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Temporal artery biopsy (TAB) is one of the diagnostic tools to confirm the diagnosis of giant cell arteritis (GCA). We aim to evaluate the clinical and technical determinants of a positive biopsy. Demographics, clinical, technical, and laboratory data of all TAB’s performed between 2007 and 2019 at a single academic medical center. 107 biopsies performed for 103 patients were included; 72.9% were female, and 27.1% were male. The mean age at the time of biopsy was 67.1 ± 9.3 years. One biopsy was excluded for lack of arterial tissue content. Of the remaining 106, 19.6% were positive. The length of the biopsy and the number of arterial cross-sections were not significantly associated with its result. A positive biopsy was seen more in patients with low albumin (p = 0.010) and hypothyroidism (p = 0.017) but less in those with prior glucocorticoids treatment (p = 0.028). Predictors of a positive biopsy included male gender [OR 4.029, 95% CI (1.330–12.209), p = 0.014]; elevated ESR [OR 3.998, 95% CI (1.908–6.787), p = 0.023]; polymyalgia rheumatica (PMR) symptoms [OR 5.121, 95% CI (2.094–9.872), p = 0.001]; and advancing in age (6.5% per every additional year), [OR 1.065, 95% CI (1.005–1.130), p = 0.033]. 53.7% of the patients were eventually diagnosed with GCA; 39.2% of them were based on positive biopsy. In conclusion, old age, male gender, elevated ESR, and PMR symptoms increase the odds of positive TAB. Technical factors, such as biopsy length and the number of cross-sections, did not influence eventual biopsy results, highlighting the pivotal role of the clinical presentation of the patients in selecting patients for TAB.

To study the clinical presentation, treatment and outcome of southern Chinese patients with Takayasu's arteritis (TA). This is a retrospective chart review study of 78 patients managed in 14 public hospitals in Hong Kong between the years 2000 and 2010. Patients were identified from the hospital registry using the ICD-10 diagnostic code of the disease. The classification of TA was based on the American College of Rheumatology (ACR) or modified Ichikawa's criteria. Demographic data, clinical presentation, angiographic findings, pattern of vascular involvement (Numano's classification), treatment and outcome of these patients were presented. 78 patients were studied (82% women, age at presentation 34.2 ± 14 years). The estimated point prevalence of TA was 11/million population. The commonest initial manifestations were hypertension (62%) and vascular ischemic symptoms (38%). Systemic symptoms occurred in nine (12%) patients only. The proportion of patients fulfilling the angiographic subtypes of the Numano's classification was: types I (13%), IIa (4%), IIb (12%), III (12%), IV (20%) and V (39%), respectively. Thirty-two patients (41%) were treated with high-dose glucocorticoids (GCs) and 22 patients (28%) received additional non-GC immunosuppressive drugs. Vascular complications occurred in 26 (33%) patients and revascularization surgery was performed in 23(29%) patients. Three (4%) patients died of vascular complication at a median of 8 years after disease onset. TA is rare in southern Chinese patients of Hong Kong. Most patients present with ischemic symptoms during the stenotic phase of the disease. Although mortality is low, a significant proportion of patients developed vascular stenosis that required surgical interventions. More awareness of TA as a differential diagnosis of non-specific systemic symptoms with elevated inflammatory markers in younger patients is needed for earlier diagnosis.

To assess the effectiveness of cyclophosphamide (CYC) versus methotrexate (MTX) for active Takayasu's arteritis (TA). The current study was based on a cohort of TA at Zhongshan Hospital, Fudan University. TA was diagnosed using the 1990 American College of Rheumatology criteria. Fifty-eight subjects receiving induction treatment with CYC (n = 46) or MTX (N = 12) were included in the analysis. Effectiveness and toxicity were assessed in all 58 cases. Clinical remission was defined as: Kerr score reduction to ≤ 1 and glucocorticoids (GC) treatment at a dose of ≤ 0.2 mg/kg/day (≤ 15 mg/day) at the end of the 6th month. At the baseline, the CYC group had higher Kerr scores (60.9% vs. 16.7% at ≥3, p = 0.044), higher ESR (55 ± 52 vs. 25 ± 22 mm/H, p = 0.048), ITAS_ESR (12.4 ± 1.7 vs. 9.1 ± 1.1 mg/L, p = 0.043). The 6-month clinical remission rate was 71.7% vs. 75% in the CYC and MTX group, respectively. In the CYC group, a significant decrease was observed in ESR (55 ± 52 vs. 25 ± 48 mm/H, p = 0.008), hs-CRP (27 ± 23 vs. 6.9 ± 6.6 mg/L, p = 0.007), ITAS (11.7 ± 2.2 vs. 7.0 ± 1.5, p = 0.048), and ITAS_ESR (7.1 ± 2.0 vs. 12.4 ± 1.7, p = 0.033). However, no significant reductions in these measures were demonstrated in the MTX group. Whole-body contrast enhanced magnetic resonance angiography (MRA) revealed significant radiologic improvement (wall enhancement scores: 4.2 ± 2.3 vs. 10.3 ± 3.8, p = 0.032) in the CYC group, but not in the MTX group. No severe adverse events occurred in any subject. Cyclophosphamide could be a better choice than methotrexate as induction treatment for patients with more severe Takayasu's arteritis.

The aim of this was to assess clinical features and outcome in pediatric-onset Takayasu's arteritis (TA). Retrospective data analysis of patients diagnosed with TA over last 13 years with onset before 18 years of age was done. Their presenting features, activity (by NIH criteria, ITAS2010, ITAS-A), disease extent (by DEI.Tak) and angiographic findings were retrieved from clinic files. Treatment received and follow-up data on disease activity and damage by TA damage score (TADS) were also analyzed. Wherever repeated angiography data were available, the same was analyzed. Values are expressed as median with interquartile range in brackets. There were 29 patients (19 females) with median age at diagnosis of 14 (13-16) years and delay to diagnosis of 1 (0.4-2) year. Common presenting symptoms were pulse loss (23/29) and hypertension (22/29). Patients had extensive disease at presentation with median DEI.Tak of 12 (9.5-15); 23/29 had elevated acute-phase reactants, and 28/29 were active at presentation [median ITAS2010 13 (8-15.5), ITAS-A 14 (10-17)]. Numano's type V was the commonest angiographic type (22/29). At a median follow-up of 2.4 (1.5-5.1) years, 2/20 were active whereas all had sustained damage despite a majority (17/20) being on immunosuppression. The median TADS was 8 (6.3-9.8) with pulse loss, claudication and hypertension being the commonest damage item. Two needed renal artery stenting to control hypertension. Angiographic assessment at least 2 years apart demonstrated disease progression in 5 of 6 patients despite immunosuppression. Significant damage accrued on follow-up despite immunosuppression and control of disease activity. Hypertension remains the major long-term morbidity.

Takayasu's arteritis (TA) rarely occurs in infants. There are only four reports on TA in children below 1 year of age, revealing diversity of its symptoms. We describe a 7-month-old female infant hospitalized for hectic fever, irritability, high acute phase indices, and coronary artery dilatations found on echocardiography, which suggested Kawasaki disease (KD). Despite of standard treatment, rapid development of thoracic and abdominal aorta aneurysms occurred, while coronary artery abnormalities regressed. The initial diagnosis was changed for TA. Subsequently implemented glucocorticosteroids led to clinical and laboratory normalization. However, 2 months after treatment discontinuation, TA relapsed. Glucocorticosteroid therapy was restarted with additional introduction of intravenous immunoglobulins (IVIG), resulting in full and stable remission lasting over 1 year. Thus, diagnosis and treatment of infant TA pose a big challenge to physicians. TA onset in infants may mimics KD. Prolonged glucocorticosteroids and IVIG administration can be recommended in the youngest patients with TA.

Takayasu's arteritis (TA) is an inflammatory vasculitis of aorta and its branches, its low incidence limited our recognition to this entity. We sometimes can confuse this disease with polyarteritis nodosa and other vasculitis when no conventional "big artery" involved in TA cases. Here we report a 26-year-old man with Takayasu's arteritis who presented with a provisional intracranial granulomatosis first and then saccular aneurysms between celiac trunk and arteria hepatica communis and many other proteus manifestations, which is seldom described before.

Cerebral vasculitis represents a rare form of vascular inflammatory involvement caused by heterogeneous conditions. In this study, the wide spectrum of cerebral vasculitis despite primary angiitis of the CNS is analyzed. Our cohort included 16 white patients with cerebral vasculitis treated in a single German institution between 2003 and 2008. Clinical and diagnostic features were obtained by retrospective chart review; follow-up information and outcome were obtained prospectively. The spectrum of conditions responsible for cerebral vasculitis included seven patients with Behçet syndrome and one case each of giant cell arteritis, Wegener's granulomatosis and Churg-Strauss syndrome, respectively. Vasculitis secondary to systemic diseases included two patients with systemic lupus erythematodes, one with sarcoidosis and one with ANA-positive systemic vasculitis. Two patients suffered from infectious angiitis caused by borreliosis and syphilis. The mean age at onset of cerebral symptoms was 41.38 years. The most frequent clinical symptoms were headache, gait disturbances and unilateral numbness. None of the patients with Behçet syndrome experienced any ischemic event, which was a significant difference compared with the other patients (P = 0.011). Gadolinium-enhancing lesions were significantly more frequent in Behçet syndrome compared to the other types of vasculitis (P = 0.041). There was no significant difference between vasculitis patients with or without Behçet syndrome regarding outcome parameters. The differential diagnosis of conditions responsible for cerebral vasculitis includes a wide spectrum of diseases. Clinical features and the course of cerebral vasculitis are highly variable. The enigma of cerebral vasculitis will only be solved by implementing large, prospective, multicenter databases.

Takayasu's arteritis (TA) is characterized by a chronic inflammatory arteriopathy with unknown etiology affecting large vessels. As TA occurs mostly in young females, elderly males with this disease may be under-diagnosed. A 65-year-old Chinese male with a 6-week history of fever, fatigue, low appetite, night sweats, and a 5-kg weight loss did not experience symptoms of jaw claudication, localized headache, sudden visual change, upper or lower extremity claudication, arthralgia, and rashes. On physical examination and laboratory findings, there was no evidence of infectious disease or malignancy. (18)F-Fluorodeoxyglucose positron emission tomography/computed tomography ((18)F-FDG PET/CT) was performed which suggested TA. The patient was treated with methylprednisolone and clopidogrel. His symptoms subsided gradually and hematological examination showed decreases of erythrocyte sedimentation rate and high-sensitivity C-reactive protein. We reported here a patient who presented with fever of unknown origin and was diagnosed with TA using PET/CT. Although elderly males are beyond the predominant population of TA, TA should be considered when there is no evidence of infectious disease and malignancy.

A 43-year-old woman reported pain in the right hypochondrium, which had started 3 years before and had been worsening for the past few days. Claudication in the superior and inferior limbs, diffuse myalgia, dyspnea, precordialgia followed by dizziness and visual turbidity were added to the clinical picture. In the physical examination bilateral carotid bruit was observed, abdominal aorta murmur and the decrease of the right radial and left pedis pulses and arterial hypertension with difference in the diastolic pressure between limbs >10 mmHg was also observed. On cardiac catheterisation with aortography, right coronary with proximal parietal irregularities, slight pressure increase in right chambers and pulmonary artery, preserved left ventricle contractility, competent valves, carotid and subclavian partial obstruction, severe narrowing of the abdominal aorta below the diaphragm (80%) and right renal artery significant stenosis were observed. Takayasu’s arteritis (TA) diagnosis was established according to the ACR criteria based on the clinical symptomatology, on physical and image test findings. Two years later she presented malar rash, photosensitivity, nephropathy, leukopenia, lymphopenia and hemolytic anemia confirming the systemic lupus erythematosus (SLE) diagnosis. TA coexisting with SLE has rarely been reported.

Raynaud's phenomenon is characteristic three-phase color change of digits that occurs when hands are exposed to cold and subsequently rewarmed. Raynaud's phenomenon has many possible causes, but evaluation tends to focus on a few notorious etiologies, such as, connective tissue diseases. Thus, having reached a diagnosis, detailed physical exam to rule out other possible causes is often not performed. The authors present a case of mixed connective tissue disease (MCTD) and Takayasu's arteritis overlap in a woman, who showed Raynaud's phenomenon as an initial manifestation. She was first diagnosed as having MCTD, but her treatment did not improve the persistent Raynaud's phenomenon. Several years later, follow-up chest CT showed underlying Takayasu's arteritis and a subsequent physical examination revealed that typical abnormalities consistent with Takayasu's arteritis were present. The authors advocate thorough history taking and complete physical examinations on a routine basis to help unearth other underlying causes.

Cardiac involvement is common in primary systemic vasculitides and may be due to direct effect of the disease on the heart or due to therapy. We shall review involvement of the heart in the various forms of primary systemic vasculitis. Among anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), eosinophilic granulomatosis with polyangiitis most commonly involves the heart. Involvement of the heart confers poorer prognosis in AAV, which is also complicated by increased risk of cardiovascular events. Kawasaki's disease (KD) is the most common form of medium-vessel vasculitis to affect the heart, with coronary artery aneurysms being the most common manifestation. These predispose patients with KD to develop premature ischemic heart disease. Takayasu's arteritis is the most common large-vessel vasculitis to involve the heart and can result in aortic incompetence, myocarditis, or coronary heart disease. Involvement of the heart in Behcet's disease is usually in the form of intracardiac mass lesions, thrombosis, or endomyocardial fibrosis. Drugs used in the treatment of systemic vasculitis influence the risk of developing cardiovascular events. Corticosteroid therapy has been shown to increase the risk of myocardial infarction, whereas methotrexate, azathioprine, mycophenolate mofetil, rituximab, and anti-tumor necrosis alpha agents favorably modulate the risk of cardiovascular events, predominantly by dampening systemic inflammation. Awareness of cardiac involvement in vasculitis and accelerated cardiovascular risk in these patients should help clinicians to maximize the modulation of modifiable risk factors for heart disease in these individuals.

Takayasu’s arteritis (TA) is a granulomatous large vessel vasculitis more commonly seen in India. The vascular inflammation in TA results in stenoses of affected vessels. Usually this is a slow process with good collateral circulation; only rarely does critical limb ischemia result. We present two patients of TA who presented with gangrene of extremities, and review eight prior such patients reported in the literature. With appropriate diagnosis and treatment with oral corticosteroids and low-dose aspirin, none of our patients had recurrence at a mean follow-up of 3.8 ± 2.8 years. Although rare, TA can present with gangrene and rheumatologists need to be aware of this unusual but limb-threatening manifestation of TA to institute appropriate therapy in a timely manner.

Bilateral anterior ischemic optic neuropathy and bilateral ocular ischemic syndrome have been rarely reported as initial manifestations of Takayasu's arteritis (TA). Appearance of ocular symptoms in TA is closely related to extension and severity of involvement of the aorta and its major branches. Here we reported a case of 'carotid steal syndrome' secondary to TA in a 45 year old Turkish man, who had severe ocular and brain ischemia, presented initially with symptoms of ocular ischemic syndrome.

Giant cell arteritis/temporal arteritis (GCA/TA) and polymyalgia rheumatica (PMR) are closely related conditions that affect middle age and older patients and frequently occur together. Many authorities consider them to be different phases of the same disease. A study was undertaken of the possible contribution of gallium-67 ((67)Ga) single photon emission tomography (SPECT) scintigraphy to the diagnosis of GCA/TA and PMR, and to support the possible hypothesis of vasculitic nature of PMR. Nine consecutive patients with PMR, and nine consecutive patients with GCA/TA were included in the study. All patients fulfilled the American College of Rheumatology criteria for GCA/TA and Healey's criteria for PMR. The control patients group consisted of those who underwent (67)Ga scintigraphy for febrile illness. All patients and controls underwent (67)Ga SPECT scan of the skull on a dual-head Helix gamma camera 2 days after I.V. injection of 8-10 mCi of (67)Ga citrate. (67)Ga uptake ratio of temporal bone region to bone out of temporal area (TR/Bone) was estimated on transaxial and coronary slices after SPECT scan reconstruction. For each patient and control the calculations of both temporal areas were done. All GCA/TA and PMR patients showed increased uptake in both temporal areas of the skull, while (67)Ga uptake among GCA/TA was the highest, 1.31+/-0.14 in tranaxial view and 1.47+/-0.16 in coronary view. (67)Ga uptake among PMR patients was lower, 1.19+/-0.11 and 1.28+/-0.13, respectively. In comparison, the control patients showed the lowest (67)Ga uptake, 1.04+/-0.09 in transaxial view and 1.11+/-0.06 in coronary view. The results differ significantly between the three groups. It seems that (67)Ga SPECT scan may become a useful tool in the investigation of patients with suspicion of GCA/TA and PMR. Our findings of the increased (67)Ga uptake in the temporal areas among PMR patients add new arguments for vasculitic nature of this disorder.

Takayasu arteritis (TA) is a chronic vasculitis of unknown etiology. Experience with anti-tumor necrosis factor alpha (anti-TNF) agents in difficult-to-treat patients with TA is limited and refers to adult patients. Here, we present two cases of pediatric TA treated with infliximab, in which clinical remission was observed. Anti-TNF treatment represents a useful therapy in pediatric Takayasu arteritis too, especially to avoid the risk of long-term corticosteroids toxicity.

Takayasu’s arteritis (TA) is a chronic vasculitis, primarily affecting large vessels, such as the aorta and its main branches. Several reports suggest that the vascular inflammatory process is not always confined to large vessels. Here, a new case of vasculitis in intra-muscular arteries associated with TA is reported. This case provides further support for the idea that TA also involves small arterial vessels.

Takayasu's arteritis (TA) is a rare, idiopathic, inflammatory, granulomatous vasculitis that affects the aorta and its primary branches. Clinical features and the pattern of arterial involvement show differences in different regions of the world according to ethnic influences. Our aim in this retrospective study was to evaluate the demographic, clinic, laboratory, and angiographic findings of 22 patients with TA followed by our clinic and also compare our results with series from the literature. The hospital files of the 22 patients followed by our clinic between 1998 and 2009 were retrospectively evaluated. We also compared our results with the series from the literature that we were able to reach by US National Library of Medicine, National Institute of Health. Gender distribution, age at diagnosis, and type of aortic involvement were similar with the study from Turkey. Different clinical manifestations of Takayasu's arteritis have been described in different ethnic groups. We also want to underline the coincidence of TA and other rheumatic diseases such as sarcoidosis, SLE, RA, and psoriatic arthritis, different from other published series.

Giant cell arteritis (GCA) is the most common systemic vasculitis affecting people over 50 years. This disease is a diagnostic challenge with a range of clinical symptoms and findings due to different affected vessels. Because of this, the initial diagnosis can be tricky, and some of the patients present at first time with a real unusual initial manifestation. One of these can be tongue necrosis, which is according to the literature in accordance with scalp necrosis, the rarest initial manifestation of GCA We describe a patient who presented with tongue necrosis as initial symptom of GCA. The belated diagnose resulted in subtotal necrosis of the mobile part of the tongue.

Takayasu's arteritis (TA) is a rare large vessel vasculitis that is difficult to diagnose in the early stages. Therefore, it is also very difficult to manage and prevent irreversible vascular damage in TA. A 19-year-old female patient with back pain was examined using [(18)F]-FDG-PET to detect the source of inflammation. Specific accumulation of [(18)F]-FDG was observed in the thoracic and abdominal aorta, leading to the diagnosis of TA. Corticosteroid treatment resulted in clinical remission. However, the serum amyloid A (SAA) levels remained elevated. A follow-up scan showed residual uptake of [(18)F]-FDG in the thoracic aorta suggesting subclinical vascular inflammation. Methotrexate was combined with the corticosteroid, and the elevated levels of SAA became normalized. The present case suggests that monitoring serum levels of SAA and [(18)F]-FDG-PET could help clinicians to make adequate treatment adjustments in TA patients.

Takayasu’s arteritis (TA) is a rare chronic vasculitis of the aorta and its main branches. Infectious agents or autoimmunity are thought to influence the pathophysiology. Ethnic preponderance in East Asia and usually affects young Asian women suggesting a possible role of genetic factor in the etiology. We present a rare case of familial TA in female siblings with the involvement of the main branches of aortic arch and renal arteries described by three-dimensional computed tomography (3D-CT). This case is rare familial TA in female siblings with CT angiography, which is feasible in diagnosis and informative for the stage of TA.

The prevalence of Takayasu’s arteritis (TA) varies greatly among world populations, and little is known about this disease in Eastern Mediterranean Arab populations. We conducted a retrospective chart review of patients diagnosed with TA from 1996 to 2008 at a single large referral center in Jordan. Eight patients (seven females, one male) with angiographically diagnosed TA were seen at the Jordan University Hospital between 1996 and 2008. All patients were of Arabic ethnicity. The age at presentation ranged from 14 to 50 years, and delay in diagnosis ranged from 1 to 10 years. Extra-vascular manifestations included nodular episcleritis, elevated liver enzymes, erythema nodosum, inflammatory-bowel-disease-like illness, Raynaud’s phenomena, and constitutional symptoms. Vascular symptoms included postural dizziness, central nervous system deficits, amauroses fugax, and transient ischemic attacks. Aortic arch vessels were involved in all patients, the abdominal aorta was involved in five patients, and the renal arteries in four patients. Major clinical events including severe stroke and cardiac failure were associated with mortality in two patients. Treatment with corticosteroids and immunosuppressive agents resulted in improvement in five patients with follow-up ranging from 3 to 12 years. In conclusion, TA is seen in Arabs, and the clinical spectrum of TA in Arabs in Jordan is similar to that reported in other countries. Increased awareness of the disease may shorten the time to diagnosis and result in a more reliable estimate of disease prevalence.

Temporal (giant cell) arteritis is a chronic vasculitis of large and medium sized vessels which usually occurs in individuals above 50 years of age. In patients less than 50 years temporal artery vasculitis is extremely rare. The clinical presentations of the vasculitis in younger patients appear to be different from the older patients. We present two case reports of temporal artery vasculitis in patients less than 50 years, one of them with human immunodeficiency virus infection. Both the cases had variable clinical presentations and good response to treatment.

Takayasu’s arteritis is a rare but potentially life-threatening disorder which may present for the first time in pregnancy. Although the cause is unknown, a link with infection has been suggested. Basic clinical examination skills with particular attention to cardiovascular assessment may help to diagnose this condition at an earlier stage and thus improve prognosis.

Color-coded sonography is an interesting option for the diagnosis of temporal arteritis. We present our experiences regarding examination technique and diagnostic accuracy, comparing biopsy and clinical results in a series of 83 patients with suspected temporal arteritis. A dark halo around the vessel wall (representing inflammatory oedema), reduced or absent vessel wall pulsations (demonstrated by M mode), and vessel occlusions were used as diagnostic criteria. Forty-eight patients underwent biopsy of the temporal artery following ultrasound examination. Comparing these findings with biopsy yielded a sensitivity of 73%, specificity of 93%, positive predictive value (PPV) of 96%, and negative predictive value (NPV) of 58%. The halo sign alone had a lower sensitivity (67%). Comparison with overall clinical assessment (n = 83) yielded a sensitivity of 65%, specificity of 100%, PPV of 100%, and NPV of 73%. Irregular atherosclerotic vessel wall changes were the main differential diagnosis. Important pitfalls were false focus setting, too much/less color gain, and 'bifurcation halo'. In conclusion, a positive sonographic result in combination with typical clinical signs might replace the need for biopsy, while a negative result should not be used for exclusion of temporal arteritis. Considering the low PPV and high NPV of the clinical criteria defined by the American College of Rheumatology, color-coded sonography is a useful tool in the noninvasive diagnostic workup of temporal arteritis.

Fuentes and colleagues described a case with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis suspected to be complicated by posterior reversible encephalopathy syndrome (PRES) [1]. This adds to the accumulating evidence that PRES may be associated with ANCA-associated vasculitis. However, we have some concern about the diagnosis of this case. PRES (also termed reversible posterior leukoencephalopathy syndrome, RPLS) represents a clinical and radiological syndrome characterised by reversible vasogenic oedema [2] in the posterior brain, which primarily arises from autoregulation failure and endothelial dysfunction [3]. The reversible vasogenic oedema, as most specifically and sensitively detected by apparent diffusion coefficient (ADC) map of diffusion-weighted imaging (DWI), preferably involving posterior white matter, and typical clinical manifestations like acute onset of headache, confusion or decreased level of consciousness, visual disturbance and seizures can help to diagnose a typical PRES. Although lesions might be revealed by T2-weighed imaging (T2WI) or fluid attenuated inversion recovery images (FLAIR) of magnetic resonance imaging (MRI), they are not specific for vasogenic oedema [4]. Atypical cases are not rare [3], whereas they should be diagnosed with caution, especially after rigid exclusion of other confounding disorders. Differential diagnoses of PRES include metabolic encephalopathy, inflammatory demyelinating diseases, vasculitis, etc. Connective-tissue diseases, including systemic lupus erythematosus (SLE), polyarteritis nodosa, Wegener’s granulomatosis, microscopic polyangiitis, Henoch Schonlein purpura, Takayasu’s arteritis and thrombotic thrombocytopenic purpura, have been suggested as disorders in which PRES can develop [1, 5–7]. The central nervous system (CNS) complications in connective-tissue diseases are not uncommon, which include vasculitis [8], acute or subacute infarction [9] and PRES [1, 5–7]. Demyelination, inflammatory lesions, ischaemia or oedema may present as reversible changes in the brain as revealed by neuroimaging modalities. Therefore, reversibility, not only clinical but radiological, and vasogenic oedema as detected by ADC map of DWI, as well as well-established risk factors predisposing to hypertension, such as phaeochromocytoma, glomerulonephritis, eclampsia and receiving cytotoxic and immunosuppressant drugs [3], should be confirmed by clinical and neuroimaging examinations to diagnose this case as a typical PRES. In summary, PRES represents a clinicoradiological syndrome, the diagnosis of which relies on typical clinical and neuroimaging findings.