Atypical presentation of giant cell arteritis associated with SIADH: a rare association

We present here an interesting case of a 67-year-old man with 3 weeks history of lethargy, loss of appetite, generalised weakness and weight loss. Following thorough investigations, occult malignancy was ruled out. Hyponatraemia was a consistent finding which needed further classification. Additional workup including cerebral imaging and neurophysiological studies excluded cerebral vascular events and myopathies. Vasculitis screening was undertaken, and the diagnostic dilemma was revealed by the temporal arteries Doppler ultrasound which showed classical ‘halo sign’. Diagnosis of temporal arteritis was made and linked with syndrome of inappropriate antidiuretic hormone secretion, which appears to be a rare association with few instances on record. Patient was treated with high dose of prednisolone with marked improvement of clinical features.

Giant cell arteritis complicated by tongue necrosis and bilateral cerebellar ischaemic stroke

Giant cell arteritis (GCA) typically presents with headache, scalp tenderness or visual disturbance. Other symptoms include orofacial pain, constitutional symptoms and ischaemic stroke. An 81-year-old woman with a background of type-2 diabetes and hypertension presented with headache, oral pain and right visual loss. Examination showed hypertension, nodular temporal arteries, reduced visual acuity and suspected oral candida. Inflammatory markers were raised and she was diagnosed with GCA and commenced on corticosteroids. During treatment she developed tongue ulceration, then acute vertigo and incoordination with nystagmus and ataxia. Neuroimaging confirmed bilateral, cerebellar ischaemic strokes and temporal artery biopsy was consistent with GCA. With corticosteroids and secondary prevention of stroke measures she is now functionally independent. Oral pain is an uncommon symptom of GCA and delays in recognition may lead to catastrophic consequences. Clinicians should be aware of uncommon presentations and to optimise additional ischaemic stroke risk-factors.

Vascular anatomy of temporal fossa

Τα ενέσιμα υαλουρονικού οξέος εφαρμόζονται για τη διόρθωση παραμορφώσεων του κροταφικού βόθρου που σχετίζονται με την ηλικία, παρέχοντας αύξηση μαλακών ιστών καθώς και υποστήριξη αυτών (ιστών). Αν και γενικά θεωρούνται ασφαλή, σημαντικές ανεπιθύμητες ενέργειες (ΑΕ) έχουν συσχετιστεί με χρήση αυτών. Η ενδελεχής γνώση της ανατομίας της περιοχής, καθώς και η αξιολόγηση των κινδύνων και τα οφέλη που σχετίζονται με τις διαφορετικές τεχνικές έγχυσης, είναι θεμελιώδεις για τη διασφάλιση βέλτιστων αποτελεσμάτων και την αποφυγή των ΑΕ. Ειδικότερα, η αγγείωση της κροταφικής περιοχής απαιτεί ιδιαίτερη προσοχή καθώς μπορεί να σχετίζεται με σοβαρές ΑΕ, όπως η αρτηριακή απόφραξη, ισχαιμία και εμβολή. Στα πλαίσια αυτά, διερευνήσαμε την ανατομία του κροταφικού βόθρου και τον τρόπο με τον οποίο σχετίζεται με τις αγγειακές AE ύστερα από ενέσιμα υαλουρονικού οξέος. Επιπλέον, εξαιτίας της έλλειψης δεδομένων στον τομέα της αισθητικής καθώς και των επιπτώσεων στις βαθιές κροταφικές αρτηρίες (deep temporal arteries), διερευνήσαμε και αξιολογήσαμε την ανατομία της περιοχής και την επίδραση αυτών των ενέσιμων θεραπειών επί των αγγείων.

A case of giant cell arteritis lacking typical symptoms presenting with recurrent cerebellar infarctions: A case report and case-based review

ABSTRACT Giant cell arteritis (GCA) occasionally presents with ischaemic stroke. Generally, symptoms related to GCA or elevated levels of inflammation markers would be a clue for the diagnosis of GCA. However, we encountered a rare case of GCA that presented with recurrent cerebellar infarctions without symptoms related to GCA (headache, fever, or jaw claudication). Furthermore, C-reactive protein levels, measured at the time of two of the stroke attacks, were within the normal range. On physical examination, the temporal arteries were prominent and weakly pulsatile. Temporal artery ultrasonography showed halo signs, and temporal artery biopsy revealed GCA. To our knowledge, this is the first case of GCA presenting with recurrent ischaemic stroke lacking GCA features but diagnosed before death. Considering this case-based review, we suggest that GCA may have been missed in elderly patients with ischaemic stroke, especially in those with posterior circulation infarction. Therefore, physical examination of the temporal arteries, temporal artery ultrasonography, and vessel wall magnetic resonance imaging may be useful in those patients.

The sensitivity of temporal artery biopsy in the detection of giant cell arteritis is independent of tissue length

Introduction/Objective Giant cell arteritis (GCA) is the most common vasculitis of the elderly, and the most common primary systemic vasculitis overall, with an annual incidence of 200/million. The long term sequelae, namely vision loss and stroke, are permanent and devastating. While GCA is often treated empirically based on clinical presentation, panarteritis on temporal artery biopsy is required for diagnosis. However, these biopsies have the tendency to be falsely negative due to skip lesions, a common feature of GCA. Therefore, we set out to determine whether longer biopsy specimens were more sensitive in the detection of GCA. Methods/Case Report A census of temporal artery biopsies performed with the indication of clinical symptoms of GCA was taken at our institution. The patient age, sex, biopsy laterality, biopsy length, and pathological diagnosis were recorded for each cataloged sample. Statistical significance of difference in biopsy length was tested using an unpaired t-test in R 4.1.0. Results (if a Case Study enter NA) A total of 114 temporal artery specimens were biopsied from 94 different patients with the indication of GCA and assigned a definitive positive or negative diagnosis. Of the 94 patients, 54 were female and 40 were male. Of the total pathological specimens, 11 were positive and 103 were negative. The overall average length of biopsy specimens was 2.13 cm with a standard deviation of 0.65 cm. The average positive biopsy was 2.26 cm long, and the average negative was 2.12 cm, an insignificant difference (0.14 cm, t = 0.7, p = 0.43). In 25 patients, biopsies were taken from both the left and right temporal arteries. Of those patients, 2 were positive for GCA and the remaining 23 were negative. Interestingly, the biopsy result in every case was identical between the left and right samples; we found no instances of pathological evidence of GCA in only one of the two samples from the same patient. Conclusion According to data taken at our institution, there is no indication to lengthen the biopsy requirements from the existing 1.5 cm. However, we have demonstrated evidence that it may be unnecessary to biopsy both temporal arteries in a single patient. Larger studies would be required to confirm our findings.

Targeted RNA Sequencing of Formalin-Fixed, Paraffin-Embedded Temporal Arteries From Giant Cell Arteritis Cases Reveals Viral Signatures

Background and ObjectivesVaricella zoster virus (VZV) antigen has been detected in temporal arteries (TAs) of individuals with giant cell arteritis (GCA), the most common systemic vasculitis in older adults. Thus, we explored the contribution of VZV to GCA pathogenesis.MethodsFormalin-fixed, paraffin-embedded TA sections from biopsy-positive GCA participants with VZV antigen (GCA/VZV-positive; n = 20) and without (GCA/VZV-negative, n = 20) and from normal participants with VZV antigen (control/VZV-positive, n = 11) and without (control/VZV-negative, n = 20) were analyzed by targeted RNA sequencing of the whole human transcriptome (BioSpyder TempO-Seq). Ingenuity pathway analysis and R-computational program were used to identify differentially expressed genes and pathways between groups.ResultsCompared with control/VZV-negative TAs, GCA/VZV-negative and GCA/VZV-positive TAs were significantly enriched for human transcripts specific for pathways involved in viral infections, including viral entry, nuclear factor kappa B activation by viruses, and other pathogen-related immune activation pathways. Similarly, human gene sets supporting viral infection were found in control/VZV-positive TAs that showed no morphological signs of inflammation, suggesting that the enriched pathways were not nonspecific signatures of infiltrating immune cells. All GCA TAs and control/VZV-positive TAs showed enrichment of transcripts involved in vascular remodeling, including smooth muscle cell migration.DiscussionThe detection of viral and immune activation pathways in GCA TAs supports a role for virus infection in GCA pathogenesis. In addition, the detection of viral pathways in control/VZV-positive TAs, along with vascular remodeling pathways, suggests that these samples may represent early infection with progression to clinical disease, depending on host and other environmental factors.

Glucocorticoid Effects on Tissue Residing Immune Cells in Giant Cell Arteritis: Importance of GM-CSF

Giant cell arteritis (GCA) is a systemic granulomatous vasculitis clinically characterized by a prompt response to glucocorticoid therapy. Dendritic cells (DCs) play a central role in the pathogenesis of the disease and are increased in temporal arteries from GCA patients. The aim of this study was to determine the effects of glucocorticoid therapy on granulomatous infiltrates and on peripheral DCs of GCA patients. Immunohistochemical staining of temporal artery specimens from 41 GCA patients revealed a rapid reduction of the number of DCs after initiation of glucocorticoid treatment. TUNEL staining was performed to quantify apoptotic S100+ DC, CD3+ T cells, and CD68+ macrophages in the granulomatous infiltrates. An increase of apoptotic cells up to 9 ± 2% after 4–5 days of glucocorticoid therapy and up to 27 ± 5% (p < 0.001, compared to earlier timepoints) after 6–10 days was detected. A decrease of CCL19 and CCL21 expression was observed after starting glucocorticoid therapy. Granulocyte-macrophage colony-stimulating factor (GM-CSF) expression also significantly decreased under glucocorticoid therapy. No GM-CSF expression was detected in the control specimens. Glucocorticoid therapy leads to a rapid, time-dependent reduction of DCs in temporal arteries from GCA patients and reduction of mediators for cell migration. Our data suggest GM-CSF as a novel therapeutic target of GCA.

Hyperechogenic intimal lesions and wall thickness of the temporal and facial arteries in elderly patients with arterial occlusions of the eye

ObjectiveTo determine the association of arteriosclerosis, characterised by hyperechogenic intimal lesions (HIL), with wall thickness of the temporal and facial arteries in elderly patients with ocular arterial occlusions.MethodsPatients suffering from non-arteritic ocular perfusion disorders were included. High-resolution compression sonography (18 MHz) images of the temporal arteries (frontal and parietal branch at the upper margin of the auricle) and facial arteries (at the crossing point of the artery over the mandible) were analysed for the presence of HIL (grade 0: absent; grade 1: moderate; grade 2: severe). Characteristics of patients with and without evidence of HIL >grade 1 were compared.ResultsIn total, 330 cranial artery segments of 55 patients were analysed. HIL ≥grade 1 was present in 13.0% of all artery segments and in 38.1% of all patients. Patients with HIL ≥grade 1 in at least one arterial segment displayed significantly increased maximum wall thickness of the temporal arteries (0.62±0.23 mm vs 0.50±0.13 mm; p<0.01) and facial arteries (0.71±0.20 mm vs 0.54±0.19 mm; p=0.01). Patients with at least one temporal or facial artery segment with HIL were older, more often male and more frequently suffered from diabetes mellitus.ConclusionThe presence of HIL goes along with a significantly increased wall thickness of the temporal and facial arteries. These findings should be considered when interpreting the results of sonography of the cranial arteries in the diagnostic workup of suspected giant cell arteritis.

Imaging Tests in the Early Diagnosis of Giant Cell Arteritis

Early recognition of giant cell arteritis (GCA) is crucial to avoid the development of ischemic vascular complications, such as blindness. The classic approach to making the diagnosis of GCA is based on a positive temporal artery biopsy, which is among the criteria proposed by the American College of Rheumatology (ACR) in 1990 to classify a patient as having GCA. However, imaging techniques, particularly ultrasound (US) of the temporal arteries, are increasingly being considered as an alternative for the diagnosis of GCA. Recent recommendations from the European League Against Rheumatism (EULAR) for the use of imaging techniques for large vessel vasculitis (LVV) included US as the first imaging option for the diagnosis of GCA. Furthermore, although the ACR classification criteria are useful in identifying patients with the classic cranial pattern of GCA, they are often inadequate in identifying GCA patients who have the extracranial phenotype of LVV. In this sense, the advent of other imaging techniques, such as magnetic resonance imaging (MRI), computed tomography (CT), and positron emission tomography (PET)/CT, has made it possible to detect the presence of extracranial involvement of the LVV in patients with GCA presenting as refractory rheumatic polymyalgia without cranial ischemic manifestations. Imaging techniques have been the key elements in redefining the diagnostic work-up of GCA. US is currently considered the main imaging modality to improve the early diagnosis of GCA.