scholarly journals A rare variant of ANCA-associated eosinophilic granulomatosis with polyangiitis complicated by diffuse alveolar hemorrage

2019 ◽  
Vol 29 (2) ◽  
pp. 229-234
Author(s):  
Anna S. Zaytseva ◽  
Igor' E. Stepanyan ◽  
Irina Yu. Shabalina ◽  
Lyudmila I. Dmitriyeva ◽  
Evgeniy I. Shmelev
Keyword(s):  
Clinical Case ◽  
Granulomatosis With Polyangiitis ◽  
Cardiac Injury ◽  
Diffuse Alveolar Hemorrhage ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Elderly Female ◽  
Anca Associated Vasculitis ◽  
Eosinophilic Granulomatosis ◽  
Limited Usefulness

Diagnostic criteria of vasculitis associated with antineutrophil cytoplasmic antibodies (ANCA) are of limited usefulness as they involve resembling conditions and do not make possible distinguishing nosology of vasculitis. A challenging clinical case of an elderly female patient with ANCA-associated vasculitis, diffuse alveolar hemorrhage syndrome, cardiac injury and blood eosinophilia, but without bronchial asthma, is described in the article.

scholarly journals Eosinophilic Cholecystitis Associated with Eosinophilic Granulomatosis with Polyangiitis

2020 ◽  
Vol 14 (3) ◽  
pp. 668-674
Author(s):  
Hiroyuki Ito ◽  
Yusuke Mishima ◽  
Tsubomi Cho ◽  
Naoki Ogiwara ◽  
Yoshimasa Shinma ◽  
...  
Keyword(s):  
Cerebral Hemorrhage ◽  
Granulomatosis With Polyangiitis ◽  
Epigastric Pain ◽  
White Blood Cells ◽  
Gallbladder Wall ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Test Results ◽  
Laboratory Investigations ◽  
Eosinophilic Granulomatosis

We report a case of eosinophilic cholecystitis associated with eosinophilic granulomatosis with polyangiitis (EGPA) complicated by cerebral hemorrhage. A 60-year-old man presented to a local hospital with a diagnosis of acute cholecystitis, with persistent fever and epigastric pain for 2 weeks. His symptoms persisted despite 3-week hospitalization; therefore, he was transferred to our hospital for further evaluation. Laboratory investigations upon admission showed white blood cells 26,300/µL and significant eosinophilia (eosinophils 61%). Abdominal computed tomography revealed no gallbladder enlargement but a circumferentially edematous gallbladder wall. Additional blood test results were negative for antineutrophil cytoplasmic and perinuclear antineutrophil cytoplasmic antibodies; however, immunoglobulin (Ig)G and IgE levels were high at 1,953 mg/dL and 3,040/IU/mL, respectively. He improved following endoscopic transnasal gallbladder drainage for cholecystitis and was diagnosed with EGPA and received corticosteroid and immunosuppressant combination therapy. The eosinophil count decreased immediately after treatment, and abdominal pain and numbness resolved. He returned with left-sided suboccipital hemorrhage likely attributed to EGPA 6 months after discharge. EGPA is characterized by inflammation of small blood vessels and clinically manifests with an allergic presentation of bronchial asthma, as well as renal dysfunction, interstitial pneumonia, enteritis, and cerebral hemorrhage. Few reports have described cholecystitis as a presenting symptom of EGPA. We report a rare case of such a presentation with added considerations.

scholarly journals Efficacy and safety of rituximab in the treatment of eosinophilic granulomatosis with polyangiitis

RMD Open ◽  
2019 ◽  
Vol 5 (1) ◽  
pp. e000905 ◽  
Author(s):  
Vítor Teixeira ◽  
Aladdin J Mohammad ◽  
Rachel B Jones ◽  
Rona Smith ◽  
David Jayne
Keyword(s):  
Partial Response ◽  
Granulomatosis With Polyangiitis ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Free Survival ◽  
Prednisolone Dose ◽  
Tertiary Centre ◽  
Limited Experience ◽  
Treatment Responses ◽  
Eosinophilic Granulomatosis

IntroductionEosinophilic granulomatosis with polyangiitis (EGPA) is a subset of antineutrophil cytoplasmic antibodies (ANCA) associated vasculitis with distinct pathophysiological mechanisms, clinical features and treatment responses. Rituximab is a licensed therapy for granulomatosis with polyangiitis and microscopic polyangiitis but there is limited experience of rituximab in EGPA.MethodsEGPA patients from a tertiary centre who received rituximab for mostly refractory EGPA or in whom cyclophosphamide was contra indicated were studied. A standardised dataset was collected at time of initial treatment and every 3 months for 24 months. Response was defined as a Birmingham Vasculitis Activity Score (BVAS) of 0 and partial response as ≥50% reduction in BVAS from baseline. Remission was defined as a BVAS of 0 on prednisolone dose ≤5 mg.ResultsSixty-nine patients (44 female) received rituximab between 2003 and 2017. Improvement (response and partial response) was observed in 76.8% of patients at 6 months, 82.8% at 12 months and in 93.2% by 24 months, while relapses occurred in 54% by 24 months, with asthma being the most frequent manifestation. The median BVAS decreased from 6 at baseline to 1 at 6 months, and 0 at 12 and 24 months. Prednisolone dose (mg/day, median) decreased from 12.5 to 7, 7.5 and 5 at 6, 12 and 24 months, respectively. ANCA positive patients had a longer asthma/ear, nose and throat (ENT) relapse-free survival time and a shorter time to remission.DiscussionRituximab demonstrated some efficacy in EGPA and led to a reduction in prednisolone requirement, but asthma and ENT relapse rates were high despite continued treatment. The ANCA positive subset appeared to have a more sustained response on isolated asthma/ENT exacerbations.

scholarly journals Eosinophilic granulomatosis with polyangiitis in a Nigerian woman

2019 ◽  
Vol 12 (6) ◽  
pp. e228901
Author(s):  
Ngozi Lina Ekeigwe ◽  
Olufemi Adelowo ◽  
Ehiaghe Lonia Anaba ◽  
Hakeem Olaosebikan
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Rare Condition ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Systemic Autoimmune Disease ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
American College Of Rheumatology ◽  
Nigerian Woman ◽  
History Of ◽  
Perinuclear Anca ◽  
Eosinophilic Granulomatosis

Eosinophilic granulomatosis with polyangiitis (EGPA), previously called Churg-Strauss Syndrome, is a systemic autoimmune disease that is usually associated with asthma and eosinophilia. It is a rare condition associated with antineutrophil cytoplasmic antibodies (ANCA). We report a case of a 52-year-old Nigerian woman who presented with bilateral leg swelling with multiple ulcers, background history of allergic rhinitis and chronic sinusitis since adolescence. There were also associated symptoms of peripheral neuropathy, weight loss, peripheral blood eosinophilia and positive perinuclear ANCA and she was previously managed for rheumatoid arthritis and asthma. She fulfilled the American College of Rheumatology criteria for EGPA and was treated with glucocorticoids and methotrexate which led to significant improvement of her symptoms.

scholarly journals Non-infectious aortic and mitral valve vegetations in a patient with eosinophilic granulomatosis with polyangiitis

2019 ◽  
Vol 12 (5) ◽  
pp. e225947 ◽  
Author(s):  
Kamalesh Karthikeyan ◽  
Sudarshan Balla ◽  
Martin A Alpert
Keyword(s):  
Mitral Valve ◽  
Granulomatosis With Polyangiitis ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Cardiac Complications ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Rare Form ◽  
Coronary Arteritis ◽  
Endocardial Fibrosis ◽  
Arteries And Veins ◽  
Eosinophilic Granulomatosis

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of vasculitis characterised by atopic manifestations, inflammation of small-sized to medium-sized arteries and veins, hypereosinophilia and tissue infiltration with eosinophils. Cardiac complications occur most commonly in the absence of antineutrophil cytoplasmic antibodies. Cardiac complications include coronary arteritis (rare), pericarditis, myocarditis, endocardial fibrosis (Loeffler’s endocarditis) and intracavitary thrombosis of the left or right ventricle. This is the first reported case of large non-infectious valvular vegetations associated with EGPA.

scholarly journals Immunopathogenesis of ANCA-Associated Vasculitis

2020 ◽  
Vol 21 (19) ◽  
pp. 7319
Author(s):  
Andreas Kronbichler ◽  
Keum Hwa Lee ◽  
Sara Denicolo ◽  
Daeun Choi ◽  
Hyojeong Lee ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Microscopic Polyangiitis ◽  
Autoimmune Disorder ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Proteinase 3 ◽  
Disease Biomarkers ◽  
Disease Phenotypes ◽  
Anca Associated Vasculitis ◽  
Clinical Presentations ◽  
Eosinophilic Granulomatosis

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is an autoimmune disorder which affects small- and, to a lesser degree, medium-sized vessels. ANCA-associated vasculitis encompasses three disease phenotypes: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). This classification is largely based on clinical presentations and has several limitations. Recent research provided evidence that genetic background, risk of relapse, prognosis, and co-morbidities are more closely related to the ANCA serotype, proteinase 3 (PR3)-ANCA and myeloperoxidase (MPO)-ANCA, compared to the disease phenotypes GPA or MPA. This finding has been extended to the investigation of biomarkers predicting disease activity, which again more closely relate to the ANCA serotype. Discoveries related to the immunopathogenesis translated into clinical practice as targeted therapies are on the rise. This review will summarize the current understanding of the immunopathogenesis of ANCA-associated vasculitis and the interplay between ANCA serotype and proposed disease biomarkers and illustrate how the extending knowledge of the immunopathogenesis will likely translate into development of a personalized medicine approach in the management of ANCA-associated vasculitis.

scholarly journals ANCA-associated vasculitis in a 14 years-old patient: a clinical case

2020 ◽  
Vol 27 (5) ◽  
pp. 184-194
Author(s):  
A. V. Burlutskaya ◽  
N. V. Savelyeva ◽  
N. S. Тaran
Keyword(s):  
Respiratory Failure ◽  
Immunosuppressive Therapy ◽  
Clinical Case ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Pulse Therapy ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Anca Associated Vasculitis ◽  
Nasal Bleeding ◽  
Diagnostic Research

Background. ANCA-associated systemic vasculitis is a rare childhood disease. Antineutrophil cytoplasmic autoantibodies (ANCA)-related vasculitises include microscopic polyangiitis, granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis. Their rarity often leads to a late diagnosis, rapid disability and high mortality in patients due to aggressive respiratory, pulmonary lesion and renal failure.Clinical Case Description. The patient suffered from a recurrent bronchoobstructive syndrome with signs of respiratory failure, obscure origin fever and chronic rhinitis with nasal bleeding for 6 months. The patient was diagnosed with obstructive bronchitis (putative bronchial asthma debut), received antibacterial therapy and inhalation bronchodilators without stable improvement during the entire period. Skin haemorrhages and arthralgia stimulated diagnostic research to establish ANCA-associated systemic vasculitis (presence of proteinase 3-specifi c ANCAs in titre 1/80). CT lung scanning revealed frosted glass foci of reduced pulmonary pneumatisation and signs of bilateral bronchoobstruction. Immunosuppressive therapy with glucocorticosteroids (methylprednisolone pulse therapy No. 3, 1000 mg intravenously on alternate days, subsequent per os administration of 1 mg/kg/day) and cyclophosphamide (500 mg intravenously once per 28 days) was prescribed. This led to the positive dynamics with eliminated fever and skin haemorrhages, as well as essentially reduced signs of respiratory failure.Conclusion. Diagnosis of systemic vasculitis is often complicated and long-term due to commonly non-specifi c debut symptoms of autoimmune disorders. In the described case, the fi rst 6 months of illness displayed intoxication and bronchoobstruction with signs of respiratory failure. Haemorrhagic rashes, arthralgias and the presence of ANCAs are proxy to vasculitis. Standard immunosuppressive therapy for ANCA-associated vasculitis improved the patient’s condition.

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