scholarly journals Hydralazine-associated antineutrophil cytoplasmic antibody vasculitis with pulmonary–renal syndrome

2018 ◽  
pp. bcr-2018-227161 ◽  
Author(s):  
Narothama Reddy Aeddula ◽  
Samata Pathireddy ◽  
Asif Ansari ◽  
Peter J Juran
Keyword(s):  
Clinical Presentation ◽  
Severe Hypertension ◽  
Systemic Vasculitis ◽  
Rare Complication ◽  
Rapidly Progressive Glomerulonephritis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Pulmonary Haemorrhage ◽  
Adjunctive Treatment ◽  
Pulmonary Renal Syndrome ◽  
High Degree

Hydralazine, a vasodilator, is commonly used as an adjunctive treatment for moderate to severe hypertension, heart failure and hypertensive emergencies in pregnancy. Hydralazine-induced lupus was first described in 1953. Clinical presentation ranges from arthralgia, myalgia, petechiae, or rash to single or multiorgan involvement. An occurrence of systemic vasculitis is a rare complication. When presented as the pulmonary–renal syndrome, it could have a rapidly progressive course which can be fatal. Here, we describe a case of hydralazine-associated rapidly progressive glomerulonephritis and pulmonary haemorrhage. We use this case to review the current literature and discuss and highlight the importance of a high degree of clinical acumen, early diagnosis and prompt treatment for better clinical outcomes.

scholarly journals Benzylthiouracil-Induced Glomerulonephritis

2009 ◽  
Vol 2009 ◽  
pp. 1-4 ◽  
Author(s):  
Sihem Trimeche Ajmi ◽  
Rim Braham ◽  
Sarra Toumi ◽  
Molka Chadli Chaieb ◽  
Amel Maaroufi ◽  
...  
Keyword(s):  
Renal Failure ◽  
Drug Withdrawal ◽  
Rare Complication ◽  
Immunosuppressive Treatment ◽  
Laboratory Data ◽  
Rapidly Progressive Glomerulonephritis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Antithyroid Drugs ◽  
Normal Range ◽  
Respiratory Involvement

Vasculitis is a rare complication of antithyroid drugs (ATDs). It was first described with Propylthiouracil (PTU). We report a new case of antineutrophil cytoplasmic antibody (ANCA) vasculitis with glomerulonephritis induced by Benzylthiouracile (BTU). A 50-year-old man with Graves disease treated with BTU developed general malaise and haematuria without skin rash or respiratory involvement. Laboratory data revealed acute renal failure with proteinuria and haematuria. An indirect immunofluorescence test for ANCA was positive, showing a perinuclear pattern with specificity antimyeloperoxidase (MPO). A renal biopsy was performed and revealed pauci-immune extracapillary glomerular nephropathy and necrotic vasculitis lesions. Based on these findings we concluded to the diagnosis of rapidly progressive glomerulonephritis associated with ANCA induced by BTU therapy. The drug was therefore discontinued and the patient was treated with steroids and immunosuppressive treatment during 3 months. Renal failure, proteinuria and haematuria significantly improved within 2 months. However, P-ANCA remained positive until 10 months after drug withdrawal. Thyroid function was kept within normal range using iodine solution. We demonstrated clearly that BTU may induce severe forms of vasculitis with glomerulonephritis. Thus, the ANCA must be measured when confronted to systemic manifestation during treatment.

scholarly journals Significance of Small Renal Artery Lesions in Patients with Antineutrophil Cytoplasmic Antibody-associated Glomerulonephritis

2014 ◽  
Vol 41 (6) ◽  
pp. 1140-1146 ◽  
Author(s):  
Akiko Endo ◽  
Junichi Hoshino ◽  
Tatsuya Suwabe ◽  
Keiichi Sumida ◽  
Koki Mise ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Rapidly Progressive Glomerulonephritis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Pulmonary Involvement ◽  
Renal Arteries ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Necrotizing Glomerulonephritis ◽  
Group A ◽  
Group B

Objective.Antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis is a vasculitis affecting the glomerular capillaries and small renal arteries. Although crescent formation has been reported to be characteristic of this condition, the significance of coexisting vasculitis affecting the small renal arteries has not been investigated.Methods.Fifty patients with ANCA-positive rapidly progressive glomerulonephritis whose renal biopsy specimens contained arterioles and/or interlobular arteries were retrospectively evaluated. Cellular crescents and/or necrotizing glomerulonephritis were noted in all 50 patients. Ten patients had vasculitis of the small renal arteries (group A) and 40 patients were without such vasculitis (group B). The clinical features of these 2 groups were compared.Results.Group A comprised 4 patients who had granulomatosis with polyangiitis (GPA) and 6 with microscopic polyangiitis (MPA), while group B included 1 patient with GPA and 39 with MPA. No patient in either group had eosinophilic granulomatosis with polyangiitis. The C-reactive protein (CRP) level was significantly higher in group A compared with group B (11.58 ± 6.19 vs 2.7 ± 3.55 mg/dl, p < 0.05), and pulmonary involvement was more frequent in group A than group B (80% vs 37.5%, p < 0.05).Conclusion.In patients with ANCA-positive glomerulonephritis, vasculitis of small renal arteries may be associated with systemic vasculitis (including pulmonary involvement) because of elevated CRP, a systemic inflammatory marker related to overproduction of interleukin 6.

scholarly journals Systematic Histological Scoring Reveals More Prominent Interstitial Inflammation in Myeloperoxidase-ANCA Compared to Proteinase 3-ANCA Glomerulonephritis

2021 ◽  
Vol 10 (6) ◽  
pp. 1231
Author(s):  
Samy Hakroush ◽  
Ingmar Alexander Kluge ◽  
Philipp Ströbel ◽  
Peter Korsten ◽  
Désirée Tampe ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Clinical Presentation ◽  
Systemic Vasculitis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Laboratory Findings ◽  
Treatment Regimens ◽  
Anca Associated Vasculitis ◽  
Kidney Involvement ◽  
Tubulointerstitial Lesions ◽  
Severe Deterioration

Background: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a systemic vasculitis, most frequently presenting as microscopic polyangiitis (MPA) or granulomatosis with polyangiitis (GPA). Kidney involvement is a common and severe complication of ANCA AAV which is observed in a considerable subset of patients, mainly affecting glomeruli. However, tubulointerstitial lesions have also been described in ANCA glomerulonephritis (GN). Therefore, we aim to describe active and chronic tubulointerstitial lesions in ANCA GN subtypes by systematic scoring analogous to the Banff scoring system while also utilizing clinical and laboratory findings. Methods: A total of 49 kidney biopsies with ANCA GN were retrospectively included in a single-center cohort study between 2015–2020. Results: We report that MPO-ANCA GN is associated with more severe deterioration of kidney function independent of systemic markers of AAV disease activity, and is also associated with increased proteinuria in MPO-ANCA GN and a decreased fraction of normal glomeruli. Finally, MPO-ANCA GN showed distinct, active, and chronic tubulointerstitial lesions. Conclusion: New insights into the pathophysiology of both entities, as well as differences in the clinical presentation of MPO- versus PR3-ANCA GN, could potentially pave the way for more precise treatment regimens. Therefore, it is important to understand the differences in histopathological presentation, especially in yet underestimated active tubulointerstitial lesions of ANCA GN subtypes. This research could further improve our understanding of distinct pathophysiological mechanisms.

scholarly journals Hydralazine Associated Antineutrophil Cytoplasmic AntibodyAssociated Vasculitis with Glomerulonephritis and Dsdna Antibody

2020 ◽  
pp. 1-3
Author(s):  
Venu Chippa ◽  
◽  
Swetha Chenna ◽  
Keyword(s):  
Blood Pressure ◽  
Early Diagnosis ◽  
Clinical Outcomes ◽  
Rare Complication ◽  
Treatment Options ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Blood Pressure Medication ◽  
Tubular Injury ◽  
Dsdna Antibody ◽  
Pulmonary Renal Syndrome

Hydralazine is a very commonly used blood pressure medication. Though safe it can be associated with a lupus-like syndrome and antineutrophil cytoplasmic antibody-associated vasculitis due to its immunogenic nature, and rarely pulmonary-renal syndrome which can be fatal if not recognized promptly. Here we describe a case of hydralazine induced vasculitis with glomerulonephritis with positive dsDNA antibody, but the biopsy showed tubular injury with RBC casts and glomerulonephritis. We use this case to review the current literature and discuss the importance of early diagnosis, treatment options, and clinical outcomes of this rare complication from hydralazine use.

scholarly journals Bartonella endocarditis masquerading as systemic vasculitis with rapidly progressive glomerulonephritis (aka ‘Löhlein nephritis’)

2019 ◽  
Vol 12 (12) ◽  
pp. e231413 ◽  
Author(s):  
Lian Bannon ◽  
Guy Choshen ◽  
Michael Giladi ◽  
Jacob Ablin
Keyword(s):  
Case Report ◽  
Serological Test ◽  
Bartonella Henselae ◽  
Systemic Vasculitis ◽  
Immunosuppressive Treatment ◽  
Central Retinal Artery Occlusion ◽  
Rapidly Progressive Glomerulonephritis ◽  
Retinal Artery Occlusion ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Artery Occlusion

Bartonella species are fastidious, Gram-negative aerobic rods and a well-recognised pathogen responsible for culture-negative endocarditis. The histopathological appearance of glomerulonephritis (GN) caused by Bartonella endocarditis may include a pauci-immune GN similar to that usually seen in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Herein, we present an unusual case report of Bartonella endocarditis masquerading as ANCA-positive vasculitis, with crescentic GN. A 66-year-old woman, who had undergone aortic valve replacement 2 years prior to admission, presented with confusion and loss of vision in her right nasal field. Following an extensive diagnostic evaluation, the main findings were right central retinal artery occlusion, ground-glass appearance on chest CT and ANCA-positive, anti PR-3 negative, rapidly progressive GN. The patient was scheduled to start treatment with rituximab for presumed ANCA-positive GN, when a positive serological test for Bartonella henselae was received. In view of this result, a diagnosis of endocarditis was made, based on fulfilment of five Duke minor criteria, namely fever, predisposition, arterial emboli, immunological phenomena and serological evidence of active infection with an organism consistent with infective endocarditis. Immunosuppressive treatment was withheld and antibiotic treatment initiated. This case report emphasises the need for maintaining a high index of suspicion regarding the diagnosis of Bartonella infection, which might mimic ANCA-associated GN.

scholarly journals Microscopic Polyangitis in Pediatric Systemic Lupus Erythematosus: A Unique Presentation of Pulmonary - Renal Syndrome and Case Report of an Overlap Syndrome

2020 ◽  
Author(s):  
Chen-xing Zhang ◽  
Lei Yin ◽  
You-ying Mao ◽  
Zheng-yu Zhou ◽  
He Qin ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Systemic Vasculitis ◽  
Rapidly Progressive Glomerulonephritis ◽  
Overlap Syndrome ◽  
Systemic Lupus ◽  
Pediatric Systemic Lupus Erythematosus ◽  
Pulmonary Renal Syndrome ◽  
Pulmonary Alveolar Hemorrhage ◽  
Lupus Glomerulonephritis

Abstract Background: Secondary vasculitis is encountered in about one third of all cases of systemic lupus erythematosus (SLE). Skin is most commonly involved in lupus-related small vasculitis. Although antineutrophil cytoplasmic autoantibodies (ANCA) associated vasculitis (AAV) is relatively uncommon, it can be the most dangerous manifestation associated with high mortality. SLE and AAV are separate diseases with different pathophysiologies and an overlap syndrome has only been reported few times in previous literature. Case Presentation: We present a unique case of a pediatric patient of pulmonary-renal syndrome, presenting with pulmonary alveolar hemorrhage and rapidly progressive glomerulonephritis. Serological and biopsy findings were suggestive of SLE and AAV occurring simultaneously. Renal biopsy demonstrated necrotizing and crescentic glomerulonephritis, superimposed on diffuse segmental proliferative lupus glomerulonephritis class IV. Conclusions: Presentations of autoimmune diseases and vasculitis can be multi-systemic. Considering overlap syndromes, especially in patients with underlying connective tissue disease or systemic vasculitis, is vital for prompt therapy and prevention of morbidity in this population.

scholarly journals Patient with Antineutrophil Cytoplasmic Antibody Associated Small Vessel Vasculitis, Acute Renal Failure, and Coronavirus Disease-19 Pneumonia: A Diagnostic and Therapeutic Challenge

2020 ◽  
Vol 8 (T1) ◽  
pp. 542-547
Author(s):  
Biljana Gerasimovska-Kitanovska ◽  
Gjulshen Selim ◽  
Zvezdana Petronijevik ◽  
Blagica Pecanova ◽  
Gjorgi Stojchev ◽  
...  
Keyword(s):  
Renal Failure ◽  
Acute Renal Failure ◽  
Systemic Vasculitis ◽  
Renal Involvement ◽  
Rapidly Progressive Glomerulonephritis ◽  
Pulmonary Hemorrhage ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Necrotizing Glomerulonephritis ◽  
Pulmonary Renal Syndrome

BACKGROUND: Antineutrophil cytoplasmatic antibody (ANCA)-associated vasculitis (AAV) has a predilection for the kidney and more than three quarters of patients have renal involvement with rapidly progressive glomerulonephritis. Small-vessel systemic vasculitis may present as pulmonary-renal syndrome and is characterized by necrotizing glomerulonephritis and pulmonary hemorrhage. Diagnosis and therapy for AAV in coronavirus disease (COVID) COVID-19 pandemic require multi-disciplinary collaboration due to the affection of multiple systems and risks associated with immunosuppressive medications. CASE REPORT: A 69-year-old non-smoker, non-diabetic female presented in the outpatient unit at the department of pulmonology with dry cough, malaise, and sub-febrile temperature, lasting for 1 month. The patient had a high suspicion of severe pulmonary-renal syndrome, ANCA-AAV, and acute renal failure requiring hemodialysis. She was treated with corticosteroids, cyclophosphamide, and plasma exchange. The treatment led to temporary improvement. Infections with COVID-19, Enterococcus in the urine, and Acinetobacter in the tracheal aspirate further complicated the clinical picture and despite antibiotic treatment, use of tocilizumab and convalescent plasma, the outcome was lethal. CONCLUSION: It is important to establish the diagnosis and distinguish accurately between vasculitis and infection to provide adequate and timely therapy.

scholarly journals SARS-CoV-2 infection and recurrence of anti-glomerular basement disease: a case report

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Alexander Winkler ◽  
Emanuel Zitt ◽  
Hannelore Sprenger-Mähr ◽  
Afschin Soleiman ◽  
Manfred Cejna ◽  
...  
Keyword(s):  
Basement Membrane ◽  
Glomerular Basement Membrane ◽  
Plasma Cells ◽  
Rapidly Progressive Glomerulonephritis ◽  
Kidney Injury ◽  
Pulmonary Involvement ◽  
Pulmonary Haemorrhage ◽  
Basement Membranes ◽  
High Avidity ◽  
History Of

Abstract Background Anti-glomerular basement membrane disease (GBM) disease is a rare autoimmune disease causing rapidly progressive glomerulonephritis and pulmonary haemorrhage. Recently, an association between COVID-19 and anti-glomerular basement membrane (anti-GBM) disease has been proposed. We report on a patient with recurrence of anti-GBM disease after SARS-CoV-2 infection. Case presentation The 31-year-old woman had a past medical history of anti-GBM disease, first diagnosed 11 years ago, and a first relapse 5 years ago. She was admitted with severe dyspnoea, haemoptysis, pulmonary infiltrates and acute on chronic kidney injury. A SARS-CoV-2 PCR was positive with a high cycle threshold. Anti-GBM autoantibodies were undetectable. A kidney biopsy revealed necrotising crescentic glomerulonephritis with linear deposits of IgG, IgM and C3 along the glomerular basement membrane, confirming a recurrence of anti-GBM disease. She was treated with steroids, plasma exchange and two doses of rituximab. Pulmonary disease resolved, but the patient remained dialysis-dependent. We propose that pulmonary involvement of COVID-19 caused exposure of alveolar basement membranes leading to the production of high avidity autoantibodies by long-lived plasma cells, resulting in severe pulmonary renal syndrome. Conclusion Our case supports the assumption of a possible association between COVID-19 and anti-GBM disease.

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 ANCA-Associated Glomerulonephritis and Anti-Phospholipid Syndrome in a Patient with SARS-CoV-2 Infection: Just a Coincidence?

2021 ◽  
pp. 214-220
Author(s):  
Federica Maritati ◽  
Maria Ilaria Moretti ◽  
Valentina Nastasi ◽  
Roberta Mazzucchelli ◽  
Manrico Morroni ◽  
...  
Keyword(s):  
Rapidly Progressive Glomerulonephritis ◽  
Kidney Injury ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Hemodynamic Instability ◽  
Tubular Necrosis ◽  
Complex Processes ◽  
Anca Associated Vasculitis ◽  
High Incidence ◽  
New Diagnosis ◽  
The Relationship

Many reports have described a high incidence of acute kidney injury (AKI) among patients with COVID-19. Acute tubular necrosis has been reported to be the most common damage in these patients, probably due to hemodynamic instability. However, other complex processes may be involved, related to the cytokine storm and the activation of innate and adaptive immunity. Here, we describe a patient who developed an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis with rapidly progressive glomerulonephritis and lung involvement and an antiphospholipid syndrome soon after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. After viral pneumonia was excluded by bronchoalveolar lavage, the patient has been treated with rituximab for amelioration of kidney function and resolution of thrombosis without any adverse event. We conclude that COVID-19 may trigger autoimmune diseases including ANCA-associated vasculitis. Thus, this diagnosis should be taken in consideration in COVID-19 patients, especially when they develop AKI with active urinary sediment. In addition, considering the relationship between these 2 diseases, SARS-CoV-2 infection should be excluded in all patients with a new diagnosis ANCA-associated vasculitis before starting immunosuppressive therapy.

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