Vision Loss and Paresthesias in a Young Man

Acute Posterior Multifocal Placoid Pigment Epitheliopathy Complicated by Fatal Cerebral Vasculitis: Response

“…On the other hand, it is of interest that Dr. Knox unearthed retroactively from de Vries et al a positive history of smoking in the case they had reported in 2006 (previously referenced). As Dr. Knox also noted, the case report by Van Zyl et al (3) also mentioned a history of cigarette smoking in their patient. We fully agree with Dr. Knox that any patient with APMPPE should be questioned regarding smoking history, and if answering in the affirmative, should of course be counseled as to the importance of cessation.…”

“…In this case, Dr. Knox raises a provocative point that smoking cessation may have been an under used therapy for cases of APMPPE-associated cerebral vasculitis to date, considering that this is the cornerstone therapeutic strategy for preventing progression of Buerger disease and avoiding amputation in that disorder. That aside, however, neurologic complications of APMPPE are generally treated with intravenous/oral corticosteroids, not uncommonly supplemented by an immunosuppressant agent (1)(2)(3). By contrast, other therapies for Buerger disease (although generally paling in comparison to the proven benefit of smoking cessation) typically focus on vascular perfusion, for example, iloprost (intravenously) or other prostanoids, bosentan (endothelin receptor antagonist), anticoagulants/antiplatelet agents/thrombolytics, antihypertensive agents, in some cases vascular bypass surgery (usually not possible due to the distal nature of the disease), sympathectomy, spinal cord stimulator, intramuscular vascular endothelial growth factor gene therapy, etc.…”

“…Second, regarding infectious triggers, APMPPE is commonly associated with an antecedent viral illness (adenovirus), and associations with rubeola (measles) and mycobacterial infection have also been reported, whereas the infectious triggers reported to be possibly associated with Buerger disease are gram-negative bacteria ( Rickettsia and Porphyromonas gingivalis ) (9). Third, the clinical ocular manifestations of APMPPE have been reported in association with various other autoimmune diseases (e.g., Wegener granulomatosis, systemic lupus erythematosus, erythema nodosum (3), sarcoidosis, psoriatic arthritis, etc. ), whereas there has been no analogous association reported between Buerger disease and other autoimmune diseases.…”