Pulmonary Vasculitides: A Radiological Review Emphasizing Parenchymal HRCT Features

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).

Antineutrophil Cytoplasmic Autoantibody-Associated Vasculitis with Kidney Involvement in a Patient with AL Amyloidosis

Antineutrophil cytoplasmic autoantibody (ANCA) vasculitis has occasionally been associated with other systemic glomerulonephritis, such as anti-glomerular basement membrane disease. Here, we report the first clinical case of ANCA-associated crescentic glomerulonephritis with AL amyloidosis. An 81-years-old gentleman presented to the hospital with acute kidney injury (serum creatinine 4.7 mg/dL) on a background of chronic kidney disease and volume overload. Autoimmune serology was remarkable for p-ANCA and myeloperoxidase positivity. A renal biopsy confirmed pauci-immune glomerulonephritis and lambda light-chain amyloid deposition (confirmed on liquid chromatography and tandem mass spectrometry). The patient was initially managed with rituximab and subsequently transitioned to bortezomib-based chemotherapy but died due to decompensated heart failure. This case report promotes greater awareness of the unusual presentation of amyloidosis and guides future research and treatment.

Antineutrophil Cytoplasmic Antibody-associated Vasculitis With Alveolar Hemorrhage and Ruptured Renal Aneurysm: a Case Report and Literature Review

Background: Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) is characterized by necrotizing damage of small -vessel vasculitis and the main violation of the kidney or lung. Manifestation of alveolar hemorrhage and renal aneurysm is extremely rare in Granulomatosis with polyangiitis (GPA).Case presentation: A 50-year-old Chinese man was admitted due to repeated cough, expectoration, fever and shortness of breath. A chest computed tomography (CT) scan showed bilateral infiltrates in the lower lobe of the lung. GPA was considered based on the pulmonary capillaritis, alveolar hemorrhage, renal insufficiency and cANCA -PR3 positivity. Immunosuppressive therapy combined with plasma exchange (PE) was performed. The patient suffered from a rupture of renal aneurysm. Though selective renal arterial embolization was performed, the patient had a poor outcome. Conclusions: GPA could be life-threatening, especially large vessel vasculitis involved. The possibility of aneurysmal rupture should be carefully considered and checked frequently in the condition of immunosuppressive therapy.

Clinical and pathological characteristics of ANA/anti-dsDNA positive patients with antineutrophil cytoplasmic autoantibody-associated vasculitis

Antineutrophil cytoplasmic autoantibodies (ANCA) associated vasculitis (AAV) consists of a group of systemic autoimmune diseases. The roles of serum anti-nuclear antibodies (ANA) and anti-double-stranded DNA (anti-dsDNA) antibodies in AAV patients remain unknown. This study investigated the prevalence of serum ANAs and anti-dsDNA antibodies in AAV patients and characterized the clinical and pathological features of these patients. A total of 218 AAV patients were enrolled. Clinical and pathological data of patients were analyzed retrospectively. Of the 218 AAV patients, 109 (50.0%) were positive for ANA, 45 (20.6%) were positive for anti-dsDNA, and 43 (19.7%) were positive for both. The AAV patients with ANA had severer kidney damage and more chronic renal histopathological changes compared to those who were negative for ANA. Specifically, patients positive for ANA had more hypertension, higher levels of urea nitrogen and serum creatinine, lower estimated glomerular filtration rate (eGFR), more end-stage renal disease (ESRD), severer proteinuria, glomerular sclerosis, tubular interstitial fibrosis and tubular atrophy, and were more likely to receive renal biopsies compared to ANA negative patients. The study found ANA and anti-dsDNA in AVV patients were not rare, ANA-positive AAV patients had severer kidney damage and more chronic renal histopathological changes compared to ANA-negative AAV patients. Renal biopsy is strongly recommended for differential diagnosis in such cases.

Life-threatening antineutrophil cytoplasmic antibody–associated vasculitis after influenza A H1N1 infection requiring veno-venous extracorporeal membrane oxygenation

Introduction: Antineutrophil cytoplasmic autoantibody–associated vasculitis is an immune-mediated necrotizing vasculitis, affecting small- and medium-sized vessels. Case report: A 22-year-old female patient with free medical history presented with life-threatening pulmonary hemorrhage due to antineutrophil cytoplasmic autoantibody–associated vasculitis, temporarily associated with influenza A H1N1 infection. Due to rapidly worsening respiratory failure, despite conventional management, veno-venous peripheral extracorporeal membrane oxygenation was initiated and continued for 26 days, with subsequent renal replacement therapy. Discussion: We present a case of severe antineutrophil cytoplasmic autoantibody–associated pulmonary vasculitis, managed with veno-venous extracorporeal membrane oxygenation at the initial phase. Despite the significant challenges raised with the use of extracorporeal membrane oxygenation in pulmonary hemorrhage cases, extracorporeal membrane oxygenation may have a significant impact on outcome in this setting, by providing adequate time for a successful immunosuppressive treatment.