Postpartum watershed strokes as initial manifestation of a rare co-occurrence of systemic lupus erythematosus and Takayasu Vasculitis: A case report of neuroimmunologic and literature review

Objective: To highlight the characteristics of pulmonary arterial involvement in Takayasu arteritis. Methods: The clinical and radiological data of a patient with Takayasu arteritis presenting with unilateral pleural effusion were studied and relevant literature was reviewed. The key words, "Takayasu arteritis" and "pleural effusion" were analyzed through literature retrieval in databases. Results: This 58 year-old female patient presented with shortness of breath. The chest CT scan showed bilateral hilar enlargement and pleural effusion on the left side. The blood pressure was not measurable in the course of the disease. After the aorticopulmonary-arteriography, we found that the pulmonary artery and the subclavian artery were involved. The diagnosis of Takayasu arteritis was made, and glucocorticoid therapy was initiated, with significant clinical and radiological improvement after therapy. Literature review found 4 cases of Takayasu arteritis with unilateral pleura effusion, ranging from 32 to 35 years of age, with a female predominance(Female∶Male=3∶1). The chief complaints were fever, chest pain and hemoptysis. All the patients recovered after the treatment of glucocorticoids. Conclusions: Takayasu arteritis presenting with unilateral pleural effusion was easily misdiagnosed as primary pulmonary diseases. Careful physical examination and timely angiography can be used to make the diagnosis.

SAT0546 Sensorineural hearing loss in takayasu’s arteritis

SAT0335 Differential Modulation of The Chromogranin-A System in Takayasu Arteritis and Systemic Lupus Erythematosus

IntroductionLarge-vessel vasculitis in older patients is typically associated with giant cell arteritis (GCA) usually presenting with cranial features. GCA can also present with a primarily extra-cranial pattern as large-vessel GCA (LV-GCA).Takayasu arteritis is a large-vessel vasculitis that affects younger females and classically involves the aorta and its major branches. This condition can clinically manifest with claudication of the extremities, impaired peripheral pulsation and systemic symptoms. Features of Takayasu arteritis and LV-GCA can overlap. This case reviews that of a 65-year-old lady with features of non-cranial large-vessel vasculitis and the approach taken to manage her case.Case descriptionA 65-year-old female developed pains affecting the shoulder and forearms over a period of weeks. She additionally noted pins and needles of the affected limbs. Symptoms were worse with use and consistent with claudication. She denied systemic symptoms, headache, visual changes or early morning stiffness.She was referred to the emergency department for assessment. Blood pressure could not be obtained and radial pulses were absent. She was subsequently arranged for further investigation via the vascular team. CT angiogram of the upper limbs demonstrated diffuse significant subclavian and axillary artery stenoses bilaterally with appearances deemed suggestive of underlying vasculitis.She was referred for rheumatological assessment. CRP was 50 and MRA aorta confirmed findings identified on CT scan. Prednisolone was initiated at a dose of 60mg daily for 4 weeks, and was subsequently decreased at an initial rate of 10mg every 2 weeks. Methotrexate was started at a dose of 15mg weekly.Inflammatory markers were noted to improve and CRP decreased to normal range. Despite this, although her paraesthesia resolved, she reported only a modest improvement of her upper limb claudication symptoms.Blood tests were monitored, however shortly after starting methotrexate, ALT significantly increased to 1002 IU/L. Autoimmune and viral hepatitis screen returned negative, US abdomen was suggestive of fatty liver changes but was otherwise unremarkable. Rise in ALT was attributed to methotrexate use and this was stopped, and levels improved. Mycophenolate was thus introduced as an alternative at a dose of 1g BD, but unfortunately resulted in recurrence of raised ALT.At present, she is being managed with prednisolone alone, of which is slowly being reduced. Her case is being additionally reviewed at our local vasculitis centre based at Hammersmith Hospital for consideration of tocilizumab should there be difficulties on steroid reduction.DiscussionTakayasu arteritis is a rare systemic large vessel vasculitis. Incidence is 1-2 per million annually with a female preponderance of 80-90%. Based on ACR classification criteria, the above patient would meet the diagnosis of this condition. Takayasu arteritis would normally be expected for those aged <40 years and has a median onset of 25-30 years. Due to her age, the diagnosis of large vessel vasculitis would be therefore be more consistent with LV- GCA, primarily in the absence of cranial features. Aorta and branch involvement can occur in up to 15% of GCA cases. Histologically, both GCA and Takayasu arteritis share similar findings and cannot be relied upon for diagnostic differentiation. Due to overlap of features, formal diagnostic labelling can be difficult in cases such as the one described.In regards to the management of her case, the general principle was taken of initial high dose prednisolone which has been gradually reduced. Introduction of DMARD therapy was implemented early which would be more typical for Takayasu arteritis rather than GCA. Unfortunately due to deranged ALT attributed initially to methotrexate, and subsequently mycophenolate, she is not currently on any steroid sparing treatment. Studies have demonstrated efficacy of IL-6 inhibition in managing large vessel vasculitis and as mentioned, her case is being reviewed with our local vasculitis centre for consideration of tocilizumab should there be difficulties on prednisolone weaning.Although CRP is now within normal range with treatment, our patient has ongoing claudication symptoms of the upper limbs. It is felt that this is likely due to residual vascular stenotic changes rather than current active vasculitis. As such, following stabilisation of her condition, consideration would be made for vascular surgical intervention in future.Key learning pointsDiagnosis of Takayasu versus giant cell arteritis can present a diagnostic challenge in some older patients due to overlap of typical features. The underlying process of large vessel vasculitis and shared components in both these conditions suggest they are within the same spectrum of disease. We discussed our management approach of high dose prednisolone which would be typically utilised in either diagnosis.In our case, due to drug induced hepatitis from methotrexate and subsequent mycophenolate, current management is with prednisolone alone. In regards to long term steroid sparing therapy, other options including IL-6 inhibition is being considered pending response to current treatment. Vascular surgical intervention for residual stenoses will also be reviewed following stabilisation of underlying inflammation.Conflicts of interestThe authors have declared no conflicts of interest.

Giant cell arteritis (GCA, also called temporal arteritis) is a rare and Takayasu arteritis (TA) is an even rarer autoimmune disease (AID), both of which present with inflammatory vasculitis of large and medium size arteries. The risk factors are largely undefined but disease susceptibility has been associated with human leukocyte antigen locus. Population-level familial risk is not known. In the present nation-wide study we describe familial risk for GCA and for GCA and TA with any other AID based on the Swedish hospital diagnoses up to years 2012. Family relationships were obtained from the Multigeneration Register. Familial standardized incidence ratios (SIRs) were calculated for offspring whose parents or siblings were diagnosed with GCA, TA or any other AID. The number of GCA patients in the offspring generation was 4695, compared to 209 TA patients; for both, familial patients accounted for 1% of all patients. The familial risk for GCA was 2.14, 2.40 for women and non-significant for men. GCA was associated with 10 other AIDs and TA was associated with 6 other AIDs; both shared associations with polymyalgia rheumatica and rheumatoid arthritis. The results showed that family history is a risk factor for GCA. Significant familial associations of both GCA and TA with such a number of other AIDs provide evidence for polyautoimmunity among these diseases.

Advances in autoimmune rheumatic diseases

BackgroundCase reports and series suggest that Takayasu's arteritis (TA) can co-exist with various inflammatory disorders.ObjectivesWe conducted a formal study to look specifically at the frequency of inflammatory disorders and symptoms...

Fever and Back Pain with Abdominal Aorta Tenderness: Takayasu Arteritis

Introduction. Kawasaki syndrome (KS) is infrequent in children under 1 year of age; an incidence of 0.23% of KS in infants under 60 days of age has been informed. Case report. A 60 days old male was admitted with an irrelevant past history; he presented fever of 40° C of 1 week and a polymorphic and erithematous rash, as well as the other classical signs of KS. Coronary aneurysms were demonstrated with an echocardiography. He was managed with acetylsalicylic acid, a single dose of intravenous immunoglobulin (2 g/kg weight), and a second dose of immunoglobulin due to lack of response to the first one. Conclusions. KS should be considered in the differential diagnosis of infants with fever and rash, even if they are less than 60 days of age.

Takayasu's arteritis (TA) represents a rare autoimmune disease (AD) characterized by systemic vasculitis that primarily affects large arteries, especially the aorta and the aortic arch and its main branches. Genetic components in TA are largely unknown. PTPN22 is a susceptibility loci for different ADs; however, the role of different PTPN22 single-nucleotide polymorphisms (SNPs) in the susceptibility to TA is not clear. We evaluated the PTPN22 R620W (C1858T), R263Q (G788A), and - 123G/C SNPs in a group of patients with TA and in healthy individuals from Mexico. Our study included 111 patients with TA and 314 healthy individuals. Genotyping was performed with the 5' exonuclease (TaqMan®) assay. Our data showed that the PTPN22 R620W polymorphism is a risk factor for TA (CC vs. CT: OR 4.3, p = 0.002, and C vs. T: OR 4.1, p = 0.003); however, the PTPN22 R263Q and - 1123G/C polymorphisms are not associated with this AD. In addition, the PTPN22 CGT haplotype, which carries the minor allele of the PTPN22 C1858T variant, was also associated with TA susceptibility. This is the first report documenting an association between PTPN22 R620W and TA.

Contemporary Challenges of Acute Ischemic Stroke in Takayasu Arteritis.

Takayasu vasculitis is a rare type of large vessel vasculitis that primarily affects the aorta and its main branches; signs and symptoms are usually due to systemic inflammation or ischemia of an organ or limb, depending on the group of blood vessels involved. In addition, Takayasu arteritis is associated with increased platelet and coagulation activity, leading to a hypercoagulable state and thrombus formation. We report a case of a 47-year-old male who presented with a history of complete anuria for 3 days and was found to have progressively worsening kidney function. Renal Doppler ultrasound confirmed the presence of bilateral renal artery thrombosis, while Contrast- Enhanced Computed Tomography (CECT) of the abdomen and pelvis showed extensive abdominal aortic thrombosis with radiological findings consistent with large vessel vacuities. After catheter-directed thrombolytic therapy of the renal arteries, the patient started producing urine and his kidney function significantly improved. Later, Positron Emission Tomography scan (PET) confirmed large vessel Takayasu arteritis. Echocardiography showed no intracardiac thrombus, along with an extensive work up for thrombophilia, as autoimmune and vasculitis serology came back negative. This is an extremely rare presentation of Takayasu arteritis, with an unusual recovery of acute renal failure after delayed anuria due to bilateral renal artery thrombosis.

Myocardial inflammation often coexists with different types of autoimmune diseases. Our aim was to investigate the presence of myocarditis in these patients by Cardiovascular Magnetic Resonance (CMR) and endomyocardial biopsy. Twenty patients, aged 20-55 yrs with autoimmune diseases and cardiac symptoms (3 with Takayasu's arteritis, 3 with systemic lupus erythematosus, 5 with rheumatoid arthritis, 7 with autoimmune thyroid disease and 2 with systemic sclerosis) and 20 patients with the same autoimmune diseases but without cardiac symptoms (controls) were studied. The presence of myocarditis and LV function were evaluated by CMR. Myocarditis was documented using T2-weighted (T2-W), T1-weighted (T1-W) before and after contrast media injection and late enhanced images. In 10 patients (positive for myocarditis by CMR with either low LVEF or recent increase in troponin), endomyocardial biopsy was also performed. Myocardial specimens were evaluated by histology and polymerase chain reaction techniques (PCR). Myocarditis was identified in 18/20 patients by CMR. In the T2-W images the signal ratio of myocardium to skeletal muscle was 1.89+/-0.25 (control values 1.57+/-0.13, p<0.05). From the T1-W images the relative myocardial enhancement was 11.31+/-11.18 (control values 3.09+/-0.05, p<0.05). Epicardial late gadolinium enhanced areas were identified in 18/20. In myocardial specimens, histology revealed inflammation in 5/10 (50%) and PCR documented viral or microbial genomes in 8/10 (80%). Positive histology and PCR were in agreement with 50% and 80% of positive CMR examinations, respectively. Herpes virus was identified in 3/10, Adeno in 1/10, Coxsackie B6 in 1/10, echo in 1/10, Parvo-B19 in 3/10, CMV in 1/10 and Chlamydia trachomatis in 8/10. Myocardial inflammation is a common finding in patients with autoimmune diseases and cardiac symptoms. The diagnosis can be confirmed by CMR, which is a noninvasive and reliable tool for the investigation of these patients.

EDITORIAL article Front. Immunol., 31 May 2023Sec. Autoimmune and Autoinflammatory Disorders : Autoimmune Disorders Volume 14 - 2023 | https://doi.org/10.3389/fimmu.2023.1222776

THU0299 Understanding the heterogeneity of large-vessel vasculitides