Vasculitis 08-21-2018 Prof. Dr. med. Katharina Glatz Pathologie
Agenda Anatomy and histology Vasculitis: Chapel Hill Classification Examples Giant cell arteritis Single organ vasculitis
Artery or Vein?
Artery or Vein in the Subcutis? C-PAN or superficial thrombophlebitis? Features less reliable when inflammation is present Am J Dermatopathol 2013;35:463 471
Vascular Diversity: Function Nat. Rev. Rheumatol. 10, 454 462 (2014);
Vascular Diversity: Embryology differences in embryonic origins of vascular smooth muscle cells Arterioscler. Thromb. Vasc. Biol. 27, 1248 1258 (2007).
Vascular Diversity: Age
Chapel Hill Classification VASCULITIS
Pay Attention to Previous Findings!
Biopsy Border of a fresh lesion Incidental finding in resection: examine the stem vessel and «normal» tissue Site specific: Nasal mucosa: multiple, 3mm each Skin: including subcutis (DIF native in Michels) Temporal artery: 1cm segment Lung: Excision of the lesion
HE, EvG, DIF (Michels) Iron, CD15, CD3, CD68 Special stains for microorganisms
Chapel Hill Nomenclature 2012 Type and size of involved vessels Etiology, if known (f.e.anca-associated) Type of inflammation Involved organs Extravascular manifestations
Types of Vessels Affected Small arteries: all other extraparenchymal arteries Main visceral arteries: A. renalis, A. hepatica, Aa. mesentericae, coronaries) Small intraparenchymal arteries Arterioles, capillaries, venoles Aorta and branches supplying body regions: Head&Neck, extremities Jennette, J. C. et al. Arthritis Rheum. 65, 1 11 (2013)
Types of Vessels Affected Significant overlap!!! Jennette, J. C. et al. Arthritis Rheum. 65, 1 11 (2013)
If small vessels are involved: It s a small vessel vasculitis by definition! But...
Skin as an Exception Arthritis & Rheumatology Vol. 70, No. 2, February 2018, pp 171 184
Symptoms Arteritis: Ischemia Infarct Aneurysm Hemorrhage Small vessel vasculitis: Glomerulonephritis Pulmonary capillaritis Hemorrhagic alveolitis Petechiae, purpura Mucosal hemorrhage Polyneuropathy Muscle atrophy Phlebitis: Ischemia Infarction
When to Think of Vasculitis? Ischemic event Atypical localisation With systemic inflammation Atypical course Systemic illness No focus Resistant to antibiotics May become threatening ANCA V.: Acute renal failure/pulmonary hemorrhage Giant cell arteritis: blindness/stroke
Etiology Primary systemic vasculitis Single organ vasculitis Secondary vasculitis Collagenoses Infections Paraneoplastic Drugs (reactive inflammation/necrosis)
Type of Inflammation Vasculitis Giant cell arteritis Takayasu arteritis Polyarteritis nodosa Kawasaki syndrome ANCA-associated GPA EGPA MPA Immune complex vasculitis Single organ vasculitis Vasculitis in systemic disease Inflammation Pattern (acute phase) granulomatous (necrotizing <10%) necrotizing necrotizing, granulomatous necrotizing, granulomatous necrotizing necrotizing necrotizing, granulomatous necrotizing (rarely granulomatous)
Granulomatous Histiocytes & Giant Cells
Necrotizing Necrotizing vasculitis Fibrinoid necrosis Leucocytoclastic pattern call it SVV not LCV!
Type of Inflammation End stage Lymphocyte-rich Scarring Giant cells, necrosis, leucocytoclasia may be absent in late stages
Our Task Adhere to Chapel Hill Nomenclature Description: Type and size of involved vessels Type(s) of inflammation Extravascular changes Alveolar hemorrhage, crescentic glomerulonephritis: call the clinician
Epidemiology Primary Systemic Vasculitis Primary systemic vasculitis 43/m/y GCA GPA CSVV IgA, SHP MPA EGPA PAN Unclassified
Giant Cell Arteriitis EXAMPLE
Giant Cell Arteritis 3, sens. 93.5%, spec. 91% Age > 50 years (100%) New headache (75-90%) Abnormal temporal artery (25-50%) ESR>40mm/1h (95%) Classification Diagnostic Criteria! Hunder GG, Arthritis Rheum 1990, 33(8):1122-8 Positive biopsy (75-80%)
Biopsy Gold standard but: 15-40% false negative Modern imaging studies yield similar sensitivity & specificity 0.5-1cm The larger the better (skip lesions 10-30%) 1mm Examine every 0.5mm
Histological Findings Fragmentation of IEL Giant cells 50% Inflammation Various patterns <10% necrotizing Intimal hyperplasia +/- stenosing Pathologe 2012 (3): 228-235
Riesenzellarteriitis Giant Cell Arteritis Aorta Aorta Temporal artery
Treatment Effects First changes after 1 week Diagnosis is possible up to 2-3 months after initiation of treatment Treatment has priority Healed GCA: Intimal thickening Segmental fragmentation/loss of IEL Focal full-thickness loss or fibrosis of the media Adventitia with rare lymphocytes/fibrosis
Single Organ Vasculitis Gynecologic vasculitis: 0.15% of gynecologic resection specimens 70% isolated 30% systemic vasculitis Curr Opin Rheumatol 2008; 20:40 46.
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