Orbital Inflammatory Syndromes Research Articles

An Evidence-Based Approach to Diagnosing Patients Presenting With Orbital Inflammatory Syndromes (An American Ophthalmological Society Thesis)

PurposeThe initial management of patients presenting with orbital inflammatory syndromes (OIS) varies – with some pursuing a diagnostic workup while others treat without any testing. The evidence behind this rationale is limited. A review of patients with orbital inflammation is performed with analysis of diagnostic testing to develop a workflow for such patients. DesignA retrospective review of patients presenting with acute orbital inflammation at a single institution from 2011 – 2020 was conducted. MethodsOrbital imaging was required for inclusion. Patients with high suspicion of neoplasm, bacterial infection, vascular malformation, or thyroid eye disease were excluded. Review of presenting clinical findings, laboratory workup, orbital imaging, and biopsy were performed. Statistical analysis was performed identifying statistically significant diagnostic and treatment maneuvers. ResultsIn total, 172 patients met inclusion criteria (66% female, mean age 46 years). The primary clinical presentation was myositis, dacryoadenitis, and infiltrative in 67, 73, and 32 patients (39.0, 43.0, 18.6%, respectively). Laboratory studies were conducted in 145 (84%) while biopsy was performed in 55 (32%). Specific orbital inflammation (SOI) was diagnosed in 29 (16.9%) with most frequent diagnoses being sarcoidosis, IgG4-related disease, inflammatory bowel disease, and systemic lupus erythematosus. The majority (147, 85.5%) was initially treated with steroids, but steroid-sparing agent use was statistically higher in patients with SOI. ConclusionsIn patients presenting with OIS, distinguishing myositis, dacryoadenitis, and infiltrative disease has workup and differential diagnosis impacts. Because SOI is relatively common, sequelae of SOI, and potential steroid-sparing therapy needs, diagnosis via an evidence-based systemic approach is critical.

Établissement d’un arbre décisionnel diagnostique dans les orbitopathies inflammatoires non basedowiennes chroniques – à propos de 61 patients

Orbital inflammatory syndromes include a wide variety of inflammatory intraorbital processes which are very different in terms of clinical presentation and prognosis. We currently prefer to differentiate so-called "specific" inflammations, for which an etiology is able to be identified, from idiopathic orbital inflammatory syndromes (IOIS), for which the etiology remains unknown and the histology is nonspecific. To propose an efficient diagnostic approach for clinicians managing patients with non-Graves' orbital inflammations. This is a retrospective and prospective study concerning 61 patients managed by the medical team for non-Graves' orbital inflammations between May, 1999 and May, 2013 in the ophthalmology departments of Nice and Limoges university hospitals in France. Seventeen specific inflammations, 19 orbital lymphomas and 25 idiopathic orbital inflammatory syndromes were included. Patients were divided into two groups. Thirty-six patients (group 1) underwent primary biopsy, while for the other 25 (group 2), therapy was begun empirically without biopsy. We could therefore compare both approaches in terms of diagnostic efficiency and time until identification of a specific etiology. Our statistical results show that an approach without primary biopsy leads to a number of specific diagnoses statistically much lower than that obtained by the approach with primary biopsy. Also, the risk of missing a specific inflammation (with as a consequence an inappropriate treatment and a risk of functional sequelae as well as a fatal risk of missing a lymphoproliferative pathology) is very clearly higher in the case of not performing primary biopsy. Finally, the average time elapsed between the initial consultation with the ophthalmologist and a specific diagnosis was one month in the case of the first approach, while this delay was almost three times higher with the second approach, with a mean of 2.91 months (P<0.01). Our study shows that biopsy should be the mainstay of diagnostic management. A trial of empiric treatment is only performed first in myositis or in locations where biopsy could jeopardize functional prognosis. It should only be done after biopsy in all other cases. Of course, in all cases of relapse or recurrence after treatment, biopsy should be performed or repeated. The diagnostic work-up of a patient with an orbital inflammatory process must of course include blood testing and orbital imaging, but also a systematic primary biopsy for histological examination in the vast majority of cases. It must be repeated at least in the case of any doubt about the diagnosis or in the case of any recurrence or resistance to treatment.

Intérêt diagnostique d’une réunion de concertation multidisciplinaire des pathologies orbitaires inflammatoires au CHU de Nantes

Observational study of the diagnoses and the treatment modalities used for orbital inflammatory pathologies discussed in multidisciplinary meetings (MDM) in Nantes University Medical Center over 4 years.A multidisciplinary meeting to discuss eyelid and orbital pathologies (excluding cancer) has been held in Nantes three times per year since October 2008. This retrospective study focuses on the cases of orbital inflammatory pathologies discussed at this meeting from October 2008 to October 2012 (49 cases included).Twenty-eight (57%) patients were diagnosed with orbital inflammation, 16 (33%) cases with isolated myositis and 5 (10%) with dacryoadenitis. In the diagnostic work-up, orbital biopsy was performed in 64% of orbital inflammation cases, 38% of myositis cases and 80% of dacryoadenitis cases. These specimens led to the diagnosis of 9 lymphomas, 2 histiocytoses and 1 metastasis. The internal medicine work-up allowed for the diagnosis of 3 granulomatoses with polyangiitis, one patient with sarcoidosis, and one patient with tuberculosis. Despite work-up, there remained 14 (29%) idiopathic orbital inflammatory syndromes (IOIS).The MDM of orbital pathology allows for the collective approach to the most difficult cases of management and is not concerned with stable orbitopathies or those presenting no diagnostic dilemma. Despite a multidisciplinary, rational etiologic work-up (frequently involving a biopsy), we find a higher rate of IOIS than reported in the literature.A multidisciplinary approach appears to be useful in improving management of orbital disease.

Tolosa-Hunt syndrome—a rare cause of headache and ophthalmoplegia

A 54-year-old African American woman came to our hospital with a 1-week history of severe right-sided retro-orbital pain and blurry vision. 2 days after onset of unilateral throbbing eye pain she developed intermittent blurry vision, which was worse with rightward gaze. She had no facial droop or language impairment, and no previous episodes of headache or visual changes. She had no history of orbital trauma. Her only previous medical diagnoses were schizophrenia and depression. She was taking aripiprazole and escitalopram daily.

Receptor expression in orbital inflammatory syndromes and implications for targeted therapy

To investigate the expression of multiple therapeutic targets in tissue specimens from patients with orbital inflammatory syndromes, the clinical records of 16 patients treated for orbital inflammation between January 2003 and November 2005 for whom tissue blocks were available were reviewed retrospectively. Immunohistochemical staining was performed on archived specimens using commercially available monoclonal antibodies against CD3, CD20, CD22, CD23, CD25, and CD52 antigens. The histologic diagnoses were confirmed, and the immunohistochemical staining patterns were agreed upon by both collaborating pathologists (JLJ and PC-B). The study included 13 women and 3 men who ranged in age from 4 to 79 years (mean, 46 years). The histologic diagnoses were as follows: orbital pseudotumor in six patients; sarcoidosis, three; eosinophilic granuloma, one; necrobiotic xanthogranuloma, one; nonspecified granulomatous inflammation, one; Graves' ophthalmopathy, one; Wegener's granulomatosis, one; and reactive lymphoid hyperplasia, two. One orbital lymphoma specimen and one foreign body reaction specimen were used as controls. CD20 was strongly expressed in all specimens except three (Wegener's granulomatosis, eosinophilic granuloma, and nonspecified granulomatous inflammation specimens), and CD25 was strongly expressed in all specimens except the Wegener's granulomatosis specimen, in which this antigen was only moderately expressed. CD20 and CD25 were strongly or moderately expressed in most of the tested specimens of orbital inflammation. If our findings are confirmed in a larger study, rituximab, which targets CD20, and denileukin diftitox (ONTAK), which targets CD25, should be considered for future clinical trials for orbital inflammatory syndromes.

291 Orbital Myositis Presenting as Cluster Headache

Background. Cluster headache affects men in the third decade of life. Episodes centers in and around eye, start acutely, last 1 to 2 hours, and occur several times a day. Associated features are ipsilateral conjunctival hyperemia, lacrimation, ptosis, miosis, and facial sweating. Orbital myositis belongs to the group of idiopathic orbital inflammatory syndromes. Clinical picture involves proptosis, conjunctival hyperemia, lid edema, and painful eye movements. Case report. We report on a 13 year-old girl presenting with recurrent and alternating orbital myositis mimicking a cluster headache. At the age of 10 years she first experienced an episode of ocular pain, diplopia, and palpebral edema involving the right eye. Lacrimation, conjunctival hyperemia, nasal rhinorrhea and headache were also observed. The episode lasted 1 month. Neurological examination was normal. Further four episodes affecting the right and the left eyes, alternatively, occurred during a period of 18 months. Cluster headache was initially diagnosed. Neither FANS nor tryptans were able to control clinical symptoms. Magnetic resonance imaging of the orbits showed evident edema and thickening of the correspondent rectus lateralis which subsided after corticosteroids. Idiopathic ocular myositis was therefore diagnosed. The following investigations were performed in several occasions and resulted normal or negative. They included: (i) levels of complement factors and of immunoglobulins; (ii) Raitest and Waaler-Rose test; (iii) research of antinuclear antibody, antibody to ds-DNA, antibodies to extractable nuclear antigens (RNP, Sm, SS/A, SS/B), antisynthetasis (Jo1), antineutrophil cytoplasmic antibody (IFI, antiproteinase 3, antimyeloperoxidase, antilactoferrin). Thyroid function was normal. Viral and bacterial infections were also excluded. Orbital myositis may clinically mimic cluster headache. Conclusions. Orbital myositis should be considered in those patients with atypical cluster headache signs such as painful eye movements and ocular pain which do not resolve after 3–4 hours. An early diagnosis is necessary to plan a proper therapeutic strategy.

Myosite orbitaire idiopathique

Introduction. – Orbital myositis is a subtype of nonspecific orbital inflammation confined to one or more of the extraocular muscles. Exegesis. – Three female patients presented presumed orbital myositis, in each of whom the diagnosis was suspected clinically and confirmed by the computed tomography (CT) scan appearances. All presented with orbital pain, diplopia and periorbital edema. All patients responded to systemic corticosteroid with prompt resolution of symptoms and signs , but one patient had recurrent episodes of myositis, leading to the prescription of azathioprine. The clinical and radiological constellation of findings allowing a definitive diagnosis of the nonspecific orbital inflammatory syndrome in most cases is discussed. Conclusion. – The nonspecific orbital inflammatory syndromes are a heterogenous group of conditions and the CT scan is a valuable tool in differentiating these conditions. Most cases of idiopathic orbital myositis require corticosteroids, but therapeutic alternatives to corticosteroids in refractory diseases are warranted.