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Vasculiti (Introduzione) - Medicascientia
Anatomia patologica,  Reumatologia

Vasculiti (Introduzione)

Vasculiti

Vasculitis

Copyright © 2012 Raman et al. “CMR in inflammatory vasculitis.” Journal of cardiovascular magnetic resonance: official journal of the Society for Cardiovascular Magnetic Resonance. Licensee BioMed Central Ltd. (CC BY 2.0)

Indice


Fonti, link esterni, immagini, libri e approfondimenti


Introduzione


Vasculite a IgA

IgA vasculitis (Henoch–Schönlein purpura)

Copyright © 2015 Kang et al. “High-dose methylprednisolone pulse therapy for treatment of refractory intestinal involvement caused by Henoch-Schönlein purpura: a case report.” Journal of medical case reports (CC BY 4.0))

La vasculite è un quadro clinicopatologico caratterizzato da infiammazione e danno a carico della parete dei vasi sanguigni.

Può colpire vasi di qualsiasi tipo: arterie, arteriole, capillari, venule o vene.

In base alla distribuzione del coinvolgimento vascolare, la vasculite può essere sistemica o organo-specifica.

Le manifestazioni cliniche variano in base alle dimensioni e alla localizzazione dei vasi coinvolti, all’entità del coinvolgimento dell’organo, al grado e al tipo di infiammazione e alle sue conseguenze (stenosi fino all’occlusione del lume, trombosi, formazione di aneurismi, rottura della parete).

Classificazione


Le vasculiti possono essere classificate secondo vari criteri:

  • Etiologia
  • Patogenesi
  • Calibro dei vasi maggiormente coinvolti
  • Sede
  • Quadro istologico
  • etc.

Classificazione etiologica

Le vasculiti possono essere primitive o secondarie.

L’etiologia delle vasculiti sistemiche primitive è sconosciuta, probabilmente multifattoriale, dovuta sia a predisposizione genetica che a fattori ambientali.

Le vasculiti secondarie possono essere associate a numerose condizioni:

Classificazione patogenetica

Dal punto di vista patogenetico, le vasculiti possono essere suddivise in due grandi gruppi: infettive (vedere Vasculiti infettive) e immunomediate.

Pur con una certa sovrapposizione tra i diversi meccanismi, le vasculiti immunomediate possono essere dovute a:

Patogenesi della vasculite a IgA

Pathogenesis of IgA vasculitis

Copyright © 2021 Song et al. “Pathogenesis of IgA Vasculitis: An Up-To-Date Review.” Frontiers in Immunology (CC BY 4.0)

Patogenesi delle vasculiti ANCA-associate

Pathogenesis of ANCA-associated vasculitis

Copyright © 2020 Kronbichler et al. “Immunopathogenesis of ANCA-Associated Vasculitis” International Journal of Molecular Sciences. Licensee MDPI, Basel, Switzerland. CC BY 4.0

Patogenesi dell'arterite a cellule giganti

Pathogenesis of giant cell arteritis

Copyright © 2022 Szekeres and Al Othman (CC BY)

Classificazione morfologica

Vasculiti: anatomia patologica (granulomatosi eosinofila con poliangioite)

Eosinophilic granulomatosis with polyangiitis / Churg-Strauss Syndrome
In the center of this image there is a blood vessel exhibiting fibrinoid necrosis. It is surrounded by a dense mixed inflammatory infiltrate containing a large number of eosinphiles

Copyright © 2014 Dr. Yale Rosen Atlas of Pulmonary Pathology (CC BY-SA 2.0)

È possibile distinguere alcuni quadri morfologici di vasculite:

Vasculiti: anatomia patologica (arterite a cellule giganti)

Micrographs of giant cell arteritis (H&E stain)

Copyright © 2011 Michael Bonert, MD, FRCPC ( https://commons.wikimedia.org/wiki/User:Nephron / https://fhs.mcmaster.ca/pathology/contact_us/faculty/faculty_bios/Bonert.html ). CC BY-SA 3.0

Nomenclatura della Chapel Hill Consensus Conference


Vasculiti: nomenclatura della Chapel Hill Consensus Conference 2012

Copyright © 2021 H. Almoallim, M. Cheikh. “Skills in Rheumatology”, https://doi.org/10.1007/978-981-15-8323-0_20 (CC BY 4.0)

La nomenclatura più utilizzata per le vasculiti è quella della Chapel Hill Consensus Conference, elaborata nel 1994 e aggiornata nel 2012, basata sulle dimensioni dei vasi prevalentemente coinvolti:

Vasculiti: definizioni della Chapel Hill Consensus Conference 2012
Vasculiti

Epidemiologia delle vasculiti primitive


Quadro clinico


Malattia di Kawasaki

Kawasaki disease

Copyright © 2018 Hedrich  et al. “Kawasaki Disease.” Frontiers in Pediatrics (CC BY 4.0)

Il quadro clinico delle varie vasculiti sistemiche è estremamente eterogeneo.

Vasculiti: quadro clinico
Vasculiti: arterite a cellule giganti

Prominent, beaded, pulseless, tender temporal artery in a patient with biopsy proven giant cell arteritis

Copyright © 2020 Lyons et al. “A new era for giant cell arteritis.” Eye (CC BY 4.0)

Vasculiti: malattia di Kawasaki

Main clinical signs of acute Kawasaki disease

Copyright © 2018 Marrani et al. “How Should We Classify Kawasaki Disease?” Frontiers in Immunology (CC BY 4.0)

Vasculiti ANCA-associate: granulomatosi con poliangioite

Clinical features of granulomatosis with polyangiitis

Copyright © 2021 Padoan et al. ” Ear, nose, and throat in ANCA-associated vasculitis: a comprehensive review.” Vessel Plus (CC BY 4.0)

Vasculiti ANCA-associate: granulomatosi con poliangioite (gengivite a fragola)

Characteristic appearance of strawberry gingivitis (granulomatosis with polyangiitis)

Copyright © 2018 Coulter et al. ” Granulomatosis with polyangiitis presenting with facial nerve paralysis and strawberry gingivitis in a child.” Otolaryngology Case Reports (CC BY 4.0)

Sclerite

Scleritis

Copyright © 2022 Pytrus et al. https://doi.org/10.3390/jcm11247457, Journal of Clinical Medicine, Licensee MDPI, Basel, Switzerland. CC BY 4.0

Right eyelid edema due to an abscess in the lower eyelid (granulomatosis with polyangiitis)

Copyright © 2023 Byszewska et al. “Ocular Involvement of Granulomatosis with Polyangiitis” Journal of Clinical Medicine. Licensee MDPI, Basel, Switzerland. CC BY 4.0

Vasculiti: porpora palpabile

Lower extremity palpable purpura

Copyright © 2016 Morgado et al. https://doi.org/10.7759/cureus.900, Cureus (CC BY)

Vasculiti: porpora palpabile

Lower extremity palpable purpura

Copyright © 2023 Yousif et al. “Drug-Induced IgA Vasculitis in an Adult.” Cureus (CC BY 4.0)

Vasculiti: porpora palpabile e ulcere

Copyright © 2019 Sudbury et al. https://doi.org/10.7759/cureus.5783, Cureus (CC BY)

Vasculiti: lesioni cutanee

(A) Purpuric lesions; may become confluent (B). (C-D): livedo reticularis. (E) Chronic leg ulcers. (F) Ulcers healing after therapy.

Copyright © 2013 Lauletta. Licensee IntechOpen (CC BY 3.0)

Fingertips of both hands showing multiple dry gangrene

Copyright © 2020 Koh et al. ” Multiple hepatic aneurysms and dry gangrene of fingertips in eosinophilic granulomatosis with polyangiitis: a case report.” Allergy, Asthma & Clinical Immunology CC BY 4.0

Orticaria vasculitica

Severe urticarial vasculitis

Copyright © 2015 Khatri et al. DOI:10.7241/ourd.20153.99, Our Dermatol Online (CC BY)

Vasculiti: malattia di Behçet

Behçet’s disease
(A) Oral aphthous lesion (B) Genital ulceration (arrow) and scar (arrow head) (C) Erythema nodusum (D) Pustular lesion (E) Uveitis in a patient with Behçet’s disease (F) Difference in diameter between extremities suggesting deep vein thrombosis.

Copyright © 2021 Yildiz et al. “Pediatric Behçet’s Disease”, Frontiers in medicine (CC BY)

Vasculiti: malattia di Behçet

A 27 year-old pregnant woman with Behçet’s disease presented with a hypopyon panuveitis

Copyright © 2009 Tugal-Tutkun, Ilknur. “Behçet’s Uveitis.” Middle East African journal of ophthalmology (CC BY)

Vasculiti: sindrome di Cogan

Interstitial keratitis in a patient with Cogan’s Syndrome

Copyright © 2017 Thiago Gonçalves dos Santos Martins et al. “Interstitial keratitis in patients with Cogan’s Syndrome.” Revista Brasileira de Oftalmologia (CC BY 4.0)

Diagnosi


Diagnosi delle vasculiti: angio-TC in paziente con arterite di Takayasu

Computed Tomography Angiography of a 66-year-old woman with Takayasu Arteritis

Copyright © 2021 Oura et al. “Vascular Imaging Techniques to Diagnose and Monitor Patients with Takayasu Arteritis: A Review of the Literature.” Diagnostics. Licensee MDPI, Basel, Switzerland. CC BY 4.0

La diagnosi di vasculite si basa sulla combinazione dei dati clinici, sierologici, di imaging e anatomopatologici.

Devono far sospettare la presenza di vasculite soprattutto:

  • Febbre inspiegabile, cefalea, perdita di peso, malessere generalizzato, astenia, artromialgie diffuse
  • Porpora palpabile
  • Ematuria microscopica
  • Glomerulonefrite con evidenza di patologia multisistemica
  • Eventi ischemici di origine ignota
  • Mononeurite multipla

Nel sospetto di vasculite sono fondamentali:

Possono rendersi necessarie, a seconda dei casi, altre indagini:

  • Valutazione cardiaca (ecocardiografia, ECG, RM cardiaca, etc.)
  • Visita oftalmologica (lampada a fessura, esame del fundus, fluorangiografia retinica, etc.)
  • Visita otorinolaringoiatrica (fibroscopia, audiometria, etc.)
  • Spirometria, broncoscopia, lavaggio broncoalveolare
  • Endoscopia digestiva
  • Visita neurologica

La diagnosi differenziale è vasta:

Diagnosi delle vasculiti: ecocolordoppler in paziente con arterite a cellule giganti

Giant cell arteritis
Temporal artery ultrasound images showing (a) longitudinal and (b) cross sectional images of a normal artery; (c) longitudinal and (d) cross section image of the non-compressible, hypoechoic “halo sign”.

Copyright © 2020 Lyons et al. “A new era for giant cell arteritis.” Eye (CC BY 4.0)

Takayasu arteritis
Severe disease of the great vessels including stenoses of the right subclavian and common carotid arteries (arrows) and occlusion of the left subclavian and left common carotid arteries (stars).

Copyright © 2013 Perera et al. “Takayasu arteritis: criteria for surgical intervention should not be ignored.” International journal of vascular medicine (CC BY)

CT aortography of a 69-year-old male with large-vessel vasculitis

Copyright © 2023 Stojanovic et al. https://doi.org/10.3390/diagnostics13030484, Diagnostics. Licensee MDPI, Basel, Switzerland. CC BY 4.0

Diagnosi delle vasculiti: angiografia in paziente con poliarterite nodosa

Polyarteritis Nodosa
Comparative angiography of the left renal arteries: (A) Initial lesions and (B) After successful treatment and one year of follow up

Copyright © 2021 Maza et al. “Giant Coronary Artery Aneurysm due to Polyarteritis Nodosa in an Eight-Year-Old Child.” International Journal of Clinical Cardiology (CC BY)

Diagnosi delle vasculiti: malattia di Kawasaki

Kawasaki disease
Giant coronary artery aneurysm with a thrombus

Copyright © 2008 Patil et al. “Giant coronary artery aneurysm with a thrombus secondary to Kawasaki disease.” Annals of pediatric cardiology (CC BY)

Diagnosi delle vasculiti: coinvolgimento cardiaco in paziente con granulomatosi eosinofila con poliangioite

Eosinophilic granulomatosis with polyangiitis with cardiac involvement

Copyright © 2023 Fijolek and Radzikowska. “Eosinophilic granulomatosis with polyangiitis – Advances in pathogenesis, diagnosis, and treatment.” Frontiers in medicine (CC BY)

Diagnosi delle vasculiti: Rx torace in paziente con granulomatosi con poliangioite

Granulomatosis with polyangiitis
Chest radiographs demonstrating cavitation granulomas before and after treatment.

Copyright © 2012 Tagboto and Venkatesh. https://doi.org/10.1186%2F1471-2466-12-47, BMC pulmonary medicine, Licensee BioMed Central Ltd. CC BY 2.0

Diagnosi delle vasculiti: emorragia alveolare diffusa

Chest radiograph showing bilateral diffuse alveolar infiltrates and CT thorax showing diffuse ground glass opacities.

Copyright © 2011 Mahajan et al. “Diffuse alveolar hemorrhage in Wegener’s granulomatosis.” Lung India : official organ of Indian Chest Society (CC BY)

Diagnosi delle vasculiti: granulomatosi con poliangioite

Granulomatosis with Polyangiitis
MRI of coronal plane with a massive lesion in the right ethmoid sinus cavity

Copyright © 2023 Byszewska et al. “Ocular Involvement of Granulomatosis with Polyangiitis” Journal of Clinical Medicine. Licensee MDPI, Basel, Switzerland. CC BY 4.0

Diagnosi delle vasculiti: neuro-Behçet

Neuro-Behcet
A patient with neuro-Behcet showing a left-sided hyperintense lesion on axial T2-weighted (a) and coronal FLAIR (b) imaging involving the cerebellum, the midbrain, and the inferior part of the thalamus (arrows), the internal capsule, and the putamen.

Copyright © 2014 Renard et al. “Thalamic lesions: a radiological review.” Behavioural neurology (CC BY)

Diagnosi delle vasculiti: granulomatosi con poliangioite

Granulomatosis with polyangiitis
Nasal endoscopy revealing a single cavity by disappearance of the septal cartilage, an atrophic, friable, crust-covered mucosa, almost complete disappearance of the inferior and middle nasal turbinate, and rhino pharynx covered by gray crusts.

Copyright © 2022 Trandafir et al. ” Granulomatosis with Polyangiitis (GPA)—A Multidisciplinary Approach of a Case Report” Medicina. Licensee MDPI, Basel, Switzerland. CC BY 4.0

Diagnosi delle vasculiti: stenosi subglottica in paziente con granulomatosi con poliangioite

Endoscopic view of a concentric subglottic stenosis

Copyright © 2021 Padoan et al. ” Ear, nose, and throat in ANCA-associated vasculitis: a comprehensive review.” Vessel Plus (CC BY 4.0)

Diagnosi delle vasculiti: poliposi nasale in paziente con granulomatosi eosinofila con poliangioite

Eosinophilic Granulomatosis with Polyangiitis
Nasal endoscopy showing polypoid formations

Copyright © 2023 Pirola et al. ” Evolution in Endoscopic Sinus Surgery: The Adjuvant Role of Reboot Surgery in Patients with Uncontrolled Nasal Symptoms of Eosinophilic Granulomatosis with Polyangiitis” Journal of Personalized Medicine. Licensee MDPI, Basel, Switzerland. CC BY 4.0

Diagnosi delle vasculiti: esofagogastroduodenoscopia in paziente con vasculite a IgA

IgA vasculitis (Henoch–Schönlein purpura)
Endoscopic finding on EGD showing inflammation, submucosal hemorrhage, and small ulceration.

Copyright © 2010 Sohagia et al. “Henoch-schonlein purpura-a case report and review of the literature.” Gastroenterology research and practice (CC BY)

Diagnosi delle vasculiti: colonscopia in paziente con malattia di Behçet

Behçet’s Syndrome
Intestinal ulcers in four patients with intestinal Behçet’s Syndrome

Copyright © 2022 Zhao et al. https://doi.org/10.3389/fimmu.2022.981502, Frontiers in immunology (CC BY)

Vasculiti: anatomia patologica (vasculite leucocitoclastica)

Skin biopsy of one lesion in H&E staining revealing signs of a small vessel leukocytoclastic vasculitis limited to the upper dermis

Copyright © 2019 Nothhaft et al. “Hemorrhagic Bullous Henoch-Schönlein Purpura: Case Report and Review of the Literature.” Frontiers in Pediatrics (CC BY 4.0)

Vasculiti: anatomia patologica (neuropatia vasculitica)

High magnification micrograph of a vasculitic neuropathy. Resin section (plastics) with toluidine blue stain.

Copyright © 2011 Michael Bonert, MD, FRCPC (https://commons.wikimedia.org/wiki/User:Nephron / https://fhs.mcmaster.ca/pathology/contact_us/faculty/faculty_bios/Bonert.html). CC BY-SA 3.0

Vasculiti: anatomia patologica (granulomatosi con poliangioite)

Granulomatosis with polyangiitis/Wegener’s granulom­atosis
Core biopsy fragment (pulmonary nodule): (left) Low magnification view showing an area of granulomatous inflammation with giant cells and a large area of basophilic necrosis at the red arrow. Separate fragments of basophilic necrotic material are present along the right edge of the image. The nodular lesion seen at the black arrow is an inflamed artery (arteritis).
(Right) Higher magnification view of the area showing granulomatous inflammation and basophilic necrosis.

Copyright © 2012 Dr. Yale Rosen Atlas of Pulmonary Pathology (CC BY-SA 2.0)

Granulomatosis with polyangiitis, lung biopsy, H&E stain, intermediate, high and very high magnification.

Copyright © 2011 Michael Bonert, MD, FRCPC ( https://commons.wikimedia.org/wiki/User:Nephron / https://fhs.mcmaster.ca/pathology/contact_us/faculty/faculty_bios/Bonert.html ). CC BY-SA 3.0

Vasculiti: anatomia patologica

Microscopical aspect of two arterioles with vasculitic changes
(A) Vessels are surrounded by dense histiocytic infiltrates forming, in many cases, granulomas. Many vessels also show platelet-rich thrombi associated to inflammatory changes of the wall.
(B) The vascular wall shows large areas of fibrinoid necrosis with neutrophil granulocytes that were nearly complete destroyed.
* lumen of the vessel; FN: fibrinoid necrosis; HIS: histiocytic infiltration; IN: intima; SM: smooth muscle; TH: thrombus

Copyright © 2013 Timmermann et al. https://doi.org/10.1186/1752-1947-7-141, Journal of medical case reports, Licensee BioMed Central Ltd. CC BY 2.0

Vasculiti: anatomia patologica (distruzione della lamina elastica interna)

Elastic tissue stain of an inflamed pulmonary artery with focal destruction of the internal elastic lamina.

Copyright © 2012 Dr. Yale Rosen Atlas of Pulmonary Pathology (CC BY-SA 2.0)

Vasculiti: anatomia patologica (sindrome di Goodpasture)

Goodpasture’s syndrome
(a-b) Diffuse alveolar hemorrhage and hemosiderosis in the lung sections (panel a ×100, panel b ×400).
The hemosiderosis was confirmed by diffuse positive iron staining in hemosiderin-laden macrophages in panel (c) (×400)

Copyright © 2014 Ward et al. https://doi.org/10.1155/2014/286030, Case reports in pathology (CC BY)

Vasculiti: anatomia patologica (granulomatosi eosinofila con poliangioite)

Eosinophilic Granulomatosis with Polyangiitis
(A) 20x image. Microscopic examination demonstrates dense eosinophilic infiltrate involving alveolated lung parenchyma and many small venules, accompanied by patchy organization (black arrow) (H&E stained sections, 20x). (B) 40x image. Eosinophilic venulitis. Chronic inflammation including many eosinophils infiltrating venular wall (black arrow head) (H&E stained sections, 40x).

Copyright © 2018 Dalia et al. “Eosinophilic Myocarditis Demonstrated Using Cardiac Magnetic Resonance Imaging in a Patient with Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Disease).” Cureus (CC BY)

Vasculiti: anatomia patologica (glomerulonefrite pauci-immune)

Kidney biopsy showing features suggestive of pauci-immune crescentic glomerulonephritis
(Left) Section of glomerulus with methenamine silver staining showing circumferential cellular crescent with fibrinoid necrosis and leukocytic exudation.
(Right) Another section with trichrome staining showing single glomerulus with segmental endocapillary proliferation, fibrinoid necrosis, and breakage of bowman’s capsule along with leukocytic exudation.

Copyright © 2016 Roy et al. “A Case of Wegener’s Granulomatosis Presenting with Unilateral Facial Nerve Palsy.” Case reports in medicine (CC BY)

Vasculiti: anatomia patologica (glomerulonefrite crioglobulinemica)

Renal biopsy
(Left) Diffuse endocapillary proliferative and exudative glomerulonephritis with membranoproliferative features and numerous intracapillary protein thrombi.
(Right) Immunofluorescence findings of intense glomerular capillary wall and intraluminal staining in the distribution of the protein thrombi for IgM, C3 and C1q.

Copyright © 2009 Geara et al. “Pseudoleukocytosis secondary to hepatitis C-associated cryoglobulinemia: a case report.” Journal of medical case reports. Licensee BioMed Central Ltd. CC BY 2.0

Criocrito

Cryocrit determination in a patient with mixed cryoglobulinemia

Copyright © 2008 Ferri. “Mixed cryoglobulinemia.” Orphanet journal of rare diseases. Licensee BioMed Central Ltd. CC BY 2.0

Behçet’s disease
(a) Retinal photography of the right eye of a patient with Behçet’s disease showing a white infiltrate in the inter-papillomacular area associated with retinal edema and hemorrhage. (b) Fluorescein angiography showing vascular staining in the involved area. (c) Two months later, retinal photography showing retinal and optic atrophy with resolution of the infiltrate. (d) Two months later, spectral domain OCT scan showing atrophy of the inner retinal layers.

Copyright © 2021 Gueudry et al. “Old and New Challenges in Uveitis Associated with Behçet’s Disease.” Journal of Clinical Medicine, Licensee MDPI, Basel, Switzerland. CC BY 4.0

Audiogram and vHIT

Copyright © 2022 Mendis et al. “Autoimmune Vestibulopathy—A Case Series” Brain Sciences, Licensee MDPI, Basel, Switzerland. CC BY 4.0

Tabella riassuntiva


Vasculiti

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