tutorial vasculitis

Download Tutorial vasculitis

Post on 29-Aug-2014

437 views

Category:

Education

1 download

Embed Size (px)

DESCRIPTION

 

TRANSCRIPT

  • TUTORIAL PRESENTATION APPROACH TO A CASE OF VASCULITIS Moderator :Dr. Saroj Purohit
  • What is Vasculitis? Inflammation of blood vessels occlusion aneurysm ischemia hemorrhage
  • WHICH BLOOD VESSELS ARE AFFECTED? ANY BLOOD VESSEL CAN BE AFFECTED: ARTERIES ATERIOLES CAPILLARIES VENULES VEINS CAN DAMAGE VIRTUALLY ANY ORGAN OR TISSUE
  • Predominantly Small Vessel Vasculitides Immune complex mediated 1.Goodpasture's disease (anti glomerular basement membrane disease) 2.Cutaneous leukocytoclastic angiitis (hypersensitivity vasculitis) 3.Henoch-Schnlein purpura 4.Hypocomplementemic urticarial vasculitis 5.Essential cryoglobulinemia 6.Erythema elevatum diutinum ANCA-associated disorders 1.Wegener's granulomatosis 2.Microscopic polyangiitis 3.Churg-Strauss syndrome 4.Renal-limited vasculitis
  • Secondary Forms of Vasculitis Connective tissue disorders rheumatoid vasculitis, lupus erythematosus, Sjgren's syndrome, inflammatory myopathies Inflammatory bowel disease Paraneoplastic Infection Drug-induced vasculitis:
  • Pathogenesis Antigens (Microbes, drugs, tumor, autoantigens) + Antibodies Formation of immune complexes Deposition of complexes in and around blood vessels Activation of complement cascade and generation of C3 and C5a (anaphylatoxins) Mast cell degranulation and generation of chemokines and cytokines
  • cont.. Increased vascular permeability, neutrophil chemotaxis, further deposition of immune complexes Appearance of neutrophils with phagocytic activity, and release of proteolytic enzymes (e.g. collagenase and elastase) Destruction of vessels, formation of platelet thrombi Ischmia, hemorrhage, and necrosis of tissues involved Clinical signs and symptoms
  • APPROACH TO A PATIENT OF VASCULITIS
  • The vasculitic syndrome display a multitude of variable presentations. Hence there can be no uniform laid down guidelines or evaluation scale for the diagnosis of these disorders. High degree of suspicion, a detailed history,meticulous physical examination and appropriate laboratory tests to determine organ systems involved and the extent of involvement are the components of the diagnostic process.
  • When to suspect vasculitis Presence of following findings alone or in combination or other bizarre systemic manifestations should raise the suspicion of vasculitis Occlusive arterial disease or hypertension in young adults. Unexplained fever, weight loss. Unexplained proteinuria with or without casts. Splinter haemorrhages in nails cutaneous lesions - palpable purpura, erythema, subcutaneous nodules or urticaria. Sudden retinal vascular disease without hypertension or diabetes.
  • Persistent headache with sudden visual impairment (monocular blindness) in elderly. Jaw claudication Sudden appearance of peripheral neuropathy - wrist drop, foot drop. Cerebrovascular/cardiovascular events in young. Unexplained finding of pulmonary nodular/cavitatory lesions.
  • Clinical presentation Predominantly cutaneous vasculitis Usual presentation is in the form of palpable purpura, urticaria, bullous ulcers or splinter haemorrhages. Mainly limited to lower extremities. Salient features are: Absence of systemic involvement. Negative serology Small vessel leucocytoclastic vasculitis Better prognosis
  • Manifestations of Systemic vasculitis
  • Cont
  • DIAGNOSTIC WORKUP Laboratory investigations Full blood count with differential white cell count Markers of inflammation: ESR,CRP Electrolytes and hepatic transaminases, glucose Urinalysis for protein and blood Blood cultures (if pyrexial) SerologyANA ,dsDNA , ANCA,C3 and C4,ASLO titre, viral titres (e.g. hepatitis B and hepatitis C, possibly HIV, CMV, parvovirus B19 and others if recent infection). Others-rheumatoid factor, electrophoresis, immune complexes.
  • Skin biopsy Organ biopsy Angiography Other radiological investigations X-ray, CT scanning and MRI 2D Echocardiography Renal and hepatic function tests
  • Mimicks of vasculitis
  • Clinical finding according to vessel involvement
  • LARGE VESSEL VASCULITIS
  • GIANT CELL ARTERITIS (Temporal or Cranial Arteritis) Transmural inflammation> intimal hyperplasia-> luminal occlusion Fragmentation of internal elastic lamina Cellular immune response involving T-cells, APCs, macrophages Symptoms are due to end-organ ischemia
  • GIANT CELL ARTERITIS Presentation Temporal Headache Scalp tenderness Thickened temporal arteries Jaw claudication Acute visual loss Weight loss, anorexia, fever, night sweats, malaise & depression
  • Laboratory Findings Erythrocyte sedimentation rate > 50 22% of patients with biopsy-proven GCA have normal ESR Therefore, normal ESR does NOT rule out GCA Mild-moderate anemia of chronic disease Deranged LFT(1/3) CRP- Raised
  • GIANT CELL ARTERITIS Histopathology Granulomatous cell infiltration Giant cells Disruption of internal elastic lamina Proliferation of intima Occlusion of lumen
  • Treatment Goal: Reduce the symptoms and to prevent visual loss If clinical suspicion is high, treatment should NOT be delayed for biopsy Glucocorticoids Methotrexate Infliximab Azathioprine Imatinib mesylate Surgery
  • MEDIUM VESSEL VASCULITIS
  • Polyarteritis nodosa Inflammatory necrotizing vasculitis Tends to occur at bifurcations & branchings of small and medium sized muscular arteries but not venules Involvement of the renal and visceral arteries is characteristic involvement of the arterioles of the renal glomeruli or the pulmonary arteries does not occur but can involve bronchial arteries Mediated by deposition of circulating immune complexes Age 40-60 yrs Association--hepatitis B ,Hairy cell leukemia fibrinoid necrosis occlusion of the vessel lumen, thrombosis, and ischemia of the tissue supplied by the vessel. Produces aneurysmal dilatation ( upto 1 cm ) of the artery
  • ACR classification Otherwise unexplained weight loss >4 kg Livedo reticularis Testicular pain or tenderness Myalgias (excluding that of the shoulder and hip girdle), weakness, or polyneuropathy Mononeuropathy or polyneuropathy New onset diastolic blood pressure >90 mmHg Elevated levels of serum blood urea nitrogen (>40 mg/dL) or creatinine (>1.5 mg/dL) Evidence of hepatitis B virus infection via serum antibody or antigen serology Characteristic arteriographic abnormalities not resulting from noninflammatory disease processes A biopsy of medium- or small-sized artery containing polymorphonuclear cells 3/10 sensitivity/specificity-82-87%
  • TREATMENT Less severe cases - glucocorticoids Severe cases - combination of prednisone and cyclophosphamide PAN with hepatitis B - antiviral therapy in combination with glucocorticoids and plasma exchange.
  • SMALL VESSEL VASCULITIS
  • Cutaneous small vessel vasculitis SYN : Cutaneous leukocytoclastic vasculitis Hypersensitivity angiitis/vasculitis Cutaneous necrotizing venulitis. Affect mainly cutaneous post-capillary venules, cutaneous small vessel vasculitis (CSVV) is the most common type of vasculitis in dermatology. 50% of cases idiopathic 1520% - infection 1520% - inflammatory diseases (collagen vascular disorders) 1015% - medications 5% - malignancy.
  • Histopathology. swelling of endothelium fibrinoid necrosis of vessel walls Extravasation of erythrocytes Infiltrate of neutrophils with karyorrhexis of nuclei(i.e. leukocytoclasia)
  • Clinical features The major cutaneous manifestation of CSVV is palpable purpura, ranging in size from 1 mm to several centimetres. Purpura may progress to papules, nodules, vesicles, plaques,bullae or pustules. Other cutaneous findings include oedema, livedo reticularis and urticaria. Lesions typically occur in areas prone to stasis (ankles and lower legs). Typically spare intertriginous regions. Usually asymptomatic,pruritus,pain or burning may b