4th Year Rheumatology Lecture The systemic rheumatic diseases Vasculitis - PowerPoint PPT Presentation

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4th Year Rheumatology Lecture The systemic rheumatic diseases Vasculitis

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4th Year Rheumatology. Lecture. The systemic rheumatic diseases. Vasculitis. Aims and objectives ... A group of conditions characterised by: ... – PowerPoint PPT presentation

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Title: 4th Year Rheumatology Lecture The systemic rheumatic diseases Vasculitis


1
4th Year RheumatologyLecture The systemic
rheumatic diseasesVasculitis
2
Aims and objectives
  • Overview of the systemic vasculitides
  • 1. Classification
  • 2. Epidemiology
  • 3. Clinical features
  • 4. Investigations
  • 5. Therapy
  • 6. Prognosis.

3
Definitions of vasculitis
  • A group of conditions characterised by
  • 1. Inflammatory cell infiltrate and necrosis of
    the blood vessel wall with impairment of blood
    flow and sometimes damage to vessel integrity
  • 2. Present usually as multisystem diseases
  • 3. Symptoms depend on the size and site of blood
    vessel involved
  • 4. Remitting and relapsing course

4
One approach to classification
  • Primary vasculitis
  • Large artery Takayasus, Giant cell arteritis
  • Medium artery Polyarteritis nodosa, Kawasaki
  • Medium/small artery Wegeners granulomatosis,
  • Churg-Strauss, Behcets
  • Microscopic polyangiitis,
  • Small vessel Henoch- Schonlein purpura,
    Leucocytoclastic vasculitis
  • Secondary vasculitis
  • Connective tissue disease RA, SLE, Sjogrens
  • Infection, drugs, malignancy essential to exclude

5
These are rare diseases - epidemiology
  • Incidence
  • overall 43 / 106
  • Systemic rheumatoid vasculitis 12.5 /106
  • Wegeners granulomatosus 8.5 /106
  • Microscopic polyangiitis 2.4 /106
  • Polyarteritis nodosa 2.4 /106
  • Age 60 years (16-82)
  • Sex male female 1.31

6
History Multisystem disease
  • Onset may be acute or grumbling
  • Systemic upset
  • fever, anorexia, weight loss
  • Skin
  • vasculitic rash
  • digital infarction/gangrene

7
  • Eyes red /- pain /- photophobia
  • ENT epistaxis, crusting, sinusitis, hoarse voice

8
Vasculitis history continued
  • CVS/RS
  • pain, SOB, haemoptysis, asthma
  • GI
  • abdominal pain, diarrhoea /- blood loss
  • NS
  • numbness, weakness

9
Musculoskeletal history
  • arthralgia (pain /- tenderness/swelling)
  • myalgia (pain)
  • loss of muscle bulk /- weakness

10
Clinical signs in vasculitic diseases
  • General features
  • Pyrexia
  • Muscle wasting

11
Inflammatory arthritis- often asymmetrical,
oligoarthritis
12
Skin signs
  • purpuric rash nodules
  • digital infarction ulceration or
    gangrene

13
Mucous membranes
  • Oro-genital ulcers
  • mouth ulcers
  • nasal ulcers
  • genital ulcers
  • Red eyes
  • uveitis

14
Uveitis with hypopyon
15
  • Episcleritis
  • Scleritis
  • scleromalacia

16
ENTsigns Nasal collapse
Stridor from laryngeal disease
17
RS/CVS signshaemoptysiscrackleswheezeper
icarditis
18
Vasculitis neurological signs include
  • peripheral neuropathy- glove and stocking
  • mononeuropathy
  • mononeuritis multiplex

19
Neurological signs
  • Neuropathy
  • vasculitic (motor-sensory)
  • peripheral entrapment
  • sensory neuropathy
  • Cervical spine involvement in RA
  • subluxation of cervical vertebrae
  • cervical myelopathy
  • local pressure on nerve roots

20
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21
Abdominal signs
  • Tenderness eg liver, gall bladder
  • intestinal (obstruction)
  • peritonitis (local, generalised)

22
Some examples of primary vasculitis
23
Investigations to consider in a patient with
suspected vasculitis
  • Urinalysis
  • Renal function
  • urea, creatinine,
  • creatinine clearance
  • 24 hr protein excretion
  • Other biochemistry
  • LFT CRP
  • Haematology
  • FBC, eosinophils ESR
  • Immunology
  • Anti-neutrophil cytoplasmic antibody (ANCA)
  • RhF ANA

24
Investigations II
  • Microbiology
  • blood, sputum culture
  • MSU, stool culture
  • virology Hep B/C
  • Radiology
  • CXR, sinus XR
  • CT scan, MRI, Angiography
  • 2D Echo
  • (to look for SBE/myxoma)
  • Biopsy (often needed to confirm diagnosis)
  • skin, nose, lung, kidney, rectum, nerve, muscle,

25
A little bit on ANCA testing
  • IgG ANCA detected using indirect IF or ELISA
  • ethanol fixed neutrophils express intracellular
    granules on cell surface
  • ANCA against proteinase 3 (weak cationic) has a
    cytoplasmic or cANCA pattern
  • ANCA against myeloperoxidase (strongly cationic)
    has a perinuclear or pANCA pattern
  • Other ANCA against elastase, cathepsin G,
    Azurocidin, lactoferrin also exist

26
Relevance of ANCA tests
  • Anti-neutrophil cytoplasmic antibody (ANCA) test
    has been found to be positive in
  • Vasculitis
  • Inflammatory bowel disease
  • Autoimmune liver disease SLE
  • Infections
  • Malignancies

27
ANCA in vasculitis
  • cANCA ve in
  • 90 Wegeners patients
  • may reflect disease activity
  • may predict relapse
  • pANCA ve in
  • 15 WG (MPO/elastase)
  • 60 MPA
  • 50 CSS (MPO/PR3)
  • ? 10 PAN

28
Serious complications resulting from vasculitic
diseases (preventable?)
  • Gangrene or infarction of skin or a digit
  • Nasal collapse and subglottic stenosis
  • Pulmonary haemorrhage
  • Coronary arteritis
  • Neuropathy (mononeuropathy or multifocal
    neuropathy)
  • Renal failure (acute/chronic)

29
The differential diagnosis may be wide
  • Infection chronic, atypical, SBE
  • Connective tissue disease eg SLE
  • Malignancy
  • Pseudovasculitis cholesterol emboli,
  • atrial myxoma
  • Sarcoidosis
  • HIV

30
Therapy to suppress disease activity and then to
maintain remission
  • Remission induction
  • steroid /- cyclophosphamide (continuous oral or
    pulse)
  • Maintenance therapy
  • azathioprine, methotrexate, septrin
  • Adjuvant therapy
  • IVIG, pulse iv steroid, plasma exchange
  • Relapse
  • combination of above

31
Prognosis is improved by 1. early recognition
of disease activity and 2. introduction of
appropriate therapy
  • 2 year survival no therapy 10
  • cyclophosphamide 80
  • Remission induced in 90 Wegeners patients with
    cyclophosphamide
  • Relapse occurs in 50 Wegeners but
  • lt 10 PAN
  • Prognosis good for small vessel vasculitis
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