Abstract
Answer C. Orbitotomy for lateral rectus biopsy. Neither the history, clinical features (Figure 1), nor the physical examination or the radiographic findings (Figure 2) are consistent with thyroid-associated ophthalmopathy. An orbitotomy and biopsy of the lateral rectus muscle is indicated for histologic diagnosis. View LargeDownload Figure 1. Proptosis and conjunctival hyperemia with no evidence of upper eyelid retraction or eyelid lag. View LargeDownload Figure 2. Coronal and axial computed tomography. A, Coronal computed tomography shows enlargement of both the lateral rectus and inferior rectus muscles and minimal enlargement of the superior rectus (left > right) and left superior oblique muscles. The lateral rectus enlargement is out of proportion to the other muscles. The infraorbital nerves are markedly enlarged (arrows). B, Axial computed tomography shows massive enlargement of each lateral rectus muscle, which is enlarged out of proportion to the medial rectus muscle enlargement. Comment A biopsy specimen of the right lateral rectus muscle showed strong reactivity to IgG and IgG4 in a sheetlike and nodular pattern within the muscle. The nodules represent reactive germinal centers. The IgG to IgG4 ratio was 1:1 and there were on average 10 IgG4+ cells per high-power field. In some areas, there were as many as 30 IgG4+ cells per high-power field. This patient's recurrent symptoms were felt to be related to IgG4 disease rather than thyroid ophthalmopathy. Computed tomography of the patient's chest and abdomen showed periaortic lymphadenopathy along the descending aorta. No lymphadenopathy, masses, or organomegaly were demonstrated elsewhere. The remainder of his laboratory and physical examination was normal. IgG4-related sclerosing disease, especially as it relates to the orbits, is a recently described entity. It is a systemic disease involving numerous organs and systems. Among the manifestations of this disease are autoimmune pancreatitis, retroperitoneal fibrosis, Riedel thyroiditis, panniculitis, sclerosing cholangitis, and chronic sclerosing sialoadenitis.1-3 In 2003, Kamisawa and colleagues4 reviewed a series of patients with autoimmune pancreatitis. Numerous biopsy specimens of extrapancreatic sites were obtained from both involved and uninvolved organ systems. A lymphoplasmacytic infiltration with fibrosis and an abundance of IgG4 plasma cells were observed both in the involved and uninvolved tissues. In 2010, Matsuo et al5 studied a series of patients with benign lymphoid lesions of the ocular adnexa. They demonstrated that all patients found to be positive for IgG4 in these tissues also showed other systemic manifestations of the disease, while none of the patients who were IgG4 negative had any evidence of systemic involvement. Initial treatment is with corticosteroids but either corticosteroid adverse effects or recurrences are common. Rituximab has been documented as an effective treatment for this disease.6 Treatment is primarily aimed to reduce or prevent fibrosis and improve symptoms associated with this condition. Lymphoma has been reported with IgG4-related disease7 but this is uncommon in our experience.8 This patient was treated with rituximab and had an excellent response with a marked reduction in proptosis and resolution of his diplopia in primary gaze. He continues to have minimal diplopia in extremes of gaze. He had no recurrence of his symptoms after 10 months of follow-up. Return to Questions Back to top Article Information Correspondence: Francisco Castillo, MD, Ophthalmology Department at Mayo Clinic Rochester, 200 First St SW, Rochester, MN 55905 (castillo.francisco@mayo.edu). Submitted for Publication: November 14, 2012; accepted December 2, 2012. Conflict of Interest Disclosures: None reported. References 1. Batra R, Mudhar HS, Sandramouli S. A unique case of IgG4 sclerosing dacryocystitis. Ophthal Plast Reconstr Surg. 2012;28(3):e70-e7221946772PubMedGoogle ScholarCrossref 2. Hamano H, Arakura N, Muraki T, Ozaki Y, Kiyosawa K, Kawa S. Prevalence and distribution of extrapancreatic lesions complicating autoimmune pancreatitis. J Gastroenterol. 2006;41(12):1197-120517287899PubMedGoogle ScholarCrossref 3. Kamisawa T, Okamoto A. Autoimmune pancreatitis: proposal of IgG4-related sclerosing disease. J Gastroenterol. 2006;41(7):613-62516932997PubMedGoogle ScholarCrossref 4. Kamisawa T, Funata N, Hayashi Y, et al. A new clinicopathological entity of IgG4-related autoimmune disease. J Gastroenterol. 2003;38(10):982-98414614606PubMedGoogle ScholarCrossref 5. Matsuo T, Ichimura K, Sato Y, et al. Immunoglobulin G4 (IgG4)-positive or -negative ocular adnexal benign lymphoid lesions in relation to systemic involvement. J Clin Exp Hematop. 2010;50(2):129-14221123971PubMedGoogle ScholarCrossref 6. Khosroshahi A, Carruthers MN, Deshpande V, Unizony S, Bloch DB, Stone JH. Rituximab for the treatment of IgG4-related disease: lessons from 10 consecutive patients. Medicine (Baltimore). 2012;91(1):57-6622210556PubMedGoogle ScholarCrossref 7. Cheuk W, Yuen HKL, Chan ACL, et al. Ocular adnexal lymphoma associated with IgG4+ chronic sclerosing dacryoadenitis: a previously undescribed complication of IgG4-related sclerosing disease. Am J Surg Pathol. 2008;32(8):1159-116718580683PubMedGoogle ScholarCrossref 8. Plaza JA, Garrity JA, Dogan A, Ananthamurthy A, Witzig TE, Salomão DR. Orbital inflammation with IgG4-positive plasma cells: manifestation of IgG4 systemic disease. Arch Ophthalmol. 2011;129(4):421-42821482868PubMedGoogle ScholarCrossref
Journal
JAMA Ophthalmology
– American Medical Association
Published: Feb 1, 2013
Keywords: graves' ophthalmopathy
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