Vasculitides
Dr.Avinash Buche
Introduction:
In our body, there are various types of blood vessels. Arteries, arterioles, veins, venules and capillaries are the names of different blood vessels. Each type of vessel has different function to carry out. Arteries and Veins are further classified according to their internal diameter into large, medium and small sized vessels.Presence of Leukocytes (White blood cells) in the blood vessel wall and resulting vessel damage is called as vasculitis. In other words, vasculitis is inflammation of blood vessel wall. These White blood cells (WBCs) are part of the blood which is flowing through these vessels. The exact mechanism and the reason remain unknown many a times. The vasculitis is classified as primary (causes unknown) or secondary (to some viral infections like Hepatitis B and C or diseases like Lupus, Sjogren’s syndrome etc). The symptoms produced in various vasculitides are either due to bleeding because of vessel wall disruption or organ dysfunction to which the concerned vessel/vessels are supplying blood. The symptoms depend upon the size of the vessel involved and the site of the vessel.
The exact incidence of these diseases is unknown but are considered rare than common arthritis like rheumatoid arthritis.
Major categories of non-infectious vasculitis:
1) Large- vessel vasculitis- e.g. Takayasu’s arteritis and Gaint cell arteritis
2) Medium vessel vasculitis- Polyarteritis nodosa and Kawasaki disease
3) Small Vessel vasculitis- Microscopic polyangiitis, granulomatosis with Polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA; Churg- Strauss disease), anti-glomerular basement membrane (GBM) disease, cryoglobulinemic vasculitis, immunoglobulin A (IgA ) vasculitis ( Henoch- Scholein purpura [HSP] ) and hypocomplementemic urticarial vasculitis (anti c1q vasculitis)
4) Variable vessel vasculitis- Behcet syndrome and Cogan syndrome
5) Single- organ vasculitis - Primary Central nervous system vasculitis
6) Vasculitis associated with Systemic diseases such as Systemic lupus erythematosus, Rheumatoid arthritis, Sjogren’s syndrome etc
7) Vasculitis associated with probable etiology like hepatitis C associated cryoglobulinemic vasculitis, hepatitis B associated polyarteritis nodosa etc
Clinical features of vasculitis
These diseases often present with severe features and can be life threatening.
1) Constitutional symptoms like fever, unexplained weight loss, loss of appetite, fatigue
2) Clinical features due to dysfunction of specific organs Kidney- proteinuria leading to generalized body swelling, hematuria leading to anemia and fatigue, rise in blood pressure, kidney failure etc Nervous system- peripheral nerve dysfunction leading to foot drop or loss of sensations in legs or hands Brain- Paralysis of one side of the body or stroke Lungs- Hemoptysis, Cavitation in lungs, cough, breathlessness Gastrointestinal tract- pain in abdomen due to bowel ischemia Ear nose throat- perforation of the nasal septum leading to difficulty in inspiration, bleeding through nose, hoarseness of voice Heart- heart attack, palpitations
3) Ischemia of the digits leading to digital gangrene
4) Non healing ulcers over lower limbs
5) Reddish rashes over lower limb or dependent areas
6) Arthritis or arthralgia
Diagnosis:
Diagnosis of vasculitis is done by considering the history, blood investigations like complete blood count, erythrocyte sedimentation rate, C- reactive protein, Liver and kidney function tests, urine examination, Anti neutrophil cytoplasmic antibody test and Imaging findings like Ultrasonography, Computed tomography (CT) angiography, magnetic resonance (MR) angiography, Positron emission tomography (PET) scan. There are specific criteria recommended for diagnosis by various international rheumatology societies.
Certain tests are repeated at regular interval to see whether the patient is responding to the treatment or not. Also, tests are helpful to ascertain the disease activity and damage.
Treatment:
Steroids in the form of Injections or tablets are often the first line of treatment. Immunosuppressants are used to avoid the necessity of taking steroids for prolonged period. There are various options available for treatment which has improved the outcome of patients with vasculitis. Early detection and regular treatment can help the patients to lead a meaningful life.
