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Vasculitis -an approach
1. Vasculitis is characterized by inflammation of blood vessels that can lead to vessel occlusion, rupture, or thrombosis causing tissue ischemia. It often presents with non-specific symptoms and can affect multiple organs. 2. Diagnosing vasculitis requires considering mimickers, underlying causes, extent of involvement, and determining the specific type. The size and location of affected vessels impacts clinical features. 3. Treatment involves immunosuppressants like steroids, immunomodulators, and biologicals depending on severity and organs involved. Prognosis depends on extent of organ damage but outcomes have improved with aggressive therapy.
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Vasculitis -an approach
- 1. VASCULITIS A PHYSICIAN’S PERSPECTIVE DrA P Naveen Kumar DNB FICP Chief Specialist – General Medicine Visakha Steel General Hospital
- 2. • 54 Male •Fever joint pains loss of weight – 2 months • Severe pain in the tips of fingers and toes • Tingling in lower limbs rt > lt • Developed ulcers in his mouth • Anemic TC 4200 ESR 110 • LFT RFT -N
- 4. • CTD • Solumedrol •ANA Negative • SLE Antibodies negative • ANCA positive • Azathioprine • Rituximab
- 5. INTRODUCTION • Vasculitis isa heterogenous group of disorders characterised by inflammatory destruction of blood vessels • Inflammed blood vessels are liable to occlude, rupture or develop a thrombus leading to ischemia of tissues and organs supplied by the vessel • Because of multisystem involvement , it begins with non specific symptoms and has a tendency to affect multi organs • Involves immunological mechanisms and can result in variety of presentations depending upon the type and size of the vessel involved
- 7. GENERAL DIAGNOSTIC APPROACH Fiveimportant questions to ask 1.Is this a condition that could mimic the presentation of vasculitis 2. Is there a secondary underlying cause 3. What is the extent of vasculitis 4. How do I confirm the diagnosis 5. What specific type of vasculitis is this
- 8. 2012 Revised ChapelHill Nomenclature
- 10. DRUGS • Pencillin • Cephalosporin •Sulfonamide • Thiazides • Phenytoin • Allopurinol
- 11. Systemic inflammatory response Resultsfrom release of chemical mediators from inflammed vessels • Fever • Night sweats and weight loss • Arthralgia and myalgia • Lab - Normocytic normochromic anemia , Leucocytosis , Thrombocytosis ,raised ESR and CRP Conversely systemic systems are not seen in localised forms of vasculitis
- 12. Large vessels Aorta andits Major branches Medium vessels Visceral Arteries Muscular Arteries Small vessels Intraparenchymal Arteries Arterioles Capillaries Limb claudication Ulcers Purpura Assymetric blood pressure Livedo reticularis Urticaria Bruits Digital gangrene Glomerulonephritis Aortic dialatation Mononeuritis multiplex Alveolar hemorrhage Renovascular Hypertension Microaneurysms Splinter hemorrhages Absence of pulses Uvietis Episcleritis Scleritis
- 14. • 44 male •PUO • Only cervical lymph nodes • CT chest and abdomen normal • Leukocytosis • Increased ESR
- 15.
- 16. TAKAYASU ARTERITIS • 22male presented with high BP • Clinically he had aortic regurgitation • On evaluation found to have Renal artery stenosis • Finally it turned out to be Aortoarteritis • Renal artery stenting done and he did fine • Aortic valve replacement
- 17. • 48 Female •Presented with decreased vision in right eye • HTN • Investigated • Biochemistry – N • ANA SLE –N • MRA – N • Eye examination-AION
- 19. • Opined tor/o CTD • Inspite of negative workup gave three doses of Solumedrol • No significant improvement in vision • Followed up • Found that her BP is increasing • Right Radial artery weak pulsation
- 22. • Started MMF •Steroids and tapered off
- 23. Size of bloodvessel Blood vessel involved Clinical features Small vessel vasculitis Cutaneous post- capillary venules Palpable purpura Glomerular capillaries Hematuria, red cell casts in urine,proteinuria and decline in renal function Pulmonary capillaries Lung hemorrhage manifesting as breathlessness, hemoptysis and widespread alveolar shadowing on chest radiograph Medium vessel vasculitis –small and medium sized arteries Small cutaneous arteries Necrotic lesions and ulcers , nail fold infarcts Epineural arteries Mononeuritis multiplex Mesentric artery Abdominal pain, GIT bleeding and perforation because of gut infarction Branches of coeliac artery Infarction of liver ,spleen or pancreas
- 24. Size of blood vessel Bloodvessel involved Clinical features Renal artery Renal infarction Coronary artery MI or Angina ,coronary artery aneursym,ischemic cardiomyopathy Small pulmonary arteries Necrotic lesions leading to cavitating lung shadows on chest radiograph Small arteries in ear, nose and throat region Nasal crusting , epistaxis , sinusitis ,deafness , stridor because of sub glotic stenosis Large vessel vasculitis Aorta and branches Extra cranial branches of carotid Temporal headache ( temporal artery ), Blindness ( ophthalmic artery ), Jaw claudication ( vessels supplying muscles of mastication ) Thoracic aorta and its branches Limb claudication , absent pulses and unequal blood pressure, bruits , thoracic aortic aneursyms
- 26. Vasculitic mimickers • Infections SBE Meningitis-Neisseria Viral infections • Malignancies • CTD SLE APLA RA • Occlusive vascular disease Atrial myxoma Cholesterol embolism Mycotic infections Ergotism
- 27. • 44 female •Severe pain in neck and pain and numbness in right tips of all fingers • Relentless pain and more in the night • Right radial pulse weak • No deficit
- 29. • 56 Female •Recurrent URTI • Malaise weakness loss of weight • Breathlessness • Pansinusitis – took treatment
- 31. • Underwent nasalendoscopy • Biopsy s/o granulomas • CECT Chest done • Shows ILD like picture • ANCA strongly positive WEGENER’S GRANULOMATOSIS – GRANULOMATOSIS with POLYANGITIS GPA • Solumedrol • Pulse cyclophosphamide
- 33. • 58 femaleT2DM • Recurrent attacks of fever • Treated with antibiotics recovering • Cough ,vomitings and loss of weight • Examination - right axillary lymph node • FNAC – non specific lymphadenitis • Leucocytosis , ESR 75 , increased transaminases
- 34. • Mammography –normal •CT Chest – multiple lymph nodes in axilla, lungs –normal, bulky thyroid • Bone marrow study –megakaryocytic hyperplasia ,no malignancy
- 35.
- 36. Lymph node excisionbiopsy
- 37. SLE • ANA –Positive • DsDNA – 199 • Smith positive • C3 and C4 decreased • Started on steroids • Azathioprine added • Doing well
- 38. 46 Female diabetic Presentedwith peripheral neuropathy Joint pains swelling of both ankles Rash over the legs Malaise and low grade fever NCV – bilateral sensory axonal neuropathy Rapid onset of symptoms
- 39.
- 41. • PUO • Swellingof eyes lips and oral ulcers • Bilateral cervical lymphadenopathy • Multiple subcutaneous nodules • Hepatosplenomegaly • Leucopenia and thrombocytopenia • HTG increased Ferritin • Hemophagocytosis
- 42.
- 46.
- 48. MANAGEMENT • Steroids • Immunomodulants •Biologicals
- 49. SUMMARY • A comprehensiveapproach is needed for diagnosis • Prognosis and therapy depend upon on the extent of organ involvement • High degree of suspicion is necessary • Cutaneous involvement is a universal finding • Mortality has been reduced with aggressive therapy however morbidity is still high
Editor's Notes
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