Vasculitis is a diverse category of inflammatory diseases of the blood vessels. These diseases range in severity from self-limited dermatological additions to acute and rapidly fatal multisystem disorders. All forms of vasculitis are characteriz by endothelial damage, intimal proliferation, thrombosis, and eventual vascular occlusion. Vasculitis can affect every organ system typically in the back in recognizable patterns based on the size of the affected vessels and pathologic mechanisms.
Patterns of symptoms and signs:
Vasculitis tends to affect 5 organ systems the most. Which are the skin, gastrointestinal system, renal system, neuro system, and muscle system.
It can also affect the lungs but it's not common. But when it does happen its involvement is usually very prominent.
- When a patient has Vasculitis that affects the small blood vessels. Then he gets a skin condition call palpable purpura. A medium vessel vasculitis results in 2 skin conditions called erythema nodosum and Livedo reticularis and large vessel vasculitis can result in cyanosis and discoloration of the extremities.
- GI involvement leads to mucositis and minor GI bleeding in small vessel vasculitis. A medium vessel vasculitis causes abdominal pain and bowel perforation. Large vessel vasculitis bowel, an infraction occurs.
- In small vessel vasculitis, the glomerulus of the kidneys is affect by Hematuria without RBC casts and Proteinuria. Medium vessel vasculitis of kidney damage is no limit to the glomerulus but also causes hematuria with RBS casts along with flank pain from Ischemia. Large vessels vasculitis hypertension is from the involvement of the aorta or renal arteries but no hematuria occurs.
- In the brain, patients can get polyneuropathy in small vessel vasculitis. Medium vessel vasculitis patients in brain can get a wide variety of mental status changes along with strokes. Large vessel vasculitis causes strokes.
- In small vessel vasculitis the muscle involvement results in myalpias which is a fancy word for muscle pain. In muscle, a medium vessel vasculitis causes myositis which differs from myalgias. The large vessel vasculitis causes claudication (which is a pain in the extremities due to lack of blood flow).
Vasculitis is much more complicated than this because no one individual vasculitis disease leads to all the findings.
Clinical features that highly suggest vasculitis :
They are a simultaneous or sequential dysfunction and individual non-contiguous peripheral nerve in a seemingly random pattern. This typically presents as a loss of sensory and/or motor function with individual nerves over days to weeks. In addition to vasculitis, multi-plex includes diabetes and HIV.
Livedo reticularis which is a lace-like reddish discoloration of the skin. And it is frequently seen in medium and occasionally in small vessel vasculitis. It is an idiopathic form see in a young women.
Finally is palpable purpura which is the development of numerous raise non blanching purplish lesions on the skin. So, this is most prominent in the lower legs ankles and feet.
In addition to these clinical features there are a variety of other features which are less specific. But are also commonly seen a vasculitis. They include
- Pulmonary hemorrhage
- Abdominal pain
- Abnormal urinary sediment
- A skin condition called erythema nodosum
Classification scheme of Vasculitis:
Classification is confusing and unsatisfying due to many reasons including:
A mix of primary and secondary forms, evolving understanding of the underlying pathophysiology, and varied opinions on whether classification should be based primarily on the size of the affected vessels or the underlying pathologic process.
As a consequence different sources may present slightly different classification schemes the following classification is based mainly on the most recent recommendations from the American College of Rheumatology.
The first-order division within vasculitis is based on that size would target vessels.
• Large vessel vasculitis includes:
- Takayasu’s arteritis predominantly affects the great vessels of the aortic arch and is a predilection for young Asian women.
- Giant cell arteritis is also known as temporal arteritis. Which primarily affects branches at the crowded arteries and is seen almost exclusively among the elderly.
• Medium vessel vasculitis includes:
- Polyarteritis Nodosa which is the most widely distribute within the body a vascular disease as it can affect any organ.
- Kawasaki disease affects almost solely young children. It is best for causing coronary artery aneurysms.
• Small vessel vasculitis further includes:
- An autoantibody is called (an anti-neutrophil cytoplasmic antibody ANCA).it is associate with immune complexes. So, It includes three diseases with long and frustratingly similar names.
- Granulomatosis with polyangiitis is particularly dangerous. So, it primarily affects the Kidneys, lungs, upper airway, nose and eyes.
- Eosinophilic granulomatosis with polyangiitis is characterize by a combination of asthma and sinus problems.
- Microscopic polyangiitis is clinically very similar to granulomatosis polyangiitis with the exception of the upper airway involvement and the lack of granulomas on biopsy.
Different names of Vasculitis:
Some types of vasculitis previously had different, eponym-based names for example:
- Granulomatosis with polyangiitis was known as Wegener's Granulomatosis
- Eosinophilic granulomatosis with polyangiitis was known as Churg Strauss Syndrome
- IgA vasculitis was known as Henoch-Schonlien Purpura
In addition to the types of primary vasculitis just listed some systemic diseases can trigger secondary vasculitis. For example connective tissue diseases ( rheumatoid arthritis, lupus) malignancy(lymphoma, leukemia) chronic infection(HIV, hepatitis B, and C) sarcoidosis, and Behcet’s disease.
Common Vasculitis Mimics:
There are also many diseases that can mimic vasculitis like
- Disseminated intravascular coagulation (DIC)
- Buerger's disease( thromboangiitis obliterans)
- Cholesterol Emboli Syndrome
- Anti-GBM antibody disease (Goodpasture’s disease)
- Bacterial endocarditis
General steps to diagnosing vasculitis:
- Identify a collection of clinical findings ( symptoms, signs) with either suggest a vasculitis in general or optimally, a specific vasculitis.
- One should check with routine labs (including coags, UA) and consider checking imaging studies ( e.g. CTA, MRA) and/or ANCAs.This will help in the differential diagnosis of one or two specific vascular diseases.To search for an Associated systemic illness (e.g. malignancy, infection, connective tissue disease, etc.
- Confirm diagnosis with biopsy (usually preferred) and/or angiography.
Although a person should keep in mind that each individual vasculitis may have specific treatment algorithms.
For limited cutaneous vasculitis treatment includes:
- Short course prednisone
For mild systemic vasculitis treatment includes:
Rapidly progressive vasculitis treatment includes:
- Prednisone and Cytotoxic agents ( e.g. cyclosphosphophamide)
- Cyclosphosphophamide transitions to less toxic agents( azathioprine, methotrexate) after remission is achieve.