scholarly journals AB0768 NEUROLOGICAL MANIFESTATIONS IN GRANULOMATOSIS WITH POLYANGIITIS: ABOUT 11 CASES

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1410.2-1411
Author(s):  
O. Jomaa ◽  
O. Berriche ◽  
S. Arfa ◽  
A. Fraj ◽  
M. Belkhiri ◽  
...  
Keyword(s):  
Retrospective Study ◽  
Peroneal Nerve ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Cranial Nerves ◽  
Neurological Impairment ◽  
Neurological Manifestations ◽  
Study Results ◽  
Cerebral Mri ◽  
Pyramidal Syndrome

Background:Granulomatosis with polyangiitis (GPA) is a rare systemic vasculitis, affecting mainly the superior airways, lungs and kidneys. Neurological impairment is frequent, described in 22 to 54% of cases, and rarely inaugurates the disease.Objectives:The objective of our work was to evaluate its prevalence and its different aspects.Methods:A retrospective study including 11 files of GPA patients followed over a period of 10 years in The Internal Medecine Department at Tahar Sfar Hospital Mahdia TUNISIA.Results:Neurological manifestations were reported in 5 patients: a pyramidal syndrome was noted in 3 patients with a normal cerebral MRI, a disabled headache was described in one patient with a normal cerebral MRI. Peripheral neuropathy in 2 cases, and the Electroneuromyogram objectified a mononeuropathy affecting the ulnar nerve, peroneal nerve, and musculocutaneous nerve in one patient and a distal sensitivomotor poly neuropathy in one patient. Cranial nerves damage was noted in two cases, one VII (1 case) and one V (1 case).Conclusion:Our study results, show the importance of timely diagnosis of these neurological manifestations, as their evolution will depend on the early GPA treatment.References:[1]doi: 10.1016/j.autrev.2017.07.007Disclosure of Interests:None declared

Morbidity and mortality associated with subglottic laryngotracheal stenosis in granulomatosis with polyangiitis (Wegener's granulomatosis): a single-centre experience in the United Kingdom

2014 ◽  
Vol 128 (9) ◽  
pp. 831-837 ◽  
Author(s):  
N P Jordan ◽  
H Verma ◽  
A Siddiqui ◽  
G A Morrison ◽  
D P D'Cruz
Keyword(s):  
United Kingdom ◽  
Wegener’S Granulomatosis ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Wegener's Granulomatosis ◽  
Laryngotracheal Stenosis ◽  
Surgical Interventions ◽  
The United Kingdom ◽  
Single Centre Experience

AbstractObjectives:We aimed to determine the prevalence of symptomatic subglottic laryngotracheal stenosis in patients with granulomatosis with polyangiitis (Wegener's granulomatosis); we also wanted to characterise the clinical outcomes and surgical interventions required, and the relapse rate in our cohort.Methods:We undertook a retrospective clinical review of all granulomatosis with polyangiitis patients with symptomatic subglottic laryngotracheal stenosis attending St Thomas' Hospital, London, United Kingdom.Results:Symptomatic subglottic laryngotracheal stenosis developed in 16 per cent of granulomatosis with polyangiitis patients attending our clinic. The median age of patients at diagnosis was 44 years (range: 34–81 years); 78 per cent of those presenting with subglottic laryngotracheal stenosis were women and 22 per cent were men. All patients were white; 67 per cent of patients were proteinase 3-antineutrophil cytoplasmic antibody-positive and 67 per cent developed relapsing disease requiring repeated surgical intervention. Subglottic laryngotracheal stenosis relapse was not associated with active systemic vasculitis elsewhere.Conclusion:Subglottic laryngotracheal stenosis is an uncommon but significant complication of granulomatosis with polyangiitis. Management of subglottic laryngotracheal stenosis requires a multi-disciplinary approach, with both rheumatological and otolaryngological expertise involved, given the relapsing nature of the disease.

scholarly journals THU0300 RISK FACTORS FOR COMPLICATIONS AND REFRACTORY COURSE IN PATIENTS WITH ANCA-ASSOCIATED SYSTEMIC VASCULITIS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 378.2-378
Author(s):  
A. Chudinov ◽  
I. Belyaeva ◽  
M. Pervakova ◽  
V. Mazurov ◽  
O. Inamova ◽  
...  
Keyword(s):  
Risk Factors ◽  
Chronic Kidney Disease ◽  
Kidney Disease ◽  
Induction Therapy ◽  
Granulomatosis With Polyangiitis ◽  
Disease Risk ◽  
Systemic Vasculitis ◽  
Cardiovascular Complications ◽  
Infectious Complications ◽  
Eosinophilic Granulomatosis With Polyangiitis

Background:ANCA-associated systemic vasculitis (AAV) is characterized by a high incidence of complications and high mortality. The most significant complications during the first 3 years of the disease are infectious and cardiovascular. Development of chronic kidney disease also impairs the prognosis of AAV. Refractory to induction therapy can significantly increase the severity of organ lesions in patients with AAV.Objectives:The aim of this study was to determine risk factors for complications and refractory course in patients with AAV.Methods:Patients with granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA) were observed during the first 3 years of the disease and included in this study between 2010 and 2018. Most common infectious complications requiring inpatient treatment were pneumonia, mycosis, sepsis, purulent arthritis, purulent otitis media. Cardiovascular complications included pulmonary thromboembolism, myocardial infarction, ischemic stroke, venous thrombosis.Results:In total 209 (165 [79%] female and mean age 51.8 ± 13.2 years) AAV patients (94 GPA; 46 MPA; and 69 EGPA) were included in the analysis. Risk factors for infectious complications were BVAS level at the beginning of induction therapy > 25 (OR – 2.92, 95% CI (1.53;5.45) p<0.001), usage of prednisone in doses more than 60 mg / day at the induction of remission (OR – 2.76, 95% CI (1.45;5.29) p=0.003), usage of prednisone in doses ≥ 10 mg / day after 6 months of induction therapy (OR – 2.60, 95% CI (1.38;4.93) p=0.003), ANCA-PR3 positivity (OR – 2.25, 95% CI (1.13;4.46) p=0.017) and presence of diabetes mellitus in the AAV onset (OR – 1.77, 95% CI (1.14;3.45) p=0.038). Patients with AAV had following risk factors for cardiovascular complications: male (OR – 2.28, 95% CI (1.33;3.88) p=0.002), BVAS level > 25 (OR – 2.1, 95% CI (1.11;3.16) p=0.008) and presence of coronary artery disease in the AAV onset (OR – 2.2, 95% CI (1.18;4.10) p=0.015). ANCA positivity (OR – 5.62, 95% CI (2.1;14.9) p<0.001), presence of rapidly progressive glomerulonephritis in the first 3 months from onset AAV (OR – 5.02, 95% CI (3.42;7.35) p<0.001) and over 60 years of age (OR – 2.17, 95% CI (1.38;3.44) p=0.001) were risk factors of development of chronic kidney disease. Risk factors for refractory to induction therapy in patients with AAV were ANCA-PR3 positivity (OR – 3.13, 95% CI (1.63;6.02) p<0.001), BVAS level > 25 (OR – 2.63, 95% CI (1.74;4.34) p<0.001), initiation of therapy after 4 months from the onset of clinical manifestations (OR – 2.17, 95% CI (1.26;3.91) p=0.005). We additionally defined that identification of pathological phenotypes of alpha-1-antitrypsin was risk factors for refractory course in patients with GPA manifestations (OR – 2.66, 95% CI (1.12;6.33) p=0.048).Conclusion:Our study has shown that high disease activity, ANCA positivity and comorbid pathology increase risk of serious complications. Early administration of immunosuppressive therapy, adequate steroid dosing and use of risk factors for complications and refractory course in clinical practice can significantly improve the prognosis of AAV.Disclosure of Interests:None declared

Systemic Vasculitis Syndromes

2017 ◽  
Author(s):  
Alexandra Villa-Forte ◽  
Brian F Mandell
Keyword(s):  
Kawasaki Disease ◽  
Blood Vessels ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Plain Radiograph ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Saddle Nose ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Saddle Nose Deformity

Vasculitis is defined by histologic evidence of inflammation that involves the blood vessels. The diagnosis of a specific primary vasculitic disorder depends on the pattern of organ involvement, the histopathology, the size of affected blood vessels, and the exclusion of diseases that can cause “secondary” vasculitis. This review presents an approach to the patient suspected of having vasculitis, and goes on to discuss small vessel vasculitis, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis, polyarteritis nodosa, Kawasaki disease, large vessel arteritis, and Behçet disease. Figures show classification of the systemic vasculitis syndromes, the relationships among the causes of small vessel (“hypersensitivity”) vasculitis, palpable purpura of the distal extremities, saddle nose deformity, the nodular infiltrates of the lung in granulomatosis with polyangiitis shown on plain radiograph as well as computed tomography, necrotizing scleritis, livedo reticularis, and angiograms of a patient with Takayasu arteritis. Tables list selected laboratory tests for patients with multisystem disease and possible vasculitis, practical comments on immunosuppressive therapies for vasculitis, features of vasculitis, diagnostic criteria for Kawasaki disease, and giant cell arteritis. This review contains 8 highly rendered figures, 5 tables, and 59 references.

scholarly journals EFFECT OF POSTOPERATIVE EPIDURAL ANALGESIA AFTER MAJOR ORTHOPEDIC SURGERIES, A RETROSPECTIVE STUDY

2016 ◽  
Vol 3 (2) ◽  
pp. 20-25
Author(s):  
Laxmi Pathak
Keyword(s):  
Retrospective Study ◽  
Postoperative Pain ◽  
Epidural Analgesia ◽  
Epidural Catheter ◽  
Medical Sciences ◽  
Study Results ◽  
Epidural Catheterization ◽  
American Society ◽  
Upper Abdominal ◽  
Postoperative Epidural Analgesia

INTRODUCTION: Epidural analgesia has been used in many major surgeries like upper abdominal surgery, cardio-thoracic surgery, orthopedic surgery etc. Epidural analgesia is one of the commonly used methods of postoperative pain control despite its associated complications. So, this study was conducted to find out its effectiveness in major orthopedic surgeries done in Universal College of Medical Sciences. MATERIAL AND METHODS: A retrospective study was done at Universal College of Medical Sciences & Teaching Hospital, Bhairahawa, Nepal from July 2012 to June 2014. Data of 57 patients, aged between 17 to 91 years having American Society of Anesthesiologists (ASA) physical status 1 and 2 who had undergone major orthopedic surgeries under spinal anesthesia and lumber epidural catheterization were collected and important information regarding anesthesia and surgery, epidural catheter and postoperative epidural analgesia, any complications if occurred throughout the study period were recorded. Departmental Protocol for epidural analgesia was followed in these patients. Patients who received intraoperative epidural anesthesia or analgesia and any other anesthetic or analgesic agents were excluded in this study. RESULTS: This study found epidural analgesia, a very effective way to relieve pain in patients undergoing major orthopedic surgeries, when given according to the Departmental Protocol. There were no any complications related to epidural analgesia till 4th postoperative day. Out of 57, only 2 patients received injection Ketorolac intravenously as a rescue analgesic at the same day of operation before epidural top up. Average time to demand for analgesic after the last dose of epidural top up was 21.933 hours. All patients were mobilized around their beds on 2nd postoperative day. The average postoperative days of hospital stay was only 6.5 days. CONCLUSION: Epidural mixture of Bupivacaine-morphine in lower dose and concentration given as an intermittent bolus dosing via lumber epidural catheter is safe and very effective in relieving postoperative pain after major orthopedic surgeries without any significant complications.Journal of Universal College of Medical Sciences (2015) Vol.03 No.02 Issue 10   

scholarly journals Look granulomatosis with polyangiitis (GPA) straight in the face: missed opportunities leading to a delayed diagnosis

2019 ◽  
Vol 10 (1) ◽  
Author(s):  
N. Rolle ◽  
M. Muruganandam ◽  
I. Jan ◽  
F. M. Harji ◽  
J. Harrington ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Delayed Diagnosis ◽  
Organ System ◽  
Atypical Presentation ◽  
Mural Thrombus ◽  
Missed Opportunities ◽  
Original Diagnosis ◽  
The Face ◽  
High Index Of Suspicion

Abstract Granulomatosis with polyangiitis (GPA) is a systemic vasculitis with a potential to involve any organ system. It remains an important cause of kidney related morbidity and mortality. Early diagnosis can be difficult and requires high index of suspicion in all patients, but especially in cases with atypical presentation. We report a case with GPA, which was diagnosed only after new and advancing symptoms belied the original diagnosis of bilateral facial palsy and aortic mural thrombus.

Clinical features of ANCA-associated vasculitis

2013 ◽  
pp. 1090-1102
Author(s):  
Wolfgang L. Gross ◽  
Julia U. Holle
Keyword(s):  
Respiratory Tract ◽  
Clinical Features ◽  
Lower Respiratory Tract ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Systemic Disease ◽  
Clinical Signs ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Granulomatous Lesions ◽  
Organ Manifestations

The primary ANCA-associated vasculitides are granulomatosis with polyangiitis (Wegener’s, GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome, CSS). They predominantly affect small (and medium-sized) vessels and share a variable association with ANCA (anti-neutrophil cytoplasm antibody) directed against neutrophil proteinase 3 (PR3, mainly in GPA) and myeloperoxidase (MPO, mainly in MPA and CSS). Crescentic necrotizing glomerulonephritis and alveolar haemorrhage due to pulmonary capillaritis represent classical (vasculitic) organ manifestations of the ANCA-associated vasculitides (AAV). MPA occurs as a ’pure’ small (to medium-size) vessel vasculitis, whereas GPA and CSS are characterized by additional distinct clinical and pathological features. In GPA, granulomatous lesions of the upper and/or lower respiratory tract are a hallmark of the disease. Granulomatous lesions may be large in appearance and occur as space-consuming, infiltrating, and destructive inflammatory masses. GPA is believed to follow a stagewise course with an initial localized form, restricted granulomatous lesions of the upper and/or lower respiratory tract without clinical signs of vasculitis, and a consecutive generalization to systemic vasculitis which may be either non-organ-threatening (early systemic) or organ- and life- threatening (generalized GPA). Rarely, patients arrest in the localized stage and do not progress to systemic disease. In EGPA asthma, hypereosinophilia and eosinophilic organ infiltration (e.g. eosinophilic myocarditis) are typical features of the disease apart from vasculitis. Similarly to GPA, EGPA follows a stagewise course: asthma and eosinophilia may precede full-blown disease for several months or years. Recent cohort studies suggest different phenotypes in EGPA (predominantly vasculitic and MPO-ANCA-positive and predominantly with eosinophilic organ infiltration, usually ANCA-negative). This chapter focuses on the clinical features of the primary AAV and their outcome.

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.

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