Arteritis

Disease class: Vasculitis

Types

• AIDS arteritis, Central nervous system
• Endarteritis
• Giant cell arteritis
• Polyarteritis nodosa
• Takayasu arteritis

 This refers to inflammation of one or more arteries.

 

Retinal vasculitis

Disease class: Retinal diseases

Disease class: Vasculitis

Aortitis

Disease class: Vasculitis

This refers to inflammation of the aorta.

Endarteritis

Disease class: Arteritis

[Vascular surgery] Introduction

Vascular surgeons specialise in diseases of the vascular system, including the lymphatic system. 

 

Procedures

 

Vascular diseases

 

Diseases

• Abdominal aortic aneurysm: Can rupture and cause massive internal haemorrhage.
• Carotid artery disease: Can lead to strokes. 
• Peripheral vascular disease
• Arterial ulceration
• Venous ulceration
• Uncontrolled bleeding
• Foreign bodies in the vasculature : This problem is usually iatrogenic.
• These include catheter fragments, guidelines, IVC filters, stents, sheath fragments, and other medical devices. 
• Thromboangiitis obliterans

Cogan syndrome

Disease class: Eye diseases

Disease class: Vasculitis

The vascular system

Organ system

The arterial system

Flow chart of arterial supply 001

The venous system

Flow chart of venous drainage 001

The lymphatic system

Flow chart of lymphatic drainage 001

Aneurysm

Disease class: Vascular diseases  

Types

• aneurysm, Dissecting
• aneurysm, False 
• aneurysm, Infected 
• aneurysm, Ruptured
• Aortic aneurysm
• Coronary aneurysm
• Endoleak
• Heart aneurysm
• Iliac aneurysm
• Intracranial aneurysm
• Microaneurysm

A localised swelling or dilatation of an artery due to weakening of its wall.

The most common sites are the aorta, the arteries of the legs, the carotids and the subclavian arteries.

The aorta is the largest artery in the body and an aneurysm may develop anywhere in it. A dissecting aneurysm usually occurs in the first part of the aorta: it is the result of degeneration in the vessel’s muscular coat leading to a tear in the lining; blood then enters the wall and tracks along (dissects) the muscular coat. The aneurysm may rupture or compress the blood vessels originating from the aorta: the outcome is an infarction in the organs supplied by the affected vessel(s).

Aneurysms may also form in the arteries at the base of the brain, usually due to an inherited defect of the arterial wall.

Thromboangiitis obliterans

Disease class: Vasculitis

Also known as Buerger disease.

 

Symptoms

• Intermittent claudication 
• Pain at rest
• Increased cold sensitivity
• Ulceration

 

Signs

• Diminished peripheral pulses
• Skin changes
• Discolouration
• Red-blue discolouration
• Cyanosis
• Reduced hair growth
• Gangrenous necrosis

 

Pathophysiology 

The cause is currently unknown, but affected patients are usually current or former tobacco smokers. It is believed to be an autoimmune response.
Rickettsia infection may be involved in the pathogenesis.

 

Risk factors

• Tobacco smoking 

 

Epidemiology

• Typically between 20–40 years old and male. Females have also been diagnosed.

Complications

• Critical limb ischaemia
• Sepsis 

 

Management

• Smoking cessation reduces rate of progression and severity of outcomes.
• Hyperbaric medicine
• Vasodilators
• Sympathectomy
• Vascular bypass
• Debridement
• Excision
• Amputation

Anti-inflammatory and anti-coagulation therapies have not proven beneficial.

Diabetic vasculopathy

Diabetes mellitus

This refers to vascular damage secondary to chronic hyperglycaemia.

Complications

Microvascular

• Diabetic neuropathy
• Diabetic retinopathy
• Diabetic nephropathy
• Erectile dysfunction
• Diabetic myonecrosis  
• Diabetic cardiomyopathy 

Macrovascular

• Peripheral arterial disease
• Cerebrovascular accident
• Coronary artery disease
• Angina 
• Myocardial infarction

 

Multiple factors

• Diabetic foot syndrome

Deep vein thrombosis

Critical limb ischaemia

Causes

• Peripheral arterial disease

Intermittent claudication

Definition

Claudication is a cramp-like pain which occurs in the legs while walking. It may cause the sufferer to limp or stop them from walking entirely.
Intermittent claudication occurs when a person has to stop every so often to let the pain to subside. This is caused by the build-up of waste products in the muscles.

Source: Black's Medical Dictionary (42nd ed, 2010, ISBN 978-1-4081-0419-4)

 

Causes

• Peripheral arterial disease
• Thromboangiitis obliterans

Carotid artery stenosis

Giant-cell arteritis

Systemic vasculitis

GCA usually causes patients to present with headache. The patients are usually in their 50s or older. On questioning they classically show jaw claudication, tongue claudication, scalp tenderness, and a palpable, pulsatile, thickened temporal artery. In reality, few physicians find a palpable temporal artery in patients with this disease.

Patients with probable giant cell arteritis should be treated empirically with high dose steroids to reduce any inflammation. This is because giant cell arteritis has a high risk of causing a blood clot in the temporal artery which can obstruct the opthalmic artery and cause permanent blindness in the affected eye. This can subsequently develop in the other eye in a matter of days.

To confirm the diagnosis, a blood test should be taken to measure ESR. A temporal artery biopsy is necessary to confirm GCA, but the biopsy result is often a false negative, due to skip lesions in the artery.

Vascular dementia

Disease class: Cerebrovascular disorders

Disease class: Dementia

Disease class: Leukoencephalopathies

VD is the second most common cause of dementia. 

 

Pathophysiology

This is dementia caused by a series of small infarctions in the brain (strokes). Each infarction kills some neurons. With enough infarctions throughout the brain, the neuronal loss accumulates.

Symptoms

These patients often present with impaired cognition, impaired memory, and personality changes.

Prognosis

There may be a history of strokes (cerebrovascular infarctions). The disease progression is classically step-by-step, with dramatic deteriorations after each new infarction. It can appear to be a gradual decline in patients with very frequent, very small brain infarctions. It may develop over months or decades, but the changes are irreversible. 

Hyperlipidaemia

Management

• Statins

Peripheral vascular diseases

Disease class: Vascular diseases

Also known as PVD.

 

Types

• Blue toe syndrome
• Erythromelalgia
• Livedo reticularis
• May-Thurner syndrome
• Peripheral arterial disease
• Phlebitis
• Raynaud disease

Systemic vasculitis

Disease class: Vasculitis

Vasculitis, the inflammation of a blood vessel, can be a local response to common pathological processes such as infections.

However, there are a variety of diseases called systemic vasculitides, which involve vasculitis throughout the entire circulatory system. Systemic vasculitis can cause a wide range of signs and symptoms. It is often destructive and life-threatening.

 

 Examples

• Large vessel vasculitis
• Giant cell arteritis (GCA) 
• Takayasu's arteritis (TA)

• Medium vessel vasculitis
• Polyarteritis nodosa (PAN)
• Mucocutaneous lymph node syndrome (Kawasaki disease) (KD)

• Small vessel vasculitis
• Anti-glomerular basement membrane disease (Goodpasture's syndrome)
• Granulomatosis with polyangiitis (Wegener's granulomatosis)
• Eosinophilic granulomatosis with polyangiitis (Churg Strauss Syndrome) 
• Variable vessel vasculitis
• Behçet's disease (BD)

 

Classification

In 1994, the first international Chapel Hill Consensus Conference on the Nomenclature of Systemic Vasculitides proposed a classification system for vasculitis.
In 2012, a second conference developed a revised classification system.

Classification of systemic vasculitides according to CHCC 2012

Large vessel vasculitis

These disease may present as occlusion of large vessels, for example, stroke (cerebrovascular infarct), heart attack (cardiovascular infarct), limb claudication, gangrene.

• Giant cell arteritis (GCA) 
• Takayasu's arteritis (TA)

Medium vessel vasculitis

A large number of medium vessels supply the gut, nerves and kidneys.

• Polyarteritis nodosa (PAN) 
• Mucocutaneous lymph node syndrome (Kawasaki disease) (KD)

Small vessel vasculitis

Small vessels perfuse the majority of tissues in the body. These often present as disturbance in microvascular function, for example renal failure (kidneys) or asymmetric neuropathy (nerves).

• Antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV) 
• Microscopic polyangiitis (MPA)
• Granulomatosis with polyangiitis (GPA) (Wegener’s granulomatosis)
• Eosinophilic granulomatosis with polyangiitis (EGPA) (Churg-Strauss syndrome)
• Immune complex SVV
• Anti-glomerular basement membrane disease (Anti-GBM) (Goodpasture's syndrome)
• Cryoglobulinemic vasculitis (CV)
• IgA vasculitis (Henoch-Schönlein) (IgAV)
• Hypocomplementemic urticarial vasculitis (HUV) (anti-C1q vasculitis)

Variable vessel vasculitis

• Behçet's disease (BD)
• Cogan’s syndrome (CS)

Single-organ vasculitis

• Cutaneous leukocytoclastic angiitis 
• Cutaneous arteritis
• Primary central nervous system vasculitis 
• Isolated aortitis

Vasculitis associated with systemic disease

• Lupus vasculitis
• Rheumatoid vasculitis
• Sarcoid vasculitis
• Others

Vasculitis associated with probable etiology

• Hepatitis C virus–associated cryoglobulinemic vasculitis
• Hepatitis B virus–associated vasculitis
• Syphilis-associated aortitis
• Drug-associated immune complex vasculitis
• Drug-associated ANCA-associated vasculitis
• Cancer-associated vasculitis
• Others

 

Investigations

Initial immunology screening

• ANA
• ANCA
• C3, C4
• CRP
• RF
• Immunoglobulins and Cryoglobulins 

Mucocutaneous lymph node syndrome

Disease class: Vasculitis

Also known as

• Kawasaki disease

 

Pathophysiology

• Kawasaki disease (KD) often causes irritation and ulceration at mucosal surfaces.
• In 20-40% of patients, the coronary arteries are affecting, potentially causing chest pain and breathlessness.

 

Symptoms

• These patients often present with skin rashes, lymphadenopathy, tongue swelling, desquamation of the palms and soles, lip swelling, conjunctival injection (prominent vessels in the sclera of the eyes), gastrointestinal symptoms.

 

Epidemiology

• This disease usually occurs in young children (<5 years old). 

 

See also

• Systemic vasculitis