Inflammatory tumour in the course of microscopic polyangiitis may mimic testicular cancer

ntroduction Microscopic polyangiitis (MPA) is an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) that can affect any organ. Sometimes, untypical localization, together with an unusual clinical manifestation of tumour-resembling inflammatory changes, can cause a delay in diagnosis and proper treatment. Aim The aim of this study was to expose the case of very rare location of MPA manifestation. Case study In this paper, we present the case of inflammatory testicular tumour-like lesion that was a manifestation of MPA and mimicked testicular cancer. Results and discussion It is probably first desribed case of MPA mimicking testicular cancer. However, in the literature some others AAV manifestation in that location can be found. In these patients orchiectomy was recommended much more often than a testicular biopsy. Conclusions We conclude that in case of a patient with untypical features of a tumour, inflammatory processes, including vasculitis, should be taken into consideration in differential diagnosis.

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ANCA Associated vasculitis- A case of microscopic polyangiitis with proliferative glomerulonephritis

Biomedicine ◽

10.51248/.v41i3.1208 ◽

2021 ◽

Vol 41 (3) ◽

pp. 686-689

Author(s):

R.C. Akshaya ◽

R. Sathyanarayanan ◽

C.R.V. Narasimhalu ◽

Sonti Sulochana

Keyword(s):

Microscopic Polyangiitis ◽

Clinical Signs ◽

Antineutrophil Cytoplasmic Antibody ◽

Skin Lesions ◽

Proliferative Glomerulonephritis ◽

Diagnostic Approach ◽

Skin Manifestations ◽

Anca Associated Vasculitis

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) are a collection of diseases, characterised by destruction and inflammation of small and medium vessels. Microscopic Polyangiitis (MPA) is part of an ANCA-associated vasculitis (AAV).The clinical signs diverge and disturba number of organs such as the kidneys, lungs, stomach and intestine. Skin manifestations such as purpuric, urticarial, nodular, ulcerative, livedoid and necrotic skin lesions were common as in other vaso-occlusive disorder. Morphology and added features aid the diagnostic approach. Here, we report a diagnostically challenging case of microscopic polyangiitis with progressive glomerulonephritis.

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Efficacy and Safety of Rituximab Treatment in Patients with Antineutrophil Cytoplasmic Antibody-associated Vasculitides: Results from a German Registry (GRAID)

The Journal of Rheumatology ◽

10.3899/jrheum.120482 ◽

2012 ◽

Vol 39 (11) ◽

pp. 2153-2156 ◽

Cited By ~ 23

Author(s):

PETRA ROLL ◽

EVA OSTERMEIER ◽

MARION HAUBITZ ◽

SVJETLANA LOVRIC ◽

LEONORE UNGER ◽

...

Keyword(s):

Treatment Option ◽

Clinical Efficacy ◽

Granulomatosis With Polyangiitis ◽

Microscopic Polyangiitis ◽

Safety Data ◽

Antineutrophil Cytoplasmic Antibody ◽

National Registry ◽

Efficacy And Safety ◽

Anca Associated Vasculitis ◽

German Registry

Objective.Rituximab (RTX) therapy is a treatment option in patients with refractory antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). We investigated the tolerability and clinical efficacy of RTX in a cohort of patients with refractory AAV.Methods.Clinical and safety data of patients with AAV treated with RTX were retrospectively assessed from the data of a German national registry.Results.In total, 58 patients were included in this analysis (50/58 with granulomatosis with polyangiitis; 8/58 with microscopic polyangiitis who received at least 1 cycle, 17 patients who received 2 cycles, and 3 patients who received 3 cycles of RTX). Response was classified as complete and partial in 22 (40%) and in 29 cases (52.7%), respectively. Four patients (7.3%) were classified as nonresponders.Conclusion.RTX was well tolerated with good clinical efficacy in patients with refractory AAV.

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Spectrum and Prognosis of Antineutrophil Cytoplasmic Antibody–associated Vasculitis-related Bronchiectasis: Data from 61 Patients

The Journal of Rheumatology ◽

10.3899/jrheum.190313 ◽

2019 ◽

Vol 47 (10) ◽

pp. 1522-1531 ◽

Cited By ~ 1

Author(s):

Raphael Lhote ◽

Marie Chilles ◽

Matthieu Groh ◽

Xavier Puéchal ◽

Philippe Guilpain ◽

...

Keyword(s):

Median Time ◽

Granulomatosis With Polyangiitis ◽

Microscopic Polyangiitis ◽

Fold Increase ◽

Antineutrophil Cytoplasmic Antibody ◽

Mononeuritis Multiplex ◽

Biological Features ◽

Anca Associated Vasculitis ◽

Worse Prognosis

ObjectiveTo report on a large series of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and bronchiectasis, with a specific focus on the timeline of occurrence of both features.MethodsRetrospective nationwide multicenter study of patients diagnosed with both AAV and bronchiectasis.ResultsSixty-one patients were included, among whom 27 (44.25%) had microscopic polyangiitis (MPA), 27 (44.25%) had granulomatosis with polyangiitis (GPA), and 7 (11.5%) had eosinophilic GPA. Thirty-nine (64%) had myeloperoxidase (MPO)-ANCA and 13 (21%) had proteinase 3–ANCA. The diagnosis of bronchiectasis either preceded (n = 25; median time between both diagnoses: 16 yrs, IQR 4–54 yrs), was concomitant to (n = 12), or followed (n = 24; median time between both diagnoses: 1, IQR 0–6 yrs) that of AAV. Patients in whom bronchiectasis precedes the onset of AAV (B-AAV group) have more frequent mononeuritis multiplex, MPA, MPO-ANCA, and a 5-fold increase of death. The occurrence of an AAV relapse tended to be protective against bronchiectasis worsening (HR 0.6, 95% CI 0.4–0.99, P = 0.049), while a diagnosis of bronchiectasis before AAV (HR 5.8, 95% CI 1.2–28.7, P = 0.03) or MPA (HR 18.1, 95% CI 2.2–146.3, P = 0.01) were associated with shorter survival during AAV follow-up.ConclusionThe association of bronchiectasis with AAV is likely not accidental and is mostly associated with MPO-ANCA. Patients in whom bronchiectasis precedes the onset of AAV tend to have distinct clinical and biological features and could carry a worse prognosis.

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A case of ANCA associated vasculitis presented with mononeuritis multiplex

BIRDEM Medical Journal ◽

10.3329/birdem.v9i3.43094 ◽

2019 ◽

Vol 9 (3) ◽

pp. 257-260

Author(s):

Tanbin Rahman ◽

Md Rashedul Islam ◽

Mohammad Sakhawat Hossen Khan ◽

Sharif Mohammad Ruhul Quddus ◽

Dilruba Alam ◽

...

Keyword(s):

Inflammatory Cell ◽

Granulomatosis With Polyangiitis ◽

Microscopic Polyangiitis ◽

Systemic Vasculitis ◽

Antineutrophil Cytoplasmic Antibody ◽

Mononeuritis Multiplex ◽

Good Clinical Response ◽

Standard Regimen ◽

Anca Associated Vasculitis ◽

Common Manifestation

Mononeuritis multiplex is a common manifestation of many illnesses which includes diabetes, leprosy, malignancy and certain types of systemic vasculitis. The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of rare diseases which show typical characteristic inflammatory cell infiltration and blood vessel wall necrosis. AAV syndromes include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilicgranulomatosis with polyangiitis (EGPA). Here we present a patient who presented with mononeuritis multiplex and had features ofEGPA. The patient was treated with standard regimen of steroids and pulsed cyclophosphamide and she achieved good clinical response. Birdem Med J 2019; 9(3): 257-260

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Antineutrophil Cytoplasmic Antibody–Positive Vasculitis

Asthma ◽

10.1093/med/9780199918065.003.0002 ◽

2014 ◽

pp. 12-20

Author(s):

Lanny J. Rosenwasser ◽

Dennis K. Ledford

Keyword(s):

Granulomatosis With Polyangiitis ◽

Microscopic Polyangiitis ◽

Upper Airway ◽

Airway Disease ◽

Antineutrophil Cytoplasmic Antibody ◽

Eosinophilic Granulomatosis With Polyangiitis ◽

Anca Associated Vasculitis ◽

Granulomatous Polyangiitis ◽

Strauss Syndrome ◽

Eosinophilic Granulomatosis

The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis syndromes— eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome or allergic angiitis with granulomatosis), granulomatous polyangiitis (Wegener’s granulomatosis), and microscopic polyangiitis—are serious comorbidities of asthma or upper respiratory disease, or the symptoms of the vasculitis may resemble asthma or associated upper airway disease. ANCA-associated vasculitis is potentially fatal but is responsive to a variety of treatments, if the vasculitis is recognized before serious organ dysfunction. The role of ANCA in these conditions has not been defined because eosinophilic granulomatosis with polyangiitis, granulomatous polyangiitis, or microscopic polyangiitis may occur without ANCA, and the titer of ANCA does not predict clinical course. Clinical suspicion and consideration of tissue biopsy are important for recognition before irreversible complications occur. Corticosteroid therapy for asthma or suspected asthma may modify the presentations of ANCA-positive vasculitis.

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A case of ANCA associated vasculitis in a patient presenting with chest pain

Journal of International Medical Research ◽

10.1177/0300060520925940 ◽

2020 ◽

Vol 48 (5) ◽

pp. 030006052092594

Author(s):

Jinjin Fan ◽

Yu Yi ◽

Qianqian Wang ◽

Hui Li ◽

Xiaoli Jing

Keyword(s):

Computed Tomography ◽

Chest Pain ◽

Kidney Biopsy ◽

Clinical Manifestations ◽

Antineutrophil Cytoplasmic Antibody ◽

Chest Computed Tomography ◽

Blood Tests ◽

Anca Associated Vasculitis ◽

History Of

Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) is a group of multisystem autoimmune small vessel diseases. We report here a case of a 68-year-old woman who initially presented with 29-day history of chest pain, malaise and anorexia. Cardiac problems were ruled out and she was considered to have pneumonia. Her symptoms persisted and blood tests showed renal impairment and evidence of an inflammatory response. A kidney biopsy, chest computed tomography (CT) scan and ANCA testing confirmed a diagnosis of AAV renal injury. She was treated with glucocorticoids and cyclophosphamide (CTX) for six months at which time her kidney function had improved and she avoided the need for dialysis. This case study illustrates that the clinical manifestations of AVV are complex, varied, and prone to misdiagnosis.

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Sonographic Diagnosis of Unilateral Synchronous Testicular Tumors

American Journal of Sonography ◽

10.25259/ajs-3-2019 ◽

2019 ◽

Vol 2 ◽

pp. 2

Author(s):

Allison Forrest ◽

Numbereye Numbere ◽

Jerome Jean-Gilles ◽

Thomas Frye ◽

Vikram Dogra

Keyword(s):

Testicular Cancer ◽

Germ Cell ◽

Germ Cell Tumors ◽

Histological Type ◽

Synchronous Tumors ◽

Testicular Tumors ◽

Sonographic Diagnosis ◽

Histological Types ◽

Common Cancer

Testicular cancer accounts for 1% of all male cancers yet is the most common cancer affecting men aged 15–44 years. Most testicular cancers are seminomas or non-seminomatous germ cell tumors. Rarely, multiple testicular cancers may occur simultaneously, most often of the same histological type. However, synchronous tumors of different histological types may occur, although rarely. In this case study, we present the sonographic features with histopathologic correlation in a case of unilateral synchronous testicular tumors of discordant histology.

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ANCA Status or Clinical Phenotype — What Counts More?

Current Rheumatology Reports ◽

10.1007/s11926-021-01002-0 ◽

2021 ◽

Vol 23 (6) ◽

Author(s):

Martin Windpessl ◽

Erica L. Bettac ◽

Philipp Gauckler ◽

Jae Il Shin ◽

Duvuru Geetha ◽

...

Keyword(s):

Clinical Trials ◽

Granulomatosis With Polyangiitis ◽

Clinical Phenotype ◽

Antineutrophil Cytoplasmic Antibody ◽

Cardiovascular Death ◽

Genetic Associations ◽

Ongoing Debate ◽

The Past ◽

Anca Associated Vasculitis

Abstract Purpose of Review There is ongoing debate concerning the classification of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. That is, whether classification should be based on the serotype (proteinase 3 (PR3)- or myeloperoxidase (MPO)-ANCA) or on the clinical phenotype (granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA)). To add clarity, this review focused on integration of the most recent literature. Recent Findings Large clinical trials have provided evidence that a serology-based risk assessment for relapses is more predictive than distinction based on the phenotype. Research conducted in the past decade indicated that a serology-based approach more closely resembles the genetic associations, the clinical presentation (i.e., lung involvement), biomarker biology, treatment response, and is also predicting comorbidities (such as cardiovascular death). Summary Our review highlights that a serology-based approach could replace a phenotype-based approach to classify ANCA-associated vasculitides. In future, clinical trials and observational studies will presumably focus on this distinction and, as such, translate into a “personalized medicine.”

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An unusual precipitant of acute heart failure—ANCA-associated vasculitis in a patient with ischaemic cardiomyopathy: a case report

European Heart Journal - Case Reports ◽

10.1093/ehjcr/ytaa357 ◽

2020 ◽

Author(s):

Krishna Prasad ◽

Pruthvi C Revaiah ◽

Krishna Santosh Vemuri ◽

Parag Barwad

Keyword(s):

Heart Failure ◽

Renal Failure ◽

Acute Heart Failure ◽

Acute Decompensated Heart Failure ◽

Antineutrophil Cytoplasmic Antibody ◽

Left Ventricular ◽

Alveolar Haemorrhage ◽

Coronary Syndrome ◽

Anca Associated Vasculitis ◽

Renal Vasculitis

Abstract Background Antineutrophil cytoplasmic antibody (ANCA)-associated pulmonary renal vasculitis is an uncommon disease entity. Its presentation as acute heart failure for the first time in a patient with established coronary artery disease (CAD) is even rarer. We present here a case of such an association and an approach to managing this clinical situation. Case summary A 60-year-old male patient presented to the emergency room with recent-onset dyspnoea New York Heart Association Class IV. He was having hypertension, uncontrolled diabetes mellitus, chronic kidney disease (CKD), and CAD. He also underwent a percutaneous coronary intervention to left anterior descending in the past for acute coronary syndrome and had moderate left ventricular dysfunction. He was being managed as a case of acute decompensated heart failure (ADHF) and was mechanically ventilated. Suddenly his ventilator requirement increased and endotracheal aspirate contained blood. The chest radiograph showed bilateral hilar infiltrates. Simultaneously he also had recurrent episodes of ventricular tachycardia (VT) requiring direct current (DC) cardioversion. Blood investigations showed deranged renal function and severe hyperkalaemia, but no evidence of coagulopathy. High-resolution computed tomography chest showed features of diffuse alveolar haemorrhage. Further investigations revealed high titres of c-ANCA and raised inflammatory biomarkers. A diagnosis of ANCA-associated vasculitis presenting as acute on CKD with dyselectrolytaemia (hyperkalaemia) leading to VT was made. Apart from standard management for associated illness, he was treated with plasma exchange, steroids, and cyclophosphamide to which he responded and was later on discharged. Discussion Antineutrophil cytoplasmic antibody-related pulmonary renal vasculitis can lead to rapidly progressing renal failure and may present as ADHF in a patient with existent CAD. The associated VT storm in our patient can be attributed to hyperkalaemia secondary to acute renal failure. A multidisciplinary approach is required for the successful management of such a complex clinical scenario.

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

Case Reports in Nephrology and Dialysis ◽

10.1159/000517513 ◽

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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