Blood Vessels Flashcards
50 year old woman has fever associated with headache and weight loss. She has no other symptoms other than facial pain which runs along the course of superficial temporal artery as it is painful on palpating. Another patient with similar symptoms developed Diplopia progressing to complete loss of vision. She was treated with corticosteroids and anti TNF antibodies. What’s her diagnosis, it’s pathogenesis and morphological features.
Giant cell (temporal) arthritis
- T cell mediated vasculitis of large vessel and small arteries in head like
Vertebral, ophthalmic, aorta - MHC TYPE 2 haplotypes and TNF and sometimes anti EC antibodies involved
- notoriously patchy
- nodules intimacy thickening of vessel
- granulomatous inflammation
- fragments of internal elastic lamina
What is pulseless disease? List its salient features.
Takayasu arteritis
- marked weakening of pulses in the upper extremities
-older than 50 years, said to have giant cell aortitis and younger than 50 Takayasu aortitis
- Associated with Japanese ethnicity and HLA haplotype
A 40 year old man presented with initial fatigue, weight, loss and fever which progressed to neurological deficit or killer disturbances such as haemorrhage of retina and weak upper extremity pulse. The attending physician suspects prognosis to total blindness, leg, claudication, pulmonary artery involvement, leading to pulmonary hypertension and possible myocardial infarction. What is your diagnosis? Mention the pathogenesis and morphology seen in the disease.
Takayasu arteritis since age is less than 50.
Pathogenesis is autoimmune related to HLA, haplotype and prominent in Japanese.
Morphology seen classically affects aortic, arch and arch vessels
Can also affect pulmonary and renal arteries
-Adventitia mononuclear infiltrate
-vasa vasorum perivascular cuffing
-granulomatous inflammation with giant cell and patchy medial necrosis
-Irregular thickening of vessel wall
- intimal hyperplasia
— advantageous fibrosis
A 20-year-old man presents with malaise, fever, weight loss accompanied by diffuse, muscular aches, abdominal pain, bloody stools and peripheral neuritis of motor nerve. Patient has history of hepatitis B virus infection and has been having such episodes of illness recurrently. On examination rapidly, accelerating hypertension is seen which is suspected due to renal involvement. Pulmonary circulation is suspected to be normal. What is your diagnosis, pathogenesis, morphology?
Polyarteritis Nodosa or PAN
Pathogenesis is by immune complex disposition of hepatitis B antigen.
Morphology
- Sentimental, transmural, necrotising inflammation and occasional thrombosis
- haemorrhage, infarction, ulceration may occur
-Weak arterial wall may lead to aneurysm
- mixed infiltrate of neutrophils and monocytes
- fibrinoid necrosis
- fibrous thickening of wall
- all stages can be seen together
A three year old girl presents with conjunctival and oral erythema associated with blistering oedema of hands and feet along with redness of palm and sole. She also has cervical lymph node enlargement and desquamating rash. She has a history of viral infection recently, after which these symptoms started since four days .Diagnosis morphology pathogenesis and prognosis?
Kawasaki disease or mucocutaneous, lymph-node syndrome.
Pathogenesis - anti EC antibodies act due to delayed hyper sensitivity against cross reactive antigens
Morphology- resembles PAN but transmural necrosis is less severe with dense, infiltrate and less dense fibrinoid necrosis.
Prognosis - aneurysms and myocardial infarction might precipitate if not treated
Write a short note on microscopic polyangiitis
Necrotising vasculitis of small venues and capillaries.
-Aka hypersensitivity vasculitis, leukocytes vasculitis,
-Seen in HENOCH SCHÖLEN PURPURA, ESSENTIAL MIXED CRYOGLOBULINEMIA, SLE.
Pathogen
- immune complex and ANCA associated
- MPO ANCA m/c
clinical features
Hemoptysis, Haematuria, proteinuria, abdominal pain and bleeding, muscle pain, weakness, palpable cutaneous purpura
Morphology
- segmental fibrinoid necrosis of media
- Focal transmural necrotising lesions
- no macroscopic infarcts and no granulomatous inflammation
- show little to no ab ( pauci immune )
A middle-aged man typically presented with bilateral pneumonitis, where is nodules and cavitary lesions, chronic sinusitis, mucosal, ulceration of nasopharynx, and rapidly progressing renal disease.
Wegener’s granulomatosis also known as granulomatosis with polyangiitis characterised by
1. Necrotising granuloma in respiratory tract
2. Necrotising granulomatous vasculitis of lungs
3. Focal necrotising or cresentic glomerulonephritis
Pathogen
- cell mediated hypersensitivity to exogenous antigens
- PR3 ANCA
Morpho
- radiographically visible nodules with central cavitation of granulomatous vasculitis
- cresentic GN renal lesions
- urti lesions
Patient presented with asthma, allergic rhinitis, palpable purpura, bleeding from GIT and nephrotic syndrome. Investigations show lung infiltrates, extravascular necrotising granuloma, striking infiltration of vessels by eosinophils. Diagnosis.
Churg Strauss syndrome
Pathogen - MPO ANCA hyperresponsiveness
Cardiac involvement seen
What is Buerger disease? Is it related to smokers?
Thrombosngiitis obliterans
Affects the tibial and radial arteries mainly. Seen in heavy tobacco smokers, median age before 35 years.
Pathogenesis- ANCA associated with direct endothelial cell toxicity. HLA association.
Morpho- sharply segmental acute and chronic transmural vasculitis of medium and small arteries.
- luminal thrombosis
- small microabscesses
- artery eventually encased in fibrous tissue
- Leukocytic infiltrate
Clinical features
- raynaud’s phenomenon
- instep claudication
- vascular insufficiency and severe pain
- frank gangrene( chronic)
Characteristic features of raynaud’s phenomenon
Paroxysmal pallor, cyanosis involving digits, red white blue colour changes
Brief about raynaud’s phenomenon
Can be primary or secondary
In chronic cases of primary may lead to atrophy of skin and subcutaneous tissue and muscle
Features demonstrate central vasoconstriction and proximal vasodilatation
Name 4 diseases causing secondary raynaud phenomenon
Buerger disease, SLE, atherosclerosis, scleroderma
Pathogenesis and features of broken heart syndrome?
Ischemic dilated cardiomyopathy
Myocardial vessel vasospasm / prinzmetal angina——cardiac raynaud— MI
Shows
1. Contraction band necrosis
2. Microscopic foci of granulation tissue
A middle-aged man present, with persistent oedema in extremity associated with secondary, ischemic, skin changes like stasis dermatitis, and ulcerations.
Mention the risk factors for this disease and the anatomical vessels involved.
Varicose veins
Risks
1. Obesity
2. Pregnancy
3. Familial predisposition
Location, most commonly seen in superficial veins of upper and lower leg
What are the most common sites of varicosities apart from lower extremities? Name 3.
Gastro-oesophageal junction forming Eesophageal varices
Rectum forming haemorrhoids
Periumbical veins forming capute Medusa
