Vasculitis Prof. Dr. med. Katharina Glatz Pathologie

Transcription

1 Vasculitis Prof. Dr. med. Katharina Glatz Pathologie

2 Agenda Anatomy and histology Vasculitis: Chapel Hill Classification Examples Giant cell arteritis Single organ vasculitis

3 Artery or Vein?

4 Artery or Vein in the Subcutis? C-PAN or superficial thrombophlebitis? Features less reliable when inflammation is present Am J Dermatopathol 2013;35:

5 Vascular Diversity: Function Nat. Rev. Rheumatol. 10, (2014);

6 Vascular Diversity: Embryology differences in embryonic origins of vascular smooth muscle cells Arterioscler. Thromb. Vasc. Biol. 27, (2007).

7 Vascular Diversity: Age

8 Chapel Hill Classification VASCULITIS

9

10 Pay Attention to Previous Findings!

11 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

12 HE, EvG, DIF (Michels) Iron, CD15, CD3, CD68 Special stains for microorganisms

13 Chapel Hill Nomenclature 2012 Type and size of involved vessels Etiology, if known (f.e.anca-associated) Type of inflammation Involved organs Extravascular manifestations

14 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)

15 Types of Vessels Affected Significant overlap!!! Jennette, J. C. et al. Arthritis Rheum. 65, 1 11 (2013)

16 If small vessels are involved: It s a small vessel vasculitis by definition! But...

17 Skin as an Exception Arthritis & Rheumatology Vol. 70, No. 2, February 2018, pp

18 Symptoms Arteritis: Ischemia Infarct Aneurysm Hemorrhage Small vessel vasculitis: Glomerulonephritis Pulmonary capillaritis Hemorrhagic alveolitis Petechiae, purpura Mucosal hemorrhage Polyneuropathy Muscle atrophy Phlebitis: Ischemia Infarction

19 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

20 Etiology Primary systemic vasculitis Single organ vasculitis Secondary vasculitis Collagenoses Infections Paraneoplastic Drugs (reactive inflammation/necrosis)

21 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)

22 Granulomatous Histiocytes & Giant Cells

23 Necrotizing Necrotizing vasculitis Fibrinoid necrosis Leucocytoclastic pattern call it SVV not LCV!

24 Type of Inflammation End stage Lymphocyte-rich Scarring Giant cells, necrosis, leucocytoclasia may be absent in late stages

25 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

26 Epidemiology Primary Systemic Vasculitis Primary systemic vasculitis 43/m/y GCA GPA CSVV IgA, SHP MPA EGPA PAN Unclassified

27 Giant Cell Arteriitis EXAMPLE

28 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): Positive biopsy (75-80%)

29 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

30 Histological Findings Fragmentation of IEL Giant cells 50% Inflammation Various patterns <10% necrotizing Intimal hyperplasia +/- stenosing Pathologe 2012 (3):

31 Riesenzellarteriitis Giant Cell Arteritis Aorta Aorta Temporal artery

32 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

33 Single Organ Vasculitis Gynecologic vasculitis: 0.15% of gynecologic resection specimens 70% isolated 30% systemic vasculitis Curr Opin Rheumatol 2008; 20:40 46.

34 Was ist Gesundheit??