BACKGROUND: We describe a 75-year-old woman who experienced vision loss in her left eye due to biopsy-proven giant cell arteritis (GCA). She subsequently developed pachymeningitis causing refractory headaches and bilateral optic neuropathy and maculopathy. METHODS: Case report with literature review. RESULTS: Eighteen months after the initial diagnosis of GCA, imaging studies in our patient demonstrated pachymeningeal enhancement, and meningeal biopsy confirmed lymphoplasmacytic tissue infiltrates with low frequencies of IgG4+ plasma cells. Laboratory investigation revealed the presence of 3 antiretinal antibodies and antimyeloperoxidase antibodies, consistent with autoimmune retinopathy. Treatment with B-cell-depleting anti-CD20 antibodies suppressed meningeal inflammation and prevented further vision loss. CONCLUSIONS: This case illustrates that bilateral vision loss and chronic headaches in patients with GCA may result from retina-directed autoimmunity and pachymeningitis.
BACKGROUND: We describe a 75-year-old woman who experienced vision loss in her left eye due to biopsy-proven giant cell arteritis (GCA). She subsequently developed pachymeningitis causing refractory headaches and bilateral optic neuropathy and maculopathy. METHODS: Case report with literature review. RESULTS: Eighteen months after the initial diagnosis of GCA, imaging studies in our patient demonstrated pachymeningeal enhancement, and meningeal biopsy confirmed lymphoplasmacytic tissue infiltrates with low frequencies of IgG4+ plasma cells. Laboratory investigation revealed the presence of 3 antiretinal antibodies and antimyeloperoxidase antibodies, consistent with autoimmune retinopathy. Treatment with B-cell-depleting anti-CD20 antibodies suppressed meningeal inflammation and prevented further vision loss. CONCLUSIONS: This case illustrates that bilateral vision loss and chronic headaches in patients with GCA may result from retina-directed autoimmunity and pachymeningitis.
Authors: Vikram Deshpande; Yoh Zen; John Kc Chan; Eunhee E Yi; Yasuharu Sato; Tadashi Yoshino; Günter Klöppel; J Godfrey Heathcote; Arezou Khosroshahi; Judith A Ferry; Rob C Aalberse; Donald B Bloch; William R Brugge; Adrian C Bateman; Mollie N Carruthers; Suresh T Chari; Wah Cheuk; Lynn D Cornell; Carlos Fernandez-Del Castillo; David G Forcione; Daniel L Hamilos; Terumi Kamisawa; Satomi Kasashima; Shigeyuki Kawa; Mitsuhiro Kawano; Gregory Y Lauwers; Yasufumi Masaki; Yasuni Nakanuma; Kenji Notohara; Kazuichi Okazaki; Ji Kon Ryu; Takako Saeki; Dushyant V Sahani; Thomas C Smyrk; James R Stone; Masayuki Takahira; George J Webster; Motohisa Yamamoto; Giuseppe Zamboni; Hisanori Umehara; John H Stone Journal: Mod Pathol Date: 2012-05-18 Impact factor: 7.842
Authors: Austin R Fox; Lynn K Gordon; John R Heckenlively; Janet L Davis; Debra A Goldstein; Careen Y Lowder; Robert B Nussenblatt; Nicholas J Butler; Monica Dalal; Thiran Jayasundera; Wendy M Smith; Richard W Lee; Grazyna Adamus; Chi-Chao Chan; John J Hooks; Catherine W Morgans; Barbara Detrick; H Nida Sen Journal: Am J Ophthalmol Date: 2016-05-20 Impact factor: 5.258
Authors: Zachary S Wallace; Mollie N Carruthers; Arezou Khosroshahi; Robert Carruthers; Shweta Shinagare; Anat Stemmer-Rachamimov; Vikram Deshpande; John H Stone Journal: Medicine (Baltimore) Date: 2013-07 Impact factor: 1.889