Giant Cell Vasculitis Research Articles

Differential diagnostics of polymyalgia rheumatica in a university hospital in Finland

Objectives Diagnosing polymyalgia rheumatica (PMR) can be difficult as many conditions present with similar symptoms and findings. This study aimed to analyse how often the diagnosis of PMR changes during follow-up in a university hospital setting and to determine the most common clinical conditions initially misdiagnosed as PMR. Method All patients with a new primary diagnosis of PMR on at least one visit during the years 2016–2019 were identified from the hospital discharge register of Turku University Hospital, Finland. A diagnosis of PMR was confirmed if the patient met at least one of the five classification criteria, complete clinical follow-up (median 34 months) was compatible with PMR, and no other diagnosis better explained their condition. Results Of the patients initially diagnosed with PMR, 65.5% were considered to have PMR after further evaluation and clinical follow-up. The most common conditions initially diagnosed as PMR were inflammatory arthritides (34.9%), degenerative or stress-related musculoskeletal disorders (13.2%), infection (9.3%), malignancy (9.3%), giant cell vasculitis (6.2%) and other vasculitis (6.2%), and a wide range of other less common diseases. The diagnosis of PMR remained in 81.3% of patients who fulfilled the 2012 American College of Rheumatology/European League Against Rheumatism PMR classification criteria and in 45.5% of patients who did not. Conclusions Diagnosing PMR is challenging, even in a university hospital. One-third of the initial diagnoses of PMR changed during further evaluation and follow-up. There is a substantial risk of misdiagnosis, especially in patients with atypical presentation, and the differential diagnoses of PMR must be considered carefully.

A Slice in the Right Direction – Novel use of Directional Atherectomy for Treatment of Upper Limb Vascular Insufficiency and Concurrent Diagnosis

Large-vessel vasculitides are well-recognized entities, affecting the aorta, supra-aortic branch vessels, cranial vessels, and less commonly, peripheral arteries. Giant cell arteritis (GCA) is one of the most frequent large-vessel vasculitides affecting elderly patients. Despite diagnostic criteria, the mainstay of definitive diagnosis has been temporal artery biopsies. More recently, adjuvant ultrasound has been used to identify skip lesions before the biopsy. Downstream ischemia affecting branch vessels has traditionally been managed by open revascularization due to patient age and risk of disease recurrence. Experience with endovascular strategies for the management of acute or chronic vasculitides has been steadily increasing. We present an unusual case of acute giant cell vasculitis, diagnosed and treated with the use of an endovascular atherectomy device. The patient was a 74-year-old woman, with prior history of polymyalgia rheumatica, long-standing immunosuppression, and new onset of significant left arm claudication and rest pain. She underwent treatment for a high-grade left axillary artery lesion with directional atherectomy and drug-coated angioplasty. She had a good angiographic result. The intraoperative atherectomy specimen revealed the presence of acute GCA which prompted treatment with an acute vasculitis regimen. She had good patency of the treated segment at 3-month follow-up with complete resolution of her symptoms.

Cystic Neutrophilic Granulomatous Mastitis

Granulomatous lobular mastitis (GLM) is an infrequent inflammatory breast disease, which can simulate malignancy on the basis of clinical and imaging features. It is typically found in parous or lactating women. The differential diagnosis of granulomatous mastitis includes infections caused by bacterial, fungal or mycobacterial agents, and autoimmune disorders like rheumatoid arthritis, sarcoidosis, giant cell vasculitis, and granulomatosis with polyangiitis. Cystic neutrophilic granulomatous mastitis (CNGM) is a sporadic sub-type of GLM that can be linked with infection with Gram-positive bacilli, specifically, Corynebacterium. It is characterised by lipogranulomas that comprise of "cystic" spaces lined by neutrophils which may contain Gram-positive rod-shaped bacteria. The majority of cases of GLM are still "idiopathic" as this pattern of inflammation still remains a mystery. We herein report three cases of CNGM and review the relevant literature. Key Words: Granulomatous mastitis, Corynebacterium, Gram-positive rods, Inflammatory breast disease, Cystic neutrophilic granulomatous mastitis.

Active Giant Cell Vasculitis Diagnosis with 68Ga PSMA PET/CT Imaging.

Vasculitis is a multisystem disease characterized by inflammation with infiltration of leukocytes into the blood vessels. Giant cell arteritis (GCA) is the most common form of vasculitis that mostly affects medium- and large-sized arteries. 18Fluorine-fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) is increasingly used to diagnose inflammation of large arteries in GCA. Galium-68 prostate-specific membrane antigen (PSMA) PET/CT has a vital role in the assessment of patients with prostate cancer for recurrence and metastasis of the disease. Various benign and non-prostate malignant conditions may give rise to increased PSMA uptake. Herein, we demonstrate that PSMA uptake can be seen in GCA.

CHANGE OF LUNG MECHANICS AND DEAD SPACE FRACTION IN PATIENTS WITH DIFFUSE ALVEOLAR HEMORRHAGE REQUIRING PRONE POSITIONING: A CASE SERIES

SESSION TITLE: Fellows Diffuse Lung Disease Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: October 18-21, 2020 INTRODUCTION: Diffuse alveolar hemorrhage (DAH) is a catastrophic syndrome causing respiratory failure (1). Prone positioning improves oxygenation and decreases mortality in patients with acute respiratory distress syndrome (2). In patients with refractory hypoxemia from DAH, prone positioning may be used as rescue therapy. However, we know little about the impact of prone positioning in lung mechanics and gas exchange of patients with DAH. CASE PRESENTATION: Four patients were admitted to the intensive care unit for refractory hypoxemia requiring prone positioning, and ultimately diagnosed with DAH — three through bronchoscopy and one through autopsy. Table 1 shows the demographics, laboratory data, and interventions. Two patients were male and two patients were female. Ages ranged from 19 to 71 years. The etiologies of DAH included: acute lymphoblastic leukemia, pulmonary angiosarcoma, and giant cell vasculitis; one patient had no identified etiology. One patient had pancytopenia from leukemia and the remaining three had leukocytosis and anemia with hemoglobin count from 8.5 to 10.4 g/dL on admission. No significant coagulopathy was noted but a high fibrinogen level was observed in all cases. Three patients had positive bacterial growth in sputum cultures. All patients required vasopressors and neuromuscular blockade prior to prone positioning. Three received pulse-dose steroids (at least 1g of methylprednisone daily for three days) after the diagnosis was made. Table 2 shows the ventilatory settings, gas exchange, and lung mechanics at baseline. Prior to prone positioning, PaO2/FiO2 ratios were less than 150 and dead space fraction ranged from 27% to 60%. Figure 1 shows gas exchange and lung mechanics at different time points. Following prone positioning, all patients had decreased dead space fraction after 8 hours of prone positioning. No specific pattern was identified in the change of lung mechanics and gas exchange while minimal change was observed in mechanical power. DISCUSSION: The histopathologic changes in DAH reveal a distinctive mechanism of lung injury affecting lung mechanics and gas exchange. In an autopsy study comparing ARDS patients with and without DAH, those with DAH had significantly lower static compliance and PaO2/FiO2 (3). Prone positioning could theoretically ameliorate V/Q mismatch and reduce inflammation in DAH. The finding of dead space fraction improvement following prone positioning suggests a possible benefit of applying prone positioning to DAH patients with refractory hypoxemia. Future studies are needed to examine the role of prone positioning in this population. CONCLUSIONS: We present the first case series of change in lung mechanics and dead space fraction in patients with DAH who received prone positioning. While there exists a paucity of evidence to support this modality, prone positioning can potentially be used as a rescue therapy for refractory hypoxemia in DAH. Reference #1: Lara AR, Schwarz MI. Diffuse alveolar hemorrhage. Chest. 2010;137(5):1164-71 Reference #2: Guérin C, Reignier J, Richard JC, Beuret P, Gacouin A, Boulain T, et al. Prone positioning in severe acute respiratory distress syndrome. N Engl J Med. 2013;368(23):2159-68 Reference #3: Thille AW, Peñuelas O, Lorente JA, Fernández-Segoviano P, Rodriguez JM, Aramburu JA, et al. Predictors of diffuse alveolar damage in patients with acute respiratory distress syndrome: a retrospective analysis of clinical autopsies. Crit Care. 2017;21(1):254 DISCLOSURES: No relevant relationships by James Kornfield, source=Web Response No relevant relationships by Chi Chan Lee, source=Web Response No relevant relationships by Stephanie Nonas, source=Web Response

SAT0255 THE RATE OF RELAPSE AMONG PATIENTS WITH LARGE VESSEL VASCULITIS AND THE SYSTEMIC INFLAMMATORY RESPONSE AS A POSSIBLE PREDICTOR FOR RELAPSE

Background:Large vessel vasculitides are known relapsing diseases. However, the rate of relapses has been seldom addressed and there are only few data on relapse predictors.Objectives:We conducted the present study to investigate the prevalence of relapses in the first year after diagnosis and the overall relapse among patients with large vessel vasculitis. Furthermore, we aimed to identify if the systemic inflammatory response (SIR) is a possible predictor for relapse among patients with large cell vasculitis.Methods:The systemic inflammatory response (SIR) has been described as a potential clinical and serological score predicting the risk for relapses1. SIR estimates the systemic inflammatory activity at the time point of first diagnosis1. It was defined as follows: Temperature >38°C, weight loss >4kg, Haemoglobin <11 g/dl and erythrocyte sedimentation rate >85 mm/h. For each of the above-mentioned criteria one point was attributed, leading to a range from 0 to 4 points. Patients with 3 to 4 points were considered having a highly inflammatory response and patients with an SIR ≤ 2 were considered having a low inflammatory response and thus a lower risk for relapses. Relapses are defined as reappearance of disease-related symptoms requiring treatment adjustment. The study cohort included 75 patients with large cell vasculitis (Giant Cell Vasculitis, Takayasu Vasculitis, inflammatory non-infections Aortitis), longitudinally followed by the authors over a mean period of 5.2 ± 3.3 years (range 1-14 yr).Results:The study-cohort includes 71 patients with a mean age at diagnosis of 63.5 (16 – 85) years. Almost three quarters (73%) of the patients were women. Most of the patients were suffering from GCA (73.2%), followed by Takayasu arteritis (16.9%) and inflammatory non-infections Aortitis (9.8%). 38 patients (53.5%) relapsed at least once during the follow up, and 17 patients had two or more relapses. The vast majority of relapses (86.8%) were observed within the first year following diagnosis. Most of the patients, 54 patients (76%), were considered having a low inflammatory response (SIR <=2). The relapse rate in this group was 59.2%. On the other hand, there were 17 patients having an SIR higher or equal to 3 points. The relapse rate in this group was 33%.Conclusion:In conclusion, the results of this preliminary study reveal that the relapse rate among patients with large vessel vasculitis high is. The SRI appears to be an inadequate predictor for relapse in this cohort.

Kimura’s Disease in the West- Case Report and Review of Literature

We report a 58 yr old lady, who had right temple swelling for 2 years, which on excision biopsy showed features of vasculitis. During the Rheumatology review, Clinical evaluation didn’t reveal any features suggestive of Giant cell arteritis and inflammatory markers were normal. She had localised granulomatous changes without giant cells and pathologist gave the opinion as Kimura’s disease. It has been described in East Asia especially in Japan as Juvenile Giant cell vasculitis (also known as Kimura’s disease (KD). It was reported by Kimura et al . as chronic inflammatory disorder of unknown aetiology with granulomatous changes mimicking vasculitis but no giant cells. It is very rare in Caucasians and early diagnosis will result in appropriate treatment and sparing the immunomodulatory treatment. This case is presented to increase awareness of KD and to highlight the features which may aid the diagnosis.

Atypical inflammation of the middle ear

The objective of the present study was to characterize the patients presenting with atypical inflammation of the middle ear and consider the currently available methods for their examination. A total of 20 patients at the age from 16 to 66 years were admitted to the Department of Ear Microsurgery during the period from 2008 and 2016 for the treatment of atypical inflammation of the middle ear. Eleven of them (18 ears) were found to have tuberculous lesions (TL) of the middle ear while the remaining 9 patients (11 ears) suffered giant cell vasculitis (GCV). All the patients underwent the general clinical and otorhinolaryngological examination, computed tomography of the temporal bones and the thoracic cavity organs, cytological, bacteriological, pathomorphological, and molecular-genetic studies including PCR diagnostics, rheumatological tests, as well as counseling by a phthisiotherapist and rheumatologist. The primary localization of TL in the middle ear was documented in 6 patients including its association with lung lesions in 5 cases. The clinical picture of the disease in 5 patients was that of smoldering exudative pathology and in 6 ones was accompanied by suppurative perforative otitis media. According to the laboratory analyses, bacteriological diagnostics proved efficient in 9% of the patients, pathomorphological and cytological diagnostics in 18% and 27.3% of the cases respectively while the effectiveness of PCR diagnostics was estimated at 55%. The diagnosis in individual patients was established within the period from 1 month to 1.5 years after they first sought medical advice in connection with complaints of the ear disease. Tuberculosis of the middle ear began to develop as exudative middle otitis that acquired the form of bilateral pathology in 4 patients. Three patients had a concomitant pulmonary disease. In 4 patuents, the diagnois of middle ear tuberculosis was established based on the presence of the specific antibodies and in 5 ones based on the results of the pathomorphological study. All the studies were carried out in duplicate. The period between the beginning of the disease and the establishment of the definitive diagnosis varied from 1 month to 1 year.

18F-fluorodeoxyglucose positron emission tomography and the risk of subsequent aortic complications in giant-cell arteritis: A multicenter cohort of 130 patients.

Previous studies reported a 2- to 17-fold higher risk of aortic complications (dilation or dissection) in patients with giant-cell arteritis (GCA). We aimed to determine whether or not GCA patients with large-vessel involvement demonstrated by positron emission tomography with 18F-fluorodeoxyglucose combined with computed tomography (FDG-PET/CT) have a higher risk of aortic complications. We conducted a retrospective multicenter study between 1995 and 2014. Patients were included if they fulfilled at least 3 American College of Rheumatology criteria for GCA, or 2 criteria associated with extratemporal biopsy-proven giant-cell vasculitis; they underwent at least 1 FDG-PET/CT scan at diagnosis or during follow-up; and the morphology of the aorta was assessed by medical imaging at diagnosis. Patients with an aortic complication at the time of diagnosis were excluded. Of the 130 patients included [85 women (65%), median age 70 (50–86)], GCA was biopsy proven in 77 (59%). FDG-PET/CT was performed at diagnosis in 63 (48%) patients and during the follow-up period in the 67 (52%) remaining patients. FDG-PET/CT was positive in 38/63 (60%) patients at diagnosis and in 31/67 (46%) patients when performed during follow-up (P = NS). One hundred four patients (80%) underwent at least 1 morphological assessment of the aorta during follow-up. Nine (9%) patients developed aortic complications (dilation in all and dissection in 1) at a median time of 33 (6–129) months after diagnosis. All of them displayed large-vessel inflammation on previous FDG-PET/CT. A positive FDG-PET/CT was significantly associated with a higher risk of aortic complications (P = 0.004).In our study, a positive FDG-PET/CT was associated with an increased risk of aortic complications at 5 years.

Magnetic resonance imaging in the diagnosis and follow-up of giant cell arteritis: case report and review of literature.

A female patient with giant cell vasculitis of the abdominal aorta and its branches and strongly suspected of having extrapulmonary tuberculosis is presented. The diagnoses were based on the clinical picture, laboratory findings, and magnetic resonance imaging (MRI) findings. MRI is highly useful in cases where echosonography and/or vascular biopsy for histopathological analyses are not possible. A combination of giant cell vasculitis and extrapulmonary tuberculosis is extremely rare, and therefore, choosing the right treatment presents a considerable challenge. MRI performed after 6-month antituberculous therapy and 1-year glucocorticoid plus methotrexate therapy showed normal wall of the aorta and its branches, which was consistent with clinical and laboratory remission. Patients with large vessel vasculitis require regular follow-up by MRI.

OP0001 [18F]Fdg PET in Giant-Cell Arteritis: A Prognostic Tool for Aortic Complications

BackgroundExtracranial involvement of large vessels in giant-cell arteritis (GCA) is probably underdiagnosed. Aortic complications (dilatation and dissection) are a prominent cause of death. [18]F-fluorodeoxyglucose positron-emission tomography ([18F]FDG-PET) is an imaging...

Efficacité clinique et biologique du tocilizumab au cours de la maladie de Horton : à propos de trois observations et revue de la littérature

IntroductionTreatment of giant cell arteritis is based on prolonged corticosteroid therapy but adverse side effects are common especially in the elderly. Case reportsWe report three patients with giant cell vasculitis treated by tocilizumab, an interleukin-6 receptor antibody, owing to resistance or intolerance to corticosteroid therapy. A favorable outcome was rapidly observed both on clinical and biological data allowing a corticoid therapy sparing. ConclusionTocilizumab is a promising treatment of giant cell arteritis but controlled trials are needed to confirm its efficacy.

AB0038 B cell disturbances differ between takayasu arteritis and giant cell vasculitis

BackgroundTakayasu arteritis and giant cell vasculitis are two forms of large vessel disease mostly affecting women. One of their main differences is the age at onset this being around 20...

Giant cell arteritis in a patient with fever of unknown origin

Fever of unknown (FUO) origin challenging clinical condition. The causes of FUO are diverse: possible etiologies include infectious diseases, chronic granulomatous diseases, malignity, connective tissue disorders and vasculitis. Giant cell arteritis which affects the aorta and its main branches, contributes up to 17% of cases with fever of unknown origin above age 50. There is increasing evidence for a role for18F-FDG PET/CT in the diagnosis of vasculitis and18F-FDG PET/CT is a useful technic for evaluation of the FUO. Clinical studies show that FDG-PET is a useful diagnostic aid in obscure cases of fever of unknown origin. We report here a case of a 54-year-old woman who present to our clinic with FUO due to giant cell vasculitis.

Successful Treatment of a Patient with Giant Cell Vasculitis (Horton Arteritis) with Tocilizumab a Humanized Anti-Interleukin-6 Receptor Antibody

Giant cell arteritis (GCA) is the most common form of systemic vasculitis in adults, affecting preferentially medium-large size arteries. Here we report a case of a female with a diagnosis of GCA based on temporal artery biopsy, successfully treated with tocilizumab, a humanized anti-interleukin-6 receptor antibody.

Spontaneous bilateral necrosis of the tongue: a manifestation of giant cell arteritis?

Giant cell arteritis is a chronic vasculitis, which involves large- and medium-sized branches of the arteries originating from the aortic arch. This disease is a diagnostic challenge with a wide range of clinical symptoms and findings due to different affected vessels. Classic symptoms are temporally located headache, thickened temporal artery and jaw claudication. Furthermore, visual symptoms like diplopia or loss of vision can occur. The tongue has an excellent blood supply and ischemic ulceration due to giant cell vasculitis is usually unilateral and rarely described in literature. We present a patient with a spontaneous bilateral tongue necrosis and are convinced that this extraordinary case must be deemed to be a manifestation of giant cell arteritis, although it does not completly satisfy the usually used diagnostic criteria formulated by the American College of Rheumatology.

La péricardite : une manifestation de la maladie de Horton

Introduction Temporal arteritis is a vasculitis in which inflammatory manifestations mainly involve the external carotid artery area but not exclusively. Through a clinical observation and a review of the literature, we suggest that inflammatory pericarditis could represent a manifestation of temporal arteritis. Exegesis A 75-year-old-woman was admitted for progressive physical deterioration which had been evolving for three months, associated with fever, frontotemporal cephalalgia and severe biological inflammatory syndrome. Chest X-ray reveals a cardiomegaly and suggests a pericarditis, which was rapidly confirmed by echocardiogram. Temporal artery biopsy concludes to the diagnosis of a giant cell arteritis. Steroids treatment is prescribed, leading to a rapid regression of the inflammatory state and the pericarditis without relapse after 6 months of follow-up. Conclusion Only prospective studies on systematic echocardiography when faced with the diagnosis of giant cell arteritis, whatever clinical symptoms, will enable to appreciate the prevalence and prognosis value of this manifestation. Moreover, temporal artery analysis seems to be justified when faced with a sub-acute or chronic “idiopathic” inflammatory pericarditis occurring in the elderly patient. Physiopathogeny is unknown but some hypothesis can be proposed: inflammatory cytokines storm, immune complexes deposition, giant cell vasculitis of pericardial arteries or inflammatory interstitial lesion of the pericardium with or without granuloma.

Syndrome d'Ortner et maladie de Horton

Introduction Ortner's syndrome was first described as a left laryngeal nerve palsy caused by a dilated left atrium in mitral stenosis. Aortic aneurysm is another well-documented etiology. Case record We report the case of a 90 year-old woman with temporal artertis with recent onset hoarseness, and simultaneous discovery of aortic arch aneurysm and left vocal cord palsy. Discussion The occurence of hoarseness and aortic aneurysm in Giant-cell vasculitis is discussed. We suggest to consider Horton's disease (GCA) as a possible etiology of Orner's cardiovocal syndrome.

Isolated (localized) idiopathic granulomatous (giant cell) vasculitis in an intramuscular lipoma

Isolated (localized) idiopathic granulomatous vasculitis (IGV) is an uncommon, heterogeneous, and poorly defined group of disorders characterized by infiltration of the arterial wall caused by compactly grouped mononuclear phagocytes, with or without giant cells, in segmental distribution. We report on a 55-year-old woman with IGV limited to an intramuscular lipoma of the left thigh. The vasculitis was identified incidentally upon microscopic examination of the removed tumor. The IGV was centered on two medium-sized arteries, accompanied by narrowing of the lumens, and not associated with secondary changes such as infart or postinfart fibrosis. The inflammatory infiltrate was rich in T-lymphocytes and macrophages, with the presence of giant cells. The patient was asymptomatic and well in a follow-up period of 2 months, during which she was not treated. To our knowledge, this is the first report of lipoma involvement in localized IGV. It is important to distinguish cases of isolated intratumorous IGV from systemic disease, because the latter implies a poor prognosis and requires an aggressive treatment.

Vasculitis of the central nervous system.

Central nervous system (CNS) vasculitis occurs in a variety of clinical settings. Some exhibit a distinct age preference; others a tissue tropism. Most frequently encountered are giant cell arteritis (temporal arteritis) and vasculitis secondary to infections. The CNS may be involved in the systemic vasculitides, and neurologic abnormalities occasionally appear as a presenting manifestation of disease. Isolated angiitis of the CNS, a rare form of vasculitis restricted to the CNS, must be distinguished from other causes of CNS inflammation and from noninflammatory vascular disease. We are learning a great deal about the cellular mechanisms of vascular inflammation in the brain. Some manifestations of the clinical disease result from histologic features of the infiltrate and the size of affected vessel. However, the local consequences of inflammation such as increased coagulation and altered vasomotor tone, as well as the systemic consequences such as activation of the central noradrenergic systems, trigeminovascular system, and hypothalamic pituitary adrenal axis contribute to both pathogenesis of disease and recovery. Two central issues that confront us now are improving the accuracy of the diagnosis (including identifying any underlying infectious causes) and limiting the long-term damage both from disease and its therapies.