A case of repetitive myocardial infarction with unobstructed coronaries due to Churg-Strauss syndrome

© The Author(s) 2019. Published by Oxford University Press on behalf of the European Society of Cardiology.BackgroundMyocardial infarction is most commonly caused by thrombosis occurring on a background of coronary atherosclerosis, resulting in reduced coronary flow. Less often, myocardial infarction can occur in the absence of coronary disease. The pathomechanism of myocardial infarction in such patients is heterogeneous and more challenging to diagnose and treat. European Society of Cardiology published a position paper on myocardial infarction in patients with non-obstructive coronary disease, with definitions and recommendations for investigations, in what has hitherto been an under-recognized and under-investigated Cinderella-like condition. However, the importance of obtaining a diagnosis is all the more important, since one treatment approach with revascularization and antithrombotic treatment does not ‘fit all’.Case summaryA 70-year-old male patient presented with chest pain at rest, associated with rise in troponin and without ECG changes. A diagnosis of non-ST elevation myocardial infarction was made. Coronary angiography showed a smooth stenosis which resolved with administration of intracoronary nitrate. A diagnosis of coronary artery spasm was made, and treatment initiated. After 18 months, the patient had recurrent chest pains at rest, unresponsive to glyceryl trinitrate (GTN). Cardiac magnetic resonance revealed extension of subendocardial infarction, without inducible ischaemia. CT coronary angiogram (CTCA) showed non-obstructive coronaries. Blood tests showed significant eosinophilia, raised troponin, and C-reactive protein (CRP) that fluctuated without correlation with symptoms or any ECG changes. A diagnosis of Churg–Strauss syndrome was made, and immunosuppression commenced.DiscussionChurg–Strauss syndrome is an autoimmune vasculitis in patients with history of atopy or late-onset asthma which when involving coronary arteries can lead to myocardial injury mimicking acute coronary syndrome (ACS). Identification is important to allow initiation of immunosuppression which can prevent development or progression.Peer reviewedFinal Published versio

ANCA-associated vasculitis – Should we change the standard of care?

Collaborative clinical trials over the last 25 years have revolutionised the care of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. This has led to production of management recommendations and standards of care. This paper reviews the existing standards and the recent evidence that has fed further evolution of standards of care. Pattern recognition remains vital to early diagnosis and therefore initiation treatment. While cyclophosphamide remains the treatment of choice, the advent of rituximab has been shown to be beneficial to patients with relapsing disease. It may be safer in young females and those with a risk of urothelial cancers. Methotrexate and mycophenolate mofetil may not be as good as previously thought for inducing remission. Azathioprine and rituximab are the standards for remission maintenance. There have been recent changes to the nomenclature of vasculitides. It is possible that these will continue to evolve over time to make them more meaningful and inform treatment and prognosis. In the absence of gold-standard biomarkers, we discuss the role of ANCA and histopathology, especially in the Indian setting. Follow-up and monitoring of these patients should include structured evaluation using validated clinical tools, assessing cardiovascular risk, vigilance for infections and other co-morbidities due to exposure to glucocorticoids and immunosuppression

Two cases of lymphoepithelial cyst of the pancreas

A 35-year-old man was found to have a cystic mass in the pancreatic body on a routine health examination ; high serum CA19-9 was also detected. The enucleated cyst was diagnosed as a lymphoepithelial cyst (LEC). A 74-year-old man found to have a cystic mass in the pancreatic head by computer tomography as well as high serum CA19-9 was suspected of a cystic neoplasm of the pancreas (IPMN), and pylorus-preserving pancreaticoduodenectomy (PPPD) was performed. Pathologically, the cyst was found to be LEC. It is often difficult to diagnose pancreatic cyst as LEC preoperatively. Care should be taken not to do over-surgery for benign disease LEC

Response to: "Renal biopsies should be performed whenever treatment strategies depend on renal involvement"

We thank Chemouny et al for their letter and concur with their conclusions. As we state (1): “A positive biopsy for AAV is helpful when considering an initial diagnosis or recurrent disease.” In our view, renal biopsy is important to establish diagnosis and may also provide an indication of prognostic trajectory and although existing classification systems need further validation, changes like glomerular sclerosis have obvious adverse prognostic value for patients with AAV (2-4). The Delphi process, for the scope of the current recommendations, identified the role of biopsy at both diagnosis and follow-up as an important item for update. Histopathological evidence of vasculitis, such as pauci-immune glomerulonephritis or necrotising vasculitis in any organ, remains the gold standard for diagnostic purposes. The likely diagnostic yield varies and is dependent on the organ targeted and in patients with GPA with renal involvement can be as high as 91.5% from renal biopsy (5). As Chemouny and colleagues have demonstrated, a renal biopsy was definitive in determining their management decisions. However during follow-up when relapses occur, it may be prudent to consider judicious use of further kidney biopsy during suspected renal relapse since the cause for acute kidney injury may be due to another cause other than AAV (6). Kind regards, M Yates, C Mukhtyar and DR Jayne on behalf of co-authors

How to treat ANCA‑associated vasculitis:practical messages from 2016 EULAR/ERA‑EDTA recommendations

The European League against Rheumatism (EULAR) with the European Renal Association - European Dialysis and Transplant Association recently published an update of 2009 EULAR recommendations with a focus on the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV). In this article, we discuss the following key messages for clinical practice derived from these recommendations: 1) biopsy should be performed if possible to confirm new diagnosis or relapse; 2) glucocorticoid therapy is an extremely important adjunct to the management of AAV, but it is also responsible for the majority of adverse effects; the dose should be tapered to 7.5 to 10 mg/d at 3 to 5 months; 3) cyclophosphamide or rituximab are the mainstay of remission induction; 4) patients with major relapse should be treated like those with new disease, but rituximab is the preferred option in those patients who relapse after prior cyclophosphamide; 5) minor relapse should not be treated with glucocorticoid alone, and a change in immunosuppressive regimen should be considered; 6) rituximab can be used not only for remission induction but also for maintenance; 7) maintenance therapy should continue for at least 2 years, after which gradual taper could be considered; 8) while ANCA are extremely useful for diagnosis and rising ANCA levels seem to be associated with relapse, serial monitoring should not guide treatment decisions; 9) monitoring of AAV patients should be holistic with a structured assessment tool and monitoring for effects related to the vasculitis as well as treatment; 10) management should be either at or in conjunction with an expert center; and 11) patients should be involved in decision making and have access to educational resources

Behçet's pulmonary artery aneurysms treated with infliximab and monitored with the 6-min walk test

Pulmonary involvement in Behçet's disease (BD) is uncommon; however, it is potentially fatal due to the risk of massive haemoptysis. We describe the case of a 36-year-old male presenting with a 2-month history of worsening dyspnoea, weight loss, haemoptysis, oral ulceration, erythema nodosum and superficial thrombophlebitis. He was diagnosed with pulmonary vasculitis secondary to BD; however, his symptoms were refractory to initial treatment with cyclophosphamide, azathioprine and prednisolone. We therefore trialled infliximab alongside methotrexate, which led to a remarkable improvement in his condition, enabling eventual discontinuation of prednisolone. Whilst not being one of the treatments currently recommended for managing pulmonary involvement in BD, infliximab has previously been successfully used in cases refractory to conventional therapy. We used the 6-min walk test (distance covered and lowest oxygen saturations) to monitor his progress, which correlated with his symptoms. This may represent a useful adjunct in monitoring the activity of pulmonary vasculitis

The diagnosis, assessment and outcomes of primary systemic vasculitis

We have created definitions for ultrasonographic abnormalities of Giant Cell Arteritis. The ‘halo’ sign is a ‘homogenous, hypoechoic wall thickening, well delineated towards the luminal side, visible both in longitudinal and transverse planes, most commonly concentric in transverse scans.’ At the superficial temporal artery, the interobserver reliability in acquired and dynamic images has a k = 0.87 and 0.60 respectively; the intraobserver reliability in acquired images and live exercises has a k = 0.88 and 0.71 respectively. Ultrasonography is more reliable (k = 0.8) than temporal artery biopsy (k = 0.4) when compared against physician verified diagnosis at 100-week follow-up. Ultrasonography of 25 patients may be enough for service validation if audited against biopsy and long-term outcomes. Activity and Damage form the twin sides of vasculitis assessment. We have validated the Birmingham Vasculitis Activity Score v3 in two separate studies with convergent validity against treatment decision (r = 0.54) and excellent interobserver reliability (ICC = 0.996). A new Combined Damage Assessment index had lower interobserver (ICC = 0.78) and intraobserver reliability (ICC = 0.87) vs the Vasculitis Damage Index (ICC = 0.94 and 0.92 respectively). Granulomatosis with Polyangiitis, Microscopic Polyangiitis and Eosinophilic Granulomatosis with Polyangiitis have remission rates of 30%-93%, 75%-89% and 81%-91% respectively. The 5-year survival is 74%-91%, 45%-76% and 60%-97% respectively. At diagnosis, the quality of life as measured by the Short Form – 36 is worse than normative data. Older age and neurologic involvement at baseline are associated with lower physical composite scores. My work has resulted in improvements in the diagnosis of Giant Cell Arteritis, assessment of primary systemic vasculitis and understanding outcomes in Antineutrophil Cytoplasm Antibody associated vasculitis. They have also informed the research agenda for further developments in the field

The Role of Ultrasound Compared to Biopsy of Temporal Arteries in the Diagnosis and Treatment of Giant Cell Arteritis (TABUL): a diagnostic accuracy and cost-effectiveness study

Background: Giant cell arteritis (GCA) is a relatively common form of primary systemic vasculitis, which, if left untreated, can lead to permanent sight loss. We compared ultrasound as an alternative diagnostic test with temporal artery biopsy, which may be negative in 9–61% of true cases. Objective: To compare the clinical effectiveness and cost-effectiveness of ultrasound with biopsy in diagnosing patients with suspected GCA. Design: Prospective multicentre cohort study. Setting: Secondary care. Participants: A total of 381 patients referred with newly suspected GCA. Main outcome measures: Sensitivity, specificity and cost-effectiveness of ultrasound compared with biopsy or ultrasound combined with biopsy for diagnosing GCA and interobserver reliability in interpreting scan or biopsy findings. Results: We developed and implemented an ultrasound training programme for diagnosing suspected GCA. We recruited 430 patients with suspected GCA. We analysed 381 patients who underwent both ultrasound and biopsy within 10 days of starting treatment for suspected GCA and who attended a follow-up assessment (median age 71.1 years; 72% female). The sensitivity of biopsy was 39% [95% confidence interval (CI) 33% to 46%], which was significantly lower than previously reported and inferior to ultrasound (54%, 95% CI 48% to 60%); the specificity of biopsy (100%, 95% CI 97% to 100%) was superior to ultrasound (81%, 95% CI 73% to 88%). If we scanned all suspected patients and performed biopsies only on negative cases, sensitivity increased to 65% and specificity was maintained at 81%, reducing the need for biopsies by 43%. Strategies combining clinical judgement (clinician’s assessment at 2 weeks) with the tests showed sensitivity and specificity of 91% and 81%, respectively, for biopsy and 93% and 77%, respectively, for ultrasound; cost-effectiveness (incremental net monetary benefit) was £485 per patient in favour of ultrasound with both cost savings and a small health gain. Inter-rater analysis revealed moderate agreement among sonographers (intraclass correlation coefficient 0.61, 95% CI 0.48 to 0.75), similar to pathologists (0.62, 95% CI 0.49 to 0.76). Limitations: There is no independent gold standard diagnosis for GCA. The reference diagnosis used to determine accuracy was based on classification criteria for GCA that include clinical features at presentation and biopsy results. Conclusion: We have demonstrated the feasibility of providing training in ultrasound for the diagnosis of GCA. Our results indicate better sensitivity but poorer specificity of ultrasound compared with biopsy and suggest some scope for reducing the role of biopsy. The moderate interobserver agreement for both ultrasound and biopsy indicates scope for improving assessment and reporting of test results and challenges the assumption that a positive biopsy always represents GCA. Future work: Further research should address the issue of an independent reference diagnosis, standards for interpreting and reporting test results and the evaluation of ultrasound training, and should also explore the acceptability of these new diagnostic strategies in GCA. Funding: he National Institute for Health Research Health Technology Assessment programme

Diagnostic utility of testing for antineutrophil cytoplasmic antibodies

Antineutrophil cytoplasmic antibodies (ANCA) that target the third neutral serine proteinase (PR3) and myeloperoxidase (MPO) of human neutrophils are associated with primary systemic vasculitis that predominantly affects small and medium blood vessels.1,2 There is an international consensus that testing for ANCA should specifically be focused on identifying antibodies against those 2 antigenic targets.3 In this edition of the journal, Wójcik et al4 discuss the data from the Polish Vasculitis Register (POLVAS) including information from 536 individuals who presented with vasculitis over 27 years, in the demographic context