitis, one (9%) had indeterminate colitis, and one (9%) had lymphocytic colitis. All eleven patients were Caucasian and seven (64%) were female. The median age at OM presentation was 54 [47.5-60] years. On presentation, nine patients (82%) reported eye pain, seven (64%) reported blurry vision, four (36%) had headaches with concurrent photophobia, three (27%) had eye swelling, and three (27%) had diplopia. Six patients (55%) had unilateral OM and five (45%) had bilateral disease. Six OM cases were confirmed with ultrasound, two with MRI and three with clinical diagnoses. The median age at IBD diagnosis was 43 [37-51.5] years. At the time of OM presentation, all patients were in clinical remission for their IBD (6/11 patients). Only five patients (45%) were on IBD medication at the time of diagnosis; three were on a biologic medication (infliximab, adalimumab, and ustekinumab), with the others on sulfasalazine and budesonide. Regarding EIMs, two patients (18%) had aphthous ulcers, and nine patients (82%) had a history of IBD-related arthropathy. There were no recorded instances of uveitis, or dermatologic EIMs. Eight patients (72%) had a history of cervical (n = 7) or lumbar (n = 5) spinal disease requiring at least one neurosurgical intervention. Three patients (27%) had a positive ANA titer. Five patients (45%) were given steroids on presentation. Two patients (18%) were initially treated with NSAIDs. One patient (9%) was treated with bromfenac ophthalmic solution. Only three patients (27%) had documented OM flares after treatment; one was initially treated with NSAIDs and two with steroids. CONCLUSIONS Given its rarity, the true prevalence of OM is unknown. Our findings reaffirm prior studies’ observations about OM, including typical presentation and association with IBD. The number of patients in clinical remission for their IBD at the time of OM presentation may suggest a weaker correlation with underlying disease activity, in contrast to other EIMs. Additionally, the prevalence of cervical spinal disease among these patients, given their relatively younger age, warrants further investigation.

Purpose: To report a case of choroidal folds caused by enlargement of the extraocular muscles (EOM) and orbital fat in a patient with immunoglobulin (Ig) G4-related ophthalmic disease. A full recovery was achieved after steroid administration.Case summary: A 56-year-old male received intravenous high-dose steroids to treat IgG4-related ophthalmic disease presenting with bilateral eyelid edema, hypertrophy of all EOM, and mild exophthalmos. After 3 months, only slight improvement was observed and azathioprine was initiated. The patient exhibited only a minimal response over the next 2 months; all medications were therefore stopped. One month later, the patient presented with aggravation of the swelling (both eyelids), exophthalmos, and visual impairment of the left eye. The corrected vision was 1.0 in the right eye and 0.2 in the left eye; choroid folds were observed in the posterior pole of the left eye. Intravenous steroid (500 mg) was given once and oral steroids prescribed as maintenance therapy. After 2 weeks, the corrected visual acuity recovered to 1.0 in both eyes, the eyelid edema and exophthalmos improved and the choroidal folds resolved completely.Conclusions: Choroidal folds associated with enlargement of EOM and orbital fat tissue can cause visual impairment in patients with IgG4-related ophthalmic disease. Steroids are effective in the acute phases and prevent irreversible visual impairment.

Idiopathic orbital myositis is considered as a subgroup of idiopathic orbital inflammatory disease. It is a non-infectious inflammatory disorder primarily affecting the extraocular muscles and causes various eye symptoms including pain, diplopia and limitation of extraocular movement. Cases of isolated ptosis by idiopathic orbital myositis have been very rarely described in the literature. We report a patient who developed unilateral painless ptosis caused by idiopathic orbital myositis. A 52-year-old man presented with drooping of the right eyelid for 3 days. There was no history of headache, double vision or any other complaints. Neurological examination revealed right ptosis without pupil and extraocular muscles involvement. Repetitive nerve stimulation test was normal. Ptosis did not improve after the neostigmine injection. Magnetic resonance imaging scan showed asymmetric enlargement of right superior rectus/levator palpebrae superioris muscle complex and medial rectus muscle. Ptosis resolved dramatically after oral corticosteroid therapy. Isolated unilateral ptosis can be caused by various etiologies. Idiopathic orbital myositis should be considered in the differential diagnosis of ptosis. Keywords: Orbital myositis, Blepharoptosis, Oculomotor muscles

Background:Ophthalmic conditions are common manifestations in patients with rheumatic diseases (RDs), orbital myositis (OM) remain rare. Only 1 case each for scleroderma and undifferentiated connective tissue disease (UCTD) reported.Objectives:Report frequency...

Recurrent orbital myositis as an extra-intestinal manifestation of Crohn's disease

Immunoglobulin G4 (IgG4)-related ophthalmic disease (IgG4-ROD) typically affects the lacrimal glands, trigeminal nerves, and extraocular muscles. Although imaging and serology aid in diagnosis, histopathological confirmation is crucial. Recent updates to the diagnostic criteria have incorporated labial salivary gland biopsy as a biopsy site. We report a case of IgG4-ROD with optic neuropathy diagnosed via labial salivary gland biopsy. A 69-year-old male patient presented with progressive right-eye visual loss. Imaging revealed an orbital mass, enlarged extraocular muscle, and enlarged infraorbital nerve. Serum IgG4 level was elevated, and a labial salivary gland biopsy was performed due to the absence of accessible biopsy sites, confirming the diagnosis. Oral corticosteroid therapy resulted in rapid visual and imaging improvement with no recurrence. This case emphasizes the diagnostic value of infraorbital nerve enlargement and the utility of labial salivary gland biopsy in IgG4-ROD, highlighting the importance of early recognition and treatment to prevent irreversible visual damage.

Adenocarcinoma metastasis causing discrete extraocular muscle enlargement

Background This study was conducted to assess the clinicopathological characteristics of biopsy lesions in the lacrimal gland, in response to evolving pathological technologies and the emergence of new diseases. A retrospective review of case notes was conducted for 582 patients with biopsied lacrimal gland lesions at West China Hospital of Sichuan University in China. This study, spanning from January 1, 2010, to December 31, 2021, recorded demographics, clinical features, imaging findings, histopathological diagnoses, and recurrence rates. In a cohort of 582 patients, the mean age was 47.5 years, with 318 being female. Bilateral disease was present in 127 patients. Common signs and symptoms included a palpable mass, lid swelling, proptosis, and decreased ocular movements or diplopia. The predominant pathologies identified were inflammation, epithelial tumors, and lymphoproliferative lesions. Patients with inflammation (mean 32.8 months) experienced symptoms for a longer period compared to those with lymphoproliferative lesions (mean 22.2 months) (95% confidence interval [CI] 1.53–19.27, P = 0.022). Younger patients tended to have structural lesions (mean 42.5 years) (95%CI 4.74–21.59, P = 0.003), inflammation (mean 42.9 years) (95%CI 0.46–6.57, P<0.001), or epithelial tumors (mean 46.4 years) (95%CI 6.21–12.38, P<0.001), while older patients more frequently had lymphoproliferative lesions (mean 55.7 years). The period from 2016 to 2021 saw a higher diagnosis rate of biopsied lacrimal gland lesions than from 2010 to 2015. During this later period, there was an increase in cases of immunoglobulin G4-related ophthalmic disease (IgG4-ROD) and malignant epithelial tumors, while lymphoid hyperplasia cases declined. Adenoid cystic carcinoma showed the highest recurrence rate, with lesion extent and Ki-67 index identified as independent risk factors in our study. Inflammation, epithelial tumors, and lymphoproliferative lesions were the three primary causes of lacrimal gland lesions in our study group. Among the inflammatory conditions, IgG4-ROD was the most specific. The incidence of lacrimal gland lesions has risen, particularly for IgG4-ROD. Adenoid cystic carcinoma among the lacrimal gland lesions was prone to recurrence, with the extent of the lesion and Ki-67 index serving as independent risk factors.

BackgroundOrbital myositis (OM) is an inflammatory condition that mainly involves extraocular muscles. The exact etiology of this disease is not known [1]. However, rheumatoid arthritis (RA) has been reported as...

Purpose: To report a case with peripheral eosinophilia and elevated immunoglobulin (Ig) E levels, subsequently diagnosed as IgG4-related ophthalmic disease involving the extraocular muscles.Case summary: A 56-year-old male visited the allergy department presenting with systemic urticaria and bilateral eyelid swelling that began 5 months prior. Laboratory examinations showed elevated levels of serum eosinophil and IgE, 1,309 IU/uL and 1,793 IU/mL, respectively. Orbital computed tomography revealed that all extraocular muscles and the bilateral exophthalmos were enlarged, and the patient was referred to the ophthalmology department. Eye alignment was orthophoric for all gaze directions, and limited abduction (-1) was noted in both eyes. An incisional biopsy of the extraocular muscles was conducted. Histopathological findings showed lymphoid aggregates, diffuse fibrosis, and an increased IgG4+/IgG+ plasma cell ratio of 40%, which led to the diagnosis of IgG4-related ophthalmic disease. An elevated IgG4 serum level (1,710 mg/dL) was also noted. The patient received high-dose intravenous steroids and eyelid swelling improved after two months. Levels of serum eosinophil, IgE, and IgG4 all decreased after three months.Conclusions: IgG4-related ophthalmic disease may be accompanied by eosinophilia and elevated IgE. These findings may facilitate future diagnoses of this disease.

ABSTRACTNeoplastic infiltration of the extraocular muscle (EOM) is a rare condition which can pose a diagnostic dilemma due to its rarity and overlapping ultrasonographic features with orbital myositis. The ultrasonographic features of neoplastic enlargement of EOM have not been systematically studied and previously have been described in only a few case reports. Orbital ultrasonography, in conjunction with the pattern of ocular misalignment, was assessed for its potential role in identifying patients with neoplastic EOM enlargement. Retrospective chart review of patients with neoplasm and myositis. The clinical features of 8 patients with neoplastic infiltration of the EOM were compared to 15 patients with myositis. In the neoplastic group the width of the EOM was (10.5 mm) almost twice the normal width of the muscle with myositis (p < 0.001). All the muscles in the neoplastic category were low to medium reflective. Paretic deviation was seen in 4/8(50%), purely restrictive in 2/8 (25%) and combined pattern in 2/8 (25%) were noted. In the myositis group the average EOM enlargement was 5.8 mm and all muscles showed low reflectivity. Although ultrasonographic features overlapped between the 2 groups paretic deviations were more common in the neoplastic group versus the myositis group (50% versus 7%). Neoplastic muscle enlargement tends to be larger with paretic deviations of ocular motility seen clinically. These findings in a patient with EOM enlargement should raise the suspicion of neoplasm as the etiology and further work up should be considered.

Ophthalmic manifestations of IgG4-related disease: Single-center experience and literature review

Anti-tumor necrosis factor alpha (TNF-α) agents are used to treat a variety of autoimmune and inflammatory conditions, including psoriasis. Paradoxically, numerous reports have documented new-onset or exacerbation of psoriasis or psoriasiform skin lesions (PSO) in patients treated with these agents for conditions other than PSO-particularly in adults with inflammatory bowel disease (IBD). Not much is known regarding similar cases in children. A retrospective chart review was performed on children younger than 19 years of age with IBD seen at the Mayo Clinic between 2003 and 2015 who developed new-onset or recurrent PSO while undergoing anti-TNF-α therapy. Fourteen children developed PSO while undergoing anti-TNF-α therapy for IBD. All three anti-TNF-α agents (infliximab, adalimumab, certolizumab) used to treat IBD in this series led to induction or recurrence of PSO lesions. The median time to development of PSO was 11months (range 0-48 mos), the median age was 15years (range 12.5-17.5yrs), and 57% of patients were male. IBD activity was quiescent in 93% of cases at PSO onset. Seven patients (50%) discontinued their initial anti-TNF-α therapy because of their skin disease. Ultimately, four patients (29%) had to discontinue all anti-TNF-α therapy to induce PSO resolution. TNF-α antagonist-induced PSO in children with IBD is a rarely reported adverse reaction. PSO onset has a variable latency, but usually occurs during IBD remission, with a slight male bias. Nearly half of patients required a change in their initial anti-TNF-α agent despite conventional skin-directed therapies, and one-third of patients discontinued all anti-TNF-α therapy because of PSO.

Despite their known benefits, tumor necrosis factor alpha inhibitors (TNFi) may cause certain unexpected side effects, such as the aggravation of pre-existing autoimmune conditions or inducing the onset of new inflammatory conditions, these reactions being called “paradoxical adverse effects’’ (PAEs). The spectrum of TNFi-induced PAEs is vast and may include dermatological disease (frequently - psoriasiform skin reactions), gastroenterological disease (inflammatory bowel disease), ophthalmological disease (uveitis) and other autoimmune conditions (lupus-like reactions, vasculitis). PAEs are characterized by complex physio-pathological mechanisms which remain a matter of further research and may significantly impact the patients’ evolution and quality of life. Importantly, a large number of patients require the cessation of TNFi treatment, as well as other types of therapeutic interventions. The present review aimed to analyze recent findings regarding certain paradoxical adverse effects (psoriasis, inflammatory bowel disease, and uveitis) in patients with RA, ankylosing spondylitis (AS), and PsA treated with TNFi.