Vasculitis

Vasculitis refers to a group of rare disorders in which the immune system attacks blood vessels (arteries, veins and/or capillaries). Patients may feel generally unwell and symptoms can involve any part of the body. The combination of typical symptoms, blood test results and either advanced imaging or a biopsy of an affected area is usually required to confirm a diagnosis. Patients may describe one of more of the following: purple coloured rash, joint pain, breathing difficulties, cough, mouth or genital ulcers, sinus congestion, vision disturbance, headache, jaw pain, new cold or painful extremities, nerve pain and/or weakness. Conditions are commonly grouped by the size of involved vessels, namely large, medium and small vessel vasculitis.

Medium & Large Vessel Vasculitis

Giant cell arteritis (GCA) and Takayasu arteritis are the two conditions which most commonly cause large vessel vasculitis. GCA, also known as temporal arteritis, is the most common form of vasculitis in older people and can lead to serious complications including sudden-onset vision loss and damage to the large blood arteries in the chest and neck if not identified and treated promptly. Patients usually experience one or more of five cardinal symptoms – namely headache, jaw pain on chewing, vision disturbance, inflammatory shoulder and hip stiffness (polymyalgia rheumatica) and night sweats/unexplained weight loss. If your GP or specialist suspects you may have GCA you will be started on treatment immediately while a diagnosis is confirmed. Dr Sammel can help to decide on an appropriate diagnostic strategy which may include a PET scan, ultrasound and/or temporal artery biopsy and a management pathway to control the condition while minimising medication side effects.

Takayasu arteritis affects younger people, usually under the age of 40. It causes inflammation of the major arteries in the chest/abdomen and neck. It has historically been known as the pulseless disease as patients or their doctors may notice an absent pulse in an arm or leg due to blood vessel blockage from untreated inflammation. Patients often feel generally unwell and may have night sweats, fevers and/or weight loss due to immune system attack on their body.

As this condition affects younger people, the diagnosis and treatment strategy must take into account other life priorities including reproductive health and minimising radiation from medical imaging tests.

Polyarteritis nodosa is a condition which primarily affects medium sized arteries including those supplying the kidney, gut, nerves and skin. This condition is commonly but not always associated with hepatitis viruses. Patients can experience symptoms related to any part of the body but commonly will experience one or more of rash, new nerve pain or numbness , abdominal pain and/or unexplained sweats and weight loss.

Medium and large vessel vasculitis conditions treated by Dr Sammel include:  

  • Giant cell arteritis
  • Takayasu arteritis
  • Polyarteritis nodosa
  • Isolated aortitis 
  • Aortitis due to other inflammatory conditions (eg sarcoidosis, IgG4) 

Small Vessel Vasculitis

Small vessel vasculitis is more common than medium and large vessel vasculitis. It can affect people of any age and has a large number of causes including medications, infections and autoimmune conditions. Depending on the cause and parts of the body affected, it can either be a minor problem and resolve on its own or be a life threatening condition requiring urgent aggressive treatment. A careful clinical assessment, along with specialised blood tests and sometimes a biopsy of the affected part of the body is needed to make a diagnosis and arrive at a management plan.

Small vessel vasculitis conditions treated by Dr Sammel include:

  • ANCA associated vasculitis
  • Granulomatosis with polyangiitis (formerly Wegner Granulomatosis)
  • Eosinophilic granulomatosis with polyangiitis (formerly Churg Strauss syndrome)
  • Microscopic polyangiitis
  • IgA / Henoch Schonlein Purpura
  • Connective tissue disease vasculitis
  • Drug induced vasculitis
  • Infective and post-infective vasculitis
  • Cryoglobulinaemic vasculitis
  • Cutaneous vasculitis

Other Forms of Vasculitis

Some conditions can result in vasculitis of many vessel sizes or may only involve localised parts of the body. These are rare diseases which require a thorough assessment of all parts of the body to help determine treatment goals and the most appropriate therapy. Some conditions (organ limited vasculitis) may require no treatment while others (eg.  IgG4-related disease, Behcet disease) may respond well to targeted medications such as B cell depletion and TNF-alpha inhibitors.

Dr Sammel has expertise in the diagnosing and treating the following:

  • Behcet and autoinflammatory vasculitis
  • IgG4 vasculitis
  • Periaortitis / idiopathic retroperitoneal fibrosis
  • Cogan Syndrome
  • Organ limited vasculitis (CNS, visceral, isolated aortitis)

Other Vascular Conditions

Dr Sammel has expertise in assessing patients with unusual vascular problems to determine if vasculitis or another condition is the cause. Making a correct diagnosis means that appropriate treatment and monitoring can be instituted.

Dr Sammel has experience with the following conditions which can mimic vasculitis:

  • Antiphospholipid syndrome and other clotting disorders
  • Atherosclerosis
  • Fibromuscular dysplasia
  • Segmental arterial mediolysis
  • Thromboangiitis obliterans (Buergers syndrome)
  • Inherited connective tissue disorders (Ehlers Danlos, Marfan, Loeys-Dietz syndrome)
  • Capillaritis
  • Livedoid vasculopathy
  • Raynaud phenomenon