scholarly journals Pulmonary Vasculitides: A Radiological Review Emphasizing Parenchymal HRCT Features

Diagnostics ◽  
2021 ◽  
Vol 11 (12) ◽  
pp. 2318
Author(s):  
Stefano Palmucci ◽  
Corrado Inì ◽  
Salvatore Cosentino ◽  
Luigi Fanzone ◽  
Stefano Di Pietro ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Clinical Manifestations ◽  
Case Series ◽  
Interstitial Lung Diseases ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Immune Mediated ◽  
Antineutrophil Cytoplasmic Autoantibody ◽  
Interstitial Disease ◽  
Eosinophilic Granulomatosis

Vasculitides represent a heterogeneous group of immune-mediated disorders, characterized by a systemic inflammatory destructive process of the blood vessels resulting either in ischemia or hemorrhage. The organ involved and vessel size influence the pattern of presentation of the pathology. The lung is commonly involved in systemic vasculitides, with heterogeneous clinical, radiological, and histopathological presentations. Primary vasculitides most commonly associated with lung parenchymal involvement include small-vessel antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides, such as granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), and microscopic polyangiitis (MPA). Several studies have reported cases of interstitial lung diseases (ILDs) associated with systemic vasculitis, particularly those positive for ANCA associated vasculitis/vasculitidis: AAV. We have selected from our case series different radiological features of pulmonary vasculitis (i.e., solitary or multiple nodules, cavitary lesions, nodules with centrilobular or peribronchial distribution, airspace consolidations, “crazy paving” appearance, interstitial disease), including cases with interstitial lung alterations. Therefore, the aim of this review is to describe the typical clinical manifestations of vasculitides and their main radiologic features (especially AAV).

scholarly journals Clinical case of eosinophilic granulomatous polyangiitis debuted by myocardial infarction

2021 ◽  
Vol 40 (2) ◽  
pp. 41-48
Author(s):  
Evgeniy V. Kryukov ◽  
Dmitriy V. Cherkashin ◽  
Sergey L. Grishaev ◽  
Sayora A. Turdialieva ◽  
Elena A. Mozharovskaya ◽  
...  
Keyword(s):  
Myocardial Infarction ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Clinical Manifestations ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Therapy Strategy ◽  
Granulomatous Polyangiitis ◽  
Diagnostic Biopsy ◽  
Eosinophilic Granulomatosis

The article presents clinical observations demonstrating the difficulties of diagnostic search in establishing the diagnosis of systemic vasculitis associated with antineutrophil cytoplasmic antibodies, which include a rare disease eosinophilic granulomatosis with polyangiitis (ChurgStrauss syndrome). Carefully collected anamnesis, participation of specialists of different profiles, retrospective analysis of laboratory and instrumental data allowed to verify the diagnosis, to prescribe adequate therapy. The aim of the publication is to discuss the need for early diagnosis of eosinophilic granulomatosis with polyangiitis, which can improve the effectiveness of therapy and improve the overall prognosis for this disease, taking into account modern approaches based on the main provisions of international recommendations that were prepared in 2015 with the participation of leading experts from Europe, USA and Canada and were called to become the basis for choosing a personalized patient therapy strategy. In some cases, the diagnosis of eosinophilic granulomatous polyangiitis is complicated by the diversity of the clinical picture, the non-simultaneous appearance of the main symptoms and the violation of the stages of the process. In the differential diagnosis of systemic vasculitis, assessment of initial clinical manifestations, testing for the presence of antibodies to the cytoplasm of neutrophils, multispiral computed tomography of the chest organs and diagnostic biopsy of the affected tissues are crucial. In the histological conclusion, a picture of focal ulcerative-necrotic lesions of the nasal mucosa with signs of vasculitis and a pronounced eosinophilic cell component of inflammation was noted. The distinctive features of this case include the onset of the disease with the development of myocardial infarction and early damage to the hearing organ in the form of cochlear neuritis, complicated by sensorineural hypoacusis. The combination of high doses of glucocorticosteroids and cyclophosphamide is still the gold standard for the treatment of severe cases, but the use of biological agents such as rituximab or mepolizumab seems to be a promising therapeutic alternative (4 figs, bibliography: 3 refs).

Rituximab for Eosinophilic Granulomatosis with Polyangiitis: a Systematic Review of Observational Studies

Rheumatology ◽  
2021 ◽  
Author(s):  
Vincenzo G Menditto ◽  
Giulia Rossetti ◽  
Diletta Olivari ◽  
Alessia Angeletti ◽  
Marco Rocchi ◽  
...  
Keyword(s):  
Systematic Review ◽  
Cohort Studies ◽  
Granulomatosis With Polyangiitis ◽  
Case Reports ◽  
Case Series ◽  
Biologic Agents ◽  
Cochrane Library ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Extensive Literature ◽  
Eosinophilic Granulomatosis

Abstract Objective to analyze the available evidence about the use of rituximab (RTX) and other biologic agents in Eosinophilic Granulomatosis with Polyangiitis (EGPA) patients and to provide useful findings to inform the design of future, reliable clinical trials. Methods A systematic review was performed. A systematic search was conducted in PubMed/MEDLINE, Scopus, Web of Science and the Cochrane library databases and an extensive literature search on other biologic agents. Results 45 papers pertinent to our questions were found: 16 retrospective cohort studies, 8 case series, 3 prospective cohort studies and 18 single case reports, for a total of 368 EGPA patients. More than 80% of evaluable patients achieved complete or partial remission with a tendency towards a higher rate of complete response in pANCA positive subgroup. Conclusion Although the majority of the evaluable EGPA patients treated with RTX appears to achieve complete remission, we strongly believe that a number of sources of heterogeneity impairs a clear interpretation of results and limits their transferability in clinical practice. Differences in design, enrollment criteria, outcome definition and measurement make a comparison among data obtained from studies on RTX and other biologic agents unreliable.

scholarly journals A Progressive Case of Eosinophilic Myocarditis Due to Eosinophilic Granulomatosis With Polyangiitis in a Caucasian Male

2020 ◽  
Vol 8 ◽  
pp. 232470962096685
Author(s):  
Valerie F. Civelli ◽  
Vishal K. Narang ◽  
Rupam Sharma ◽  
Ritika Sharma ◽  
Jessica Kim ◽  
...  
Keyword(s):  
Allergic Rhinitis ◽  
Inflammatory Process ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Small Vessels ◽  
History Of ◽  
Eosinophilic Myocarditis ◽  
Eosinophilic Granulomatosis ◽  
Large Vessels

Vasculitis is an inflammatory process involving blood vessels of various sizes, including the small vessels in the kidneys to the large vessels, such as the aorta. This inflammatory condition is usually autoimmune in nature and is associated with involvement of many locations, such as the sinuses, lungs, kidneys, and even the heart. Specifically, eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic vasculitis that may initially hide as asthma, allergic rhinitis, and/or sinusitis. However, it is known to become a lethal disease once progressed to include cardiovascular manifestations. It is important to remember EGPA as a differential for any patient with a history of asthma, allergic rhinitis, and/or sinusitis who also presents with cardiovascular complaints and eosinophilia. Treatment recommendations focus on immunosuppression in such cases. In this article, we discuss the case of a 62-year-old male, with a known history of asthma, who presented to the emergency department with concern for his chest pain and right-sided weakness. He was later diagnosed with EGPA with eosinophilic myocarditis. Diagnosis and treatment are described.

scholarly journals Peripheral neuropathy in antineutrophil cytoplasmic antibody-associated vasculitides

2019 ◽  
Vol 6 (6) ◽  
pp. e615 ◽  
Author(s):  
Antje Bischof ◽  
Veronika K. Jaeger ◽  
Robert D. M. Hadden ◽  
Raashid A. Luqmani ◽  
Anne-Katrin Pröbstel ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Nerve Biopsy ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Vasculitic Neuropathy ◽  
Diagnostic Certainty ◽  
Primary Systemic Vasculitis ◽  
Organ Manifestations ◽  
Eosinophilic Granulomatosis

ObjectiveReported prevalence of vasculitic neuropathy (VN) in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is highly variable, and associations with other organ manifestations have not been studied systematically while accounting for diagnostic certainty of VN.MethodsData of all patients with AAV within the Diagnostic and Classification criteria for primary systemic VASculitis study were analyzed cross-sectionally. VN was categorized as definite (histology proven), probable (multiple mononeuropathy or nerve biopsy consistent with vasculitis), or possible (all others). Associations with other organ manifestations were compared in patients with and without VN.ResultsNine hundred fifty-five patients (mean age 57 years, range 18–91 years, 51% female) were identified. Of these, 572 had granulomatosis with polyangiitis (GPA), 218 microscopic polyangiitis (MPA), and 165 eosinophilic granulomatosis with polyangiitis (EGPA). The prevalence of VN was 65% in EGPA, 23% in MPA, and 19% in GPA. Nerve biopsy was performed in 32/269 (12%) patients, demonstrating definite vasculitis in 17/32 (53%) of patients. VN was associated with myeloperoxidase-ANCA positivity (p = 0.004) and skin (p < 0.001), musculoskeletal, (p < 0.001) and cardiovascular (p = 0.005) involvement. Patients with VN were less likely to have renal (p < 0.001), eye (p < 0.001), and gastrointestinal (p = 0.023) involvement.ConclusionsOur study provides comprehensive insights into the prevalence and organ associations of VN in a large, systematically collected AAV cohort. VN is most commonly associated with skin, musculoskeletal, and cardiovascular manifestations. In routine clinical practice, diagnosis of VN is infrequently confirmed by the gold standard of nerve biopsy but rather supported by the clinical setting of active systemic AAV.

scholarly journals Eosinophilic Granulomatosis with Polyangiitis: Diagnostic Dilemmas

2018 ◽  
Vol 15 (1) ◽  
pp. 63-68
Author(s):  
Delia Tulbă ◽  
Răzvan Adrian Ionescu ◽  
Daniela Gologanu ◽  
Marius Ioan Balea ◽  
Simona Caraiola
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Histopathological Examination ◽  
Systemic Vasculitis ◽  
Transbronchial Biopsy ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Imaging Features ◽  
Necrotizing Vasculitis ◽  
Tissue Eosinophilia ◽  
Lung Abnormalities ◽  
Eosinophilic Granulomatosis

AbstractIntroduction. Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a systemic vasculitis of the small vessels that often associates asthma and blood/tissue eosinophilia.Case presentation. A 58-year-old woman was admitted to our hospital for progressive exertional dyspnea. She had been diagnosed with asthma three years earlier. Recent multiple thoracic computed tomography scans displayed non-fixed interstitial lung abnormalities, whereas the infectious workup (HIV, parasites) was negative.On admission, the clinical examination noted prolonged expiratory phase. Paraclinical tests revealed biological inflammatory syndrome, eosinophilia, polyclonal hypergammaglobulinemia, elevated total IgE level, negative anti-neutrophil cytoplasmic antibodies and proteinuria of nephritic pattern.The pulmonary evaluation reconfirmed the obstructive ventilatory dysfunction and interstitial lung abnormalities - interlobular septal thickening and diffuse ground-glass opacification. The histopathological examination of a transbronchial biopsy specimen identified leukocytoclastic necrotizing vasculitis and tissue eosinophilia.Provided the clinical and paraclinical setting, specifically the asthma, blood and extravascular eosinophilia, paranasal sinus abnormalities, non-fixed pulmonary infiltrates and the histopathologically confirmed necrotizing vasculitis, the diagnosis of EGPA seemed appropriate.Conclusion. In the absence of diagnostic criteria, EGPA diagnosis is often challenging. Although certain clinical and imaging features could assist the diagnosis, biopsy remains the diagnostic gold standard.In the setting of lung involvement, open lung biopsy is usually required for EGPA histopathological proof, but few cases diagnosed by transbronchial biopsy have been reported. This method often identifies discrete, nonspecific lesions or an incomplete spectrum of pathognomonic abnormalities but has the advantage of minimal invasivity that justifies its use as an alternative diagnostic technique.

scholarly journals Addison’s disease presenting with perimyocarditis

2018 ◽  
Vol 31 (1) ◽  
pp. 101-105
Author(s):  
Elisa Baranski Lamback ◽  
Grazia Morandi ◽  
Eleni Rapti ◽  
Georgi Christov ◽  
Paul A. Brogan ◽  
...  
Keyword(s):  
Systemic Inflammation ◽  
Granulomatosis With Polyangiitis ◽  
Clinical Manifestations ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Polyglandular Autoimmune Syndrome ◽  
Case Presentation ◽  
Prompt Treatment ◽  
Autoimmune Syndrome ◽  
Life Saving ◽  
Eosinophilic Granulomatosis

AbstractBackground:Polyglandular autoimmune syndrome (PGA) and eosinophilic granulomatosis with polyangiitis (EGPA) do not seem to represent a coincidental association.Case presentation:A case of a 15-year-old boy is reported who presented with severe systemic inflammation, perimyocarditis and cardiogenic shock, in whom EGPA was initially suspected and later diagnosed with autoimmune adrenalitis with PGA.Conclusions:The severity of the systemic inflammation and perimyocarditis suggests a more widespread autoimmune-mediated process. Autoimmune adrenal insufficiency should be considered in all cases of pericarditis and perimyocarditis, especially when the severity of clinical manifestations exceeds the expected for the severity of the cardiac findings, as timely identification and prompt treatment may be life-saving.

Revisiting the systemic vasculitis in eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

2017 ◽  
Vol 16 (1) ◽  
pp. 1-9 ◽  
Author(s):  
Vincent Cottin ◽  
Elisabeth Bel ◽  
Paolo Bottero ◽  
Klaus Dalhoff ◽  
Marc Humbert ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Eosinophilic Granulomatosis ◽  
Churg Strauss

Comparison of patient self-reported data to physician-reported data in eosinophilic granulomatosis with polyangiitis (Preprint)

2021 ◽  
Author(s):  
Irena Doubelt ◽  
Jason M. Springer ◽  
Tanaz A. Kermani ◽  
Antoine G. Sreih ◽  
Cristina Burroughs ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Clinical Manifestations ◽  
The United States ◽  
Research Network ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
American College Of Rheumatology ◽  
Organ Systems ◽  
Patient Reported ◽  
Reported Data ◽  
Eosinophilic Granulomatosis

BACKGROUND Patient-based registries can help advance research in rare diseases such as eosinophilic granulomatosis with polyangiitis (EGPA), a complex, multi-organ form of anti-cytoplasm neutrophil antibody (ANCA)-associated vasculitis. OBJECTIVE To compare patient-reported vs. physician-reported manifestations, treatments, and outcomes for patients with EGPA. METHODS Comparative analysis of patients ≥18 years with EGPA in Canada or the United States from two separate cohorts: i) The Vasculitis Patient-Powered Research Network (VPPRN), a self-enrolled, secure portal with patient-entered data updated quarterly (2013-2019), vs. ii) The Vasculitis Clinical Research Consortium (VCRC) observational studies, a physician-entered database (2003-2019) of patients who fulfilled the 1990 American College of Rheumatology classification criteria for EGPA. Studied parameters included demographics, clinical manifestations, ANCA status, treatments, and relapses. RESULTS Data from 195 patients with a validated diagnosis of EGPA in the VPPRN and 354 patients enrolled in the VCRC were analyzed. Compared to the VCRC cohort, the patients in the VPPRN cohort were more commonly female (69.2% vs. 59.0% in the VCRC cohort; P =.02), younger at diagnosis (47.3 vs. 50.0 years; P =.03), reported similar frequencies of asthma (96.2% vs 92.9% in VCRC; P =.13), cardiac manifestations (28.8% vs 21.2%; P =.06), but less frequent lung manifestations other than asthma, and more frequent disease manifestations in all other organ systems. ANCA positivity was 48.9% in the VPPRN patients vs. 38.9% (P=.05) in the VCRC cohort. Relapsing disease after study enrollment was reported in 32.3% patients in the VPPRN compared 35.7% of patients in the VCRC. Most therapies (glucocorticoids, cyclophosphamide, mepolizumab) were used at similar frequencies in both groups, except for rituximab with VPPRN patients reporting more use than VCRC cohort (24.1% vs. 10.5%; P =<.001). CONCLUSIONS Patients with EGPA generally report having more manifestations of disease than physicians report for patients with EGPA. These differences imply the need to reconsider how patient- and physician-reported data are collected for the study of EGPA, and reevaluate disease specific definitions. CLINICALTRIAL ClinicalTrials.gov: (1) VCRC Longitudinal Study (LS) NCT00315380 https://clinicaltrials.gov/ct2/show/NCT00315380 and (2) One-Time DNA (OT) study NCT01241305 https://clinicaltrials.gov/ct2/show/NCT01241305

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