Vasculitis - Lecture notes week 11 - ortho / rheumatology PDF

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week 11 - ortho / rheumatology...

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Vasculitis

Definition  This is inflammation of the blood vessels  A response to an insult with many causes Primary / Secondary  Secondary causes are the most common: o Infection  Most likely with s short history  manifests through blood vessels  Examples:  Meningococcus o Inflammation of the blood vessels causes them to burst (purpura) or occlude (ischaemia/necrosis)  Streptococcal infection o Leads top erythema nodosum  Viral examples:  Hep C and B are the main ones o Underlying disease:  Cancer  Autoimmune disease  e.g. RA, SLE or IBD o Cold injury  Frost bite  local vascular injury resulting in inflammation and tissue necrosis  Cryoglobulinaemia  This is very rare  The cold causes autoantibodies to activate, attacking, clumping and destroying RBCs  This blocks and irritates the vessels  Peripheral (coldest bits) are the worst affected o Drugs  Anti-thyroid drugs (Carbimazole)  Minocyclin  consistent with lupus  Hydralazine  Penicillamine  Antibiotics (Sulphonamides / penicillin)  Anti-convulsants  Primary Vasculitis o General features for all:  Systemic inflammation  Fever  Malaise  Weight loss  Algia  myalgia / arthralgia  Night sweats  Specific depends on the size of the vessel affected  Large vessel  end organ ischaemia or infarction o E.g. Renal  infarction or renal artery stenosis which leads to hypertension due to RAAS  Medium  localised ischaemia or infarction o E.g. Renal  segmental infarcts

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Small  Organ specific inflammation o E.g. Renal  Glomerulonephritis Classification  Done dependent on vessel size  Large Vessel Vasculitis  Giant Cell arteritis (AKA Temporal/Cranial Arteritis) o Very common, especially in elderly o More common in women o Clinical features  Fatigue  Headaches  Jaw claudication  Scalp tenderness  struggle to shower etc. o Diagnosis  Very High ESR  Definitive test  Biopsy o Can affect other vessels nearby:  Ophthalmic artery / Internal Carotid  Presents with sudden painless onset of blindness  Treat immediately with steroids  Lingual  Presents with tingling in unilaterally tongue  CNS  Present with confusion, depression, sensorineural deafness and less commonly, stroke o Polymyalgia Rheumatica  This is considered a related disorder to giant cell arteritis  Common, especially in elderly and in women  Clinical presentation  Pain  Stiffness in muscles of neck and shoulder girdle, and pelvic girdle  proximal muscles  No weakness but very sore  Diagnosis  High ESR  Treat with steroids  Takayasu Arteritis / Pulseless disease / Aortic Arch syndrome o Very rare o Younger women (15-25) o Clinical presentation  Systemic features come early and may resolve by diagnosis  Fever  Malaise  Night sweats  Weight loss  Pain  Fatigue  Late features of vascular insufficiency in aorta and large tributaries:

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Bruits, absent or reduced pulses Claudication  arms, legs, spine, gut Ischaemic heart disease, heart failure, pulmonary hypertension  Headaches and amaurosis fugas (stroke isn’t as common)  BP variability  syncope, hypertension, variable BP between limbs  Diagnosis  Imaging angiography  MR angiography  CT o Very logical Medium Vessel Vasculitis  Polyarteritis Nodosa (PAN) o Clinical presentation  Fever and weight loss  both very typical but not specific  Infarction or ischaemia of organs  Kidney  Renal artery stenosis, Renal aneurysms, Hypertension  Brain  stroke  Heart  MI, Angina  Liver  Skin  gangrene, purpura, nodules  PNS  pain in distribution of a nerve, sensory/motor loss  Testis  pain or infarction  Kawasaki disease o Only in children o More common in Eastern Asia o Clinical presentation  Early features  High fever  Miserable  very important sign  Mouth o Mucositis  sore and red mouth o Strawberry tongue  Conjunctivitis  Skin o Red palms and soles of feet  go on to peel later o Typical Kawasaki rash  Forms aneurysms o Complication to prevent by early diagnosis  Common at bend of arteries  Pulsating  visible peripherally  Coronary arteries  MI  Axillary, ilia and popliteal arteries also o Progression:

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Acute phase (up to 10 days)  treatment here will save the life

Anti-Neutrophil Cytoplasmic Ab(ANCA)-Associated Small Vessel Vasculitis  Tests for these are specific and sensitive: o Cytoplasmic c-ANCA Proteinase 3(PR3)  Cytoplasm stains green due to autoantibody in neutrophil  Very specific for Wegener’s o Perinuclear p-ANCA Myeloperoxidase (MPO)  Just the nucleus membrane stains green as found in and around nucleus  Positive result:  MPA  Crescentic Glomerulonephritis  Churg Strauss  Negative result  IBD  RA  Sclerosing cholangitis  Examples: o ANCA o Microscopic Polyangitis (MPA)  Symptoms of chronic inflammation  Fever  Malaise  Fatigue  Weight loss  Affects a variety of organs  5 most common are:  Kidney glomerulonephritis  80%  Weight loss  >70%  Skin lesions  >60%  Peripheral Neuropathy, Mononeuritis multiplex  60%  Fevers  55% o Granulomatosis with Polyangitis  Wegener’s  Classically involves:  Upper Respiratory tract

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Investigations  Tests

o o Nasal mucosal inflammation o Saddle nose deformity o Septal perforation o Sinus disease  Lower respiratory tract o Tracheal and endobronchial disease  Kidneys o End stage renal disease o Eosinophilic Granulomatosis with Polyangitis  ChurgStrauss  Clinical presentation:  Common in Asthma  Eosinophilic infiltrates (transient pulmonary infiltrates)  Eosinophilia  Eosinophil rich granulomata  Peripheral Neuropathy  Pattern recognition:  Eosinophils everywhere  blood, lungs, biopsy  Asthma  this is an eosinophil-mediated disease  Peripheral neuropathy Immune Complex Small Vessel Vasculitis  IgA Vasculitis  Henoch-Schonlein Purpura o Epidemiology  Most common vasculitis  13.5/100,000 children  Occurs more often in children  Average age of 4 o Possibly has an infective trigger  influenced by many o Diagnosis  Triad of:  Palpable purpura  must have this o Extensor surfaces  distal to proximal  Abdominal pain  Arthritis o Mimics where the rash is  This is complicated by:  Renal involvement  Intersucception (donut sign) / infarction  Pain misery  Testis-pain / infarction  Cryoglobulinaemic Vasculitis  Hypocomplementeric Urticarial vasculitis  Anti-C1q vasculitis)

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For inflammation  Temperature chart  Height and weight  ESR and CRP  FBC Vessel Specific  Blood pressure of all 4 limbs  Examine vascular tree  feel for pulses and any aneurysms in axillae, groin, popliteal fossa etc.  Medium and large  angiography / MRA  Small  organ specific tests o GI  USS, MRI, endoscopy and biopsy o Renal  BP, urinalysis, U&Es, USS Perfusion scan, biopsy o Heart  echo and ECG, muscle enzymes o Liver  USS, Biopsy, LFTs o CNS  imaging and occasionally CSF o PNS  nerve conduction o Lungs  CXR, CT, pulmonary function tests, biopsy o Skin  biopsy Disease specific  ANCA Biopsy of an active lesion

Treatment  Corticosteroids or other immunosuppression...