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Unilateral Frosted Branch Angiitis in a Patient With Immunosuppression

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 49-year-old African American man who was hospitalized with hypervolemia due to supraventricular tachycardia presented with acute unilateral painless vision loss. His active medical problems included atrial flutter on anticoagulation, antisynthetase syndrome (which was diagnosed based on the presence of autoantibodies against aminoacyl-tRNA synthetase), fever with negative infectious workup results, and interstitial lung disease that was managed with azathioprine. Two weeks before the onset of sudden vision loss, the patient developed a rapidly evolving purpuric rash on his hands and feet that progressed to digital ischemia (Figure 1A). He was treated with intravenous methylprednisolone, which was completed 3 days before the onset of loss of vision and transitioned to treatment with azathioprine, 150 mg, and prednisone, 80 mg, that he was taking at the time of his vision loss.

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Frosted branch angiitis due to retinal vasculitis that was associated with antisynthetase syndrome

B. Diagnostic anterior chamber paracentesis and intravitreal foscarnet

Despite the patient’s clinical presentation being suggestive of autoimmune retinal vasculitis, the most appropriate initial step is to rule out viral retinitis with an anterior chamber paracentesis and empirical treatment with intravitreal foscarnet (choice B) given the patient’s immunocompromised status. Escalating immunosuppression (choice A) is inappropriate before excluding infectious etiologies. Peripheral blood smear and flow cytometry (choice C) for clonality assessment or positron emission tomography–computed tomography (choice D) for occult malignancy screening are inappropriate in the short-term setting.

Frosted branch angiitis is a form of retinal vasculitis that is characterized by diffuse vascular sheathing and various degrees of intraocular inflammation.1 Idiopathic frosted branch angiitis occurs in otherwise healthy patients in their first or third decade of life following a viral illness and has a favorable prognosis with systemic steroid therapy.13 Secondary frosted branch angiitis occurs in patients with an underlying infection, such as cytomegalovirus, herpes simplex virus, varicella-zoster virus, toxoplasmosis, tuberculosis, and syphilis, or inflammatory disease, such as Crohn disease, granulomatosis with polyangiitis, sarcoidosis, systemic lupus erythematosus, antiphospholipid syndrome, Behçet disease, multiple sclerosis, familial Mediterranean fever, or drug-induced antineutrophil cytoplasmic antibody vasculitis,1,35 and is thought to result from immune complex deposition. The frosted branchlike appearance may be associated with leukemia or lymphoma due to vessel wall infiltration with malignant cells. This patient had a normal whole-blood flow cytometry. Frosted branch angiitis may also present as a paraneoplastic manifestation of Hodgkin lymphoma6; however, this patient had an unremarkable whole-body positron emission tomography–computed tomography.

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Article Information

Corresponding Author: Narine Viruni, MD, The Wilmer Eye Institute, Retina Division, 1800 Orleans St, Woods 261, Baltimore, MD 21287 (nabgary1@jhmi.edu).

Published Online: February 18, 2021. doi:10.1001/jamaophthalmol.2020.4684

Conflict of Interest Disclosures: Dr Shifera reported grants from the Wilmer Eye Institute outside the submitted work. No other disclosures were reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
1.
Walker  S , Iguchi  A , Jones  NP .  Frosted branch angiitis: a review.   Eye (Lond). 2004;18(5):527-533. doi:10.1038/sj.eye.6700712 PubMedGoogle ScholarCrossref
2.
Ito  Y , Nakano  M , Kyu  N , Takeuchi  M .  Frosted-branch angiitis in a child.   Jpn J Ophthalmology. 1976;30:797-803.Google Scholar
3.
Kleiner  RC .  Frosted branch angiitis: clinical syndrome or clinical sign?   Retina. 1997;17(5):370-371. doi:10.1097/00006982-199709000-00002 PubMedGoogle ScholarCrossref
4.
Aguilar Lozano  LA , Gonzalez Dibildox  A .  Frosted branch angiitis associated with cytomegalovirus retinitis.   Ophthalmology. 2016;123(3):645. doi:10.1016/j.ophtha.2016.01.015 PubMedGoogle ScholarCrossref
5.
Koike  KJ , Blice  JP , Kylstra  JA ,  et al.  Frosted branch angiitis in methimazole-induced antineutrophil cytoplasmic antibody-positive vasculitis.   Retin Cases Brief Rep. 2018;12(2):136-139. doi:10.1097/ICB.0000000000000447 PubMedGoogle ScholarCrossref
6.
Alhaj Moustafa  M , Crowell  EL , Elmahdy  S , Malkovska  V , Reddy  AK .  Paraneoplastic frosted branch angiitis as first sign of relapsed Hodgkin lymphoma.   Clin Case Rep. 2018;6(10):1978-1981. doi:10.1002/ccr3.1778 PubMedGoogle ScholarCrossref
7.
Witt  LJ , Curran  JJ , Strek  ME .  The diagnosis and treatment of antisynthetase syndrome.   Clin Pulm Med. 2016;23(5):218-226. doi:10.1097/CPM.0000000000000171 PubMedGoogle ScholarCrossref
8.
Donovan  CP , Pecen  PE , Baynes  K , Ehlers  JP , Srivastava  SK .  Retinal vasculitis in anti-synthetase syndrome.   Ophthalmic Surg Lasers Imaging Retina. 2016;47(9):874-879. doi:10.3928/23258160-20160901-13 PubMedGoogle ScholarCrossref
9.
Tarabishy  AB , Khan  M , Bunyard  M , Lowder  CY .  Retinal vasculitis associated with the anti-synthetase syndrome.   Ocul Immunol Inflamm. 2010;18(1):16-18. doi:10.3109/09273940903312005 PubMedGoogle ScholarCrossref
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