Vasculitis Flashcards
What are vasculites?
The primary systemic vasculitis’s are a group of clinical disorders characterised by blood vessel inflammation and necrosis causing ischemia and organ damage.
Multi-system involvement is common, because the inflammation can involve a number of vascular beds (e.g. renal, pulmonary, cutaneous).
A high index of suspicion is required to initiate the appropriate tests (blood tests, biopsy, angiography etc) to make the diagnosis.
Primary vasculitis’s can be classified in various ways. The following examples are based on a classification according to the size of the blood vessels involved (as proposed by Chapel Hill Consensus conference on nomenclature of systemic vasculitis)
Secondary vasculitis can occur to other diseases e.g SLE, RA, Hepatitis B,C HIV
Infective
e.g. Subacute infective endocarditis
Non-infective
Vasculitis with rheumatoid arthritis, Systemic lupus erythematosus, Scleroderma, Polymyositis/dermatomyositis, Drug-induced Behçet’s disease, Goodpasture’s syndrome, Hypocomplementaemia, Serum sickness, Paraneoplastic syndromes, Inflammatory bowel disease
What are some examples of large vessel vasculitis
Large vessel vasculitis = REFERS TO THE AORTA/AND MAJOR TRIBUTARIES
• Giant cell arteritis/polymyalgia rheumatica
• Takayasu arteritis
What are some examples of some medium vessel vasculitis
REFERS TO MEDIUM AND SMALL SIZED ARTERIES AND ARTERIOLES
• Polyarteritis nodosa
• Kawasaki disease
What are some examples of small vessel vasculitis, which are ANCA associated and which are immune complex mediated?
Small vessel vasculitis (SVV) = SMALL ARTERIES, ARTERIOLE, VENULES AND CAPILLARIES
• these are the more important ones as they are antibody mediated and they go against the ANCAS- neutrophils
• GPA, MPA, EGPA are often associated with ANCA
ANCA associated vasculitis
• Granulomatosis with Polyangiitis(GPA) formerly Wegener’s Granulomatosis - get ischaemia as it can occur in the kidney and the brain
• Microscopic Polyangiitis (MPA)
• Eosinophilic Granulomatosis with Polyangiitis(EGPA) Churg-Strauss syndrome
Immune complex SVV • Anti-GBM disease • Cryoglobulinaemic vasculitis • IgA vasculitis (Henoch-Schonlein) • Hypocomplementaemic urticarial vasculitis
GPA and MPA may cause rapidly progressive glomerulonephritis and ‘pulmonary renal’ syndromes (also seen in Anti –GBM disease/Goodpasture’s).
What are some examples of variable cell vasculitis
Behcet’s and Cogan’s syndrome
What ist he aetiology and pathophysiology of vasculitis and immunodeficiency?
Inflammation of the blood vessels- immune system attacks the blood vessels therefore they become swollen and narrowed= AUTOIMMUNE
Can be triggered by infection or medicine but in most cases is not known
• WBC mix up antigens- molecular mimicry e.g large and medium cell
• Can damage indirectly e.g small vessel disease
1. Type III hypersensitivity–> immune complex deposited cause damage
2. Autoantibodies e.g ANCA
3. Cell mediated immunity
Pathophysiology
• Within adventitia find the immature dendritic cells
• When antigen is picked up to the APC- go to the LN
• Activates CD4+ cells T cells
• Activates CD8 and B cells (antibodies)- these may target the blood vessels via molecular mimicry or picked up antigens from the BV
• Chemokines can also cause inflammation in the blood vessels too!
• Antibodies can form immune complex - they can deposit causes complement activation- this triggers inflammation
• Neutrophils are circulating and the immune complex’s can premature activated them- inflammation
• May get palpable purpura
Haemostasis occurs to reduce damage- and then healing occurs but then may get stenosis and lumen narrowing- ischaemia, necrosis
Vessel injury- can lead to weakened vessel wall and aneurysm
ANCA Positive: is when B cells produce antibodies to the neutrophils called anti neutrophilic cytoplasmic antibodies (ANCA)- mainly IgG
Either to myeloperoxidase- pANCA or to neutrophil proteinase 3- c-ANCA
Pauci immune vasculitis: have a paucity or lack of immune complex
What vasculitis presents with? Eyes- sudden blindness Jaw claudication Thickened temporal artery Headache new onset Female older patient often have PMR
Segmental effect–> biopsy
Giant cell arteritis
What vasculitis presents with?
Stiffness in the morning lasting about 30 minutes
Pelvic girdle or shoulder pain
Fever and weight loss
Polymyalgia rheumatica
What vasculitis presents with?
Usually Asian females <40 years old
Thickening and narrowing of the aortic arch and proximal great vessels
Pulseless disease- upper extremity pulses weak, fever, night sweats, arthritis, myalgias, skin nodules, ocular disturbances and neurological symptoms
May have different pulse pressure and also bruits
Takayau’s arteritits
What vasculitis presents with?
Usually middle aged men - typically renal and visceral vessels - necrotizing vasculitis (where all the layers die, fibrinoid necrosis as it heals) causes aneurysm and thrombosis in middle arteries leading to infection and severe systemic symptoms
Hepatitis B seropositivity 30%- immune complex may lead to damage
May be positive for ANCA
Fever, weight loss, malaise, headache
GI abdominal pain, melaena (mesenteric arteries)
Hypertension (due to renal), neurological dysfunction (brain arteries), cutaneous eruptions (skin disease), renal damage
Polyarteritis nodosa
Presents with:
Migraine (headaches), strokes, vague aches and pains, heart attacks, bowel infarcts, nephritis/kidney failure, gangrene, peripheral nerve damage
It is a THREE LAYER PATCHY VASCULITIS- which results in strings of beads on angiograpm
At least two types: hepatitis immune vasculitis, anti neutrophil antibodies ‘
Systemic vasculitic symptoms in the presence of hepatitis B signs and in the absence of pulmonary symptoms/signs suggests a diagnosis polyarteritis nodosa usually SPARES THE LUNGS
What vasculitis presents with?
Heavy smokers, males <40 years old
Clots in the vessels supply the fingers and toes
Segmental thrombosing vasculitis with nerve involvement
Intermittent claudication- may cause gangrene
Autoamputation of the digits, superficial nodular phlebitis
Raynaud phenomenon often present
Buerger disease (thromboangiitis obliterans)
What vasculitis presents with?
Asian children <4 years old
Affects coronary arteries or large/medium sized
Collagen and TF exposed- increased clots, weak walls, aneurysms, thickened, can get ischemia
May develop coronary artery aneurysms thrombosis or rupture can cause death
CRASH and BURN
Conjunctival injections or hyperema
Rash (polymorphous -desquamating flakes, peeling)
Adenopathy (Cervical)
Strawberry tongue (oral mucositis) + red mouth and throat- cracking
Hand foot changes (edema, erythema)
Fever
Kawasaki disease (mucocutaneous LN syndrome)
Self limiting- resolves but get CVD complications
Must do echo
IVIG (antibodies from others) and Aspirin- may get reye but +>-
What presents with?
Affects middle aged- or elderly males
Systemic necrotizing granulomatous vasculitis
Presents with gangrene, sore joints
Atypical CXR
Upper respiratory tract: perforation of the nasal septum, chronic sinusitis, otitis media, mastoiditis
Lower respiratory tract: hemoptysis, cough, dyspnea
Renal: hematuria, red cell casts- resists blood to the glomeruli
Triad:
Focal necrotizing vasculitis
Necrotizing granulomas in lung and upper airway
Necrotizing glomerulonephritis
PR3-ANCA/C-ANCA (cytoplasmic neutrophilic cytoplasmic antibodies to proteinase 3-> produce Oxygen free radical- damage the BV
Wegner’s granulomatosis - granulomatosis with polyangiitis
Saddle nose deformity in the nose
Corticosteroids and cyclophosphamide
ANCA POSITIVE
What presents with?
Necrotizing vasculitis commonly involving the lung, kidney, skin with pauci-
immune glomerulonephritis and palpable purpura
This can be precipitated by medications e.g antibodies like penicillin
MPO-ANCA/P-ANCA
This pANCA is perinuclear and acts on the myeloperoxidase
Microscopic Polyangiitis (MPA)
Similar presentation to granulomatosis with polyangiitis but without nasopharyngeal involvement ONLY the kidneys and lungs also NO granulomas NO C-ANCA
ANCA POSITIVE
What presents with?
Typically aged 30-40
Adult onset asthma
Asthma, sinusitis, skin nodules or purpura, peripheral neuropathy (e.g wrist/foot drop)
Can also involve the heart, GI, kidney (pauci-immune glomerulonephritis)
Often mistake asthma or allergies
Granulomatous necrotizing vasculitis with eosinophilia
MPO-ANCA/p-ANCA, high IGE level
Eosinophilic Granulomatosis with Polyangiitis(EGPA) Churg-Strauss syndrome
ANCA POSITIVE
