Diagnostic Algorithm for Patients With Suspected Giant Cell Arteritis' Useful, but No Substitute for Thorough Histopathology: Response

Letters to the Editor "Diagnostic Algorithm for Patients With Suspected Giant Cell Arteritis" Useful, but No Substitute for Thorough Histopathology W e were delighted as a group of ophthalmologists and ophthalmology trainees to read the article in The Journal of Neuro-Ophthalmology by El-Dairi et al (1), documenting the clinical features associated with 213 temporal artery biopsies (TAB). This led them to proposing a "clinical algorithm," which they then state is "highly predictive" for a positive TAB and can be valuable in the evaluation process for suspected cases of giant cell arteritis (temporal arteritis or TA). At the recent Neuro-ophthalmology Society of Australia (NOSA) Annual Scientific Meeting, held in Auckland, New Zealand, from 16th to 21st September 2015, our group presented a template entitled "The temporal arteritis proforma: The 39 steps to clinical diagnosis," as part of a poster at the meeting. This documented 23 symptoms and 16 signs of TA, which were felt to be a predictive measure in diagnosis. This was designed to be used not only in general emergency departments but also in all clinics. This should therefore apply to ophthalmology outpatient departments, and outpatient departments of neurology, rheumatology, dermatology, endocrinology, geriatrics, gastroenterology, cardiology, psychiatry, ear nose and throat, vascular surgery, dental surgery, and others. At the NOSA meeting, laminated A4 templates were distributed to attendees, in hopes that they might find a place in such departments (see Supplemental Digital Content, Fig. E1, http://links.lww.com/WNO/A202). The authors also hope for continuing cooperation between surgical and medical specialties in the treatment of TA. However, our group believes that, although clinical algorithms may be a useful additional tool, the ultimate arbiter in assessing suspected TA must remain high-quality histopathology of the artery (2). This means that there must be at least 26-28 mm of superficial temporal artery, "Diagnostic Algorithm for Patients With Suspected Giant Cell Arteritis" Useful, but No Substitute for Thorough Histopathology: Response W e want to thank Dr. George et al for their interest in our study titled "Diagnostic Algorithm for Patients With Suspected Giant Cell Arteritis" (1). Unfortunately, none of us were able to attend the September Letters to the Editor: J Neuro-Ophthalmol 2016; 36: 343-352 and the histopathologist must be thorough. If the patient has the disease, and the diagnosis is made within 12-24 hours of the biopsy, the patient's vision and life are likely saved (3). If the histopathology is negative, the IV methylprednisolone can be ceased forthwith and the patient monitored carefully. Adarsh George, BMed Nicole S. Lim, BMed Neeranjali S. Jain, BMed (Hons) Calum W. K. Chong, BMed Farshad Abedi, MD, DMedSc Ying Liu, MBBS Sarah B. Wang, BA (Hons) Matt Govendir, BSc (Hons) Ashish Agar, FRANZCO, PhD Ian C. Francis, FRACS, PhD Prince of Wales Hospital and the University of New South Wales, Sydney, Australia The authors report no conflicts of interest. Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the full text and PDF versions of this article on the journal's Web site (www. jneuro-ophthalmology.com). REFERENCES 1. El-Dairi MA, Chang L, Proia AD, Cummings TJ, Stinnett SS, Bhatti MT. Diagnostic algorithm for patients with suspected giant cell arteritis. J Neuroophthalmol. 2015;35:246-253. 2. Dubey R, Chui J, Langford-Smith J, Danesh-Meyer H, Francis IC. Jaw dropping: the necessity of a history and a biopsy in suspected temporal arteritis. Neuroophthalmology. 2011;35:156-157. 3. Sim BWC, Karaconji T, Bhardwaj G, Dubey R, Harris JP, Francis IC. Scalp necrosis in temporal arteritis: abrupt termination of the superficial temporal artery as a possible precursor. J Dtsch Dermatol Ges. 2013;11:551-552. 2015 Neuro-Ophthalmology Society of Australia meeting to comment specifically on their data and conclusions; but we have no reservations in stating that a temporal artery biopsy (TAB) is the gold standard for diagnosing giant cell arteritis (GCA). To be clear, the goal of our study was to improve the diagnostic yield of a positive TAB with a relatively simple diagnostic algorithm to serve as a guide to help clinicians decide which patient needs a TAB. In no way did we intend or state in our article that the algorithm was a substitute for a TAB. In contrast, it would not be feasible or reasonable to perform a TAB 343 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Letters to the Editor on every patient with any one of the 39 signs and symptoms as listed by Dr. George et al. As part of their proforma, they mention "a high clinical suspicion" of GCA, but it is not stated what constitutes a "high clinical suspicion." Our algorithm allows for a simple stratification (very low, moderate, and high clinical suspicion) based on 7 signs and symptoms (in addition to accounting for comorbid conditions that can mimic GCA) to improve on the possibility of obtaining a positive TAB. Ultimately, however, to confirm or refute the diagnosis of GCA requires a TAB. Mays A. El-Dairi, MD Department of Ophthalmology Duke Eye Center and Duke University Medical Center Durham, North Carolina Thomas J. Cummings, MD Alan D. Proia, MD, PhD Departments of Ophthalmology and Pathology Duke Eye Center and Duke University Medical Center Durham, North Carolina The authors report no conflicts of interest. M. Tariq Bhatti, MD Departments of Ophthalmology and Neurology Duke Eye Center and Duke University Medical Center Durham, North Carolina Maternally Inherited Diabetes and Deafness is Phenotypically and Genotypically Heterogeneous REFERENCE 1. El-Dairi MA, Chang L, Proia AD, Cummings TJ, Stinnett SS, Bhatti MT. Diagnostic algorithm for patients with suspected giant cell arteritis. J Neuroophthalmol. 2015;35:246-253. TABLE 1. Phenotypic manifestations of MIDD Organs Affected W ith interest we read the article by Cardenas-Robledo et al (1) regarding 2 patients with maternally inherited diabetes and deafness (MIDD) who presented with multisystem involvement, including vestibular impairment. We have the following comments and concerns. As the authors point out in their introduction, numerous organ systems are affected in patients with MIDD. These are summarized in Table 1. Regarding the reported patients by Cardenas-Robledo et al, did the authors search for subclinical involvement of the gastrointestinal tract and kidneys? With regard to Patient 2, one would expect a sensory problem with regard to the bilateral thalamic lesions. Did the patient report any sensory problems or were any detected on recording of somatosensoryevoked potentials? Did Patient 2 undergo magnetic resonance spectroscopy to search for the nature of the thalamic lesions? Interestingly, Patient 2 developed rhabdomyolysis on administration of statins suggesting that statin myopathy develops particularly in patients with a mitochondrial disorder with myopathy. Which statin over what timeframe was given? What was the dosage? Were additional triggers, such as infection, ruled out as a cause of rhabdomyolysis? Both patients were reported to have cerebral atrophy. What were the clinical manifestations of cerebral atrophy? Did patients undergo neuropsychological testing to see if there was cognitive impairment or not? There are also patients with MIDD who progressed over time and developed phenotypic features of mitochondrial encephalopathy lactacidosis and stroke-like episodes (MELAS) (21). Did either patient develop 344 Central nervous system Supratentorial atrophy Cerebellar atrophy Cerebellar ataxia Basal ganglia calcification Leukoencephalopathy Nystagmus Elevated CSF protein Elevated CSF lactate Neuropsychological deficits Depression Peripheral nervous system Myopathy Rhabdomyolysis Ptosis Polyneuropathy Ears Hypoacusis, anacusis Vestibular system Impaired vestibularocular reflex Eyes Pigmentary retinopathy Macular degeneration Optic atrophy Central retinal vein occlusion Retinal/choroidal atrophy CardenasRobledo et al Literature Reference 1 1 2 1 1 1 1 1 (2) (3) (4) (5) 1 1 1 1 2 2 2 2 1 1 (1) (1) (1) (3) (3) 2 1 (2) 1 1 2 1 1 2 1 2 (6) (1) (7) (1) 1 1 (8) 1 2 (1) 1 1 1 1 1 1 2 1 (9) (10) (1) (11) 1 1 (12) Letters to the Editor: J Neuro-Ophthalmol 2016; 36: 343-352 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.