Vasculitis is a group of disorders that destroy by inflammation.
Signs and symptoms
The clinical presentation of the various vasculitides on the skin and internal organs is mostly determined by the diameter or size of the vessels mainly affected.
Non-specific symptoms are common and include
fever,
headache,
fatigue,
myalgia,
weight loss, and
arthralgia.
All forms of vasculitides, even large vessel vasculitides, may cause skin manifestations. The most common skin manifestations include purpura, nodules, livedo reticularis, skin ulcers, and purpuric Hives.
|
| Giant cell arteritis | Headache, scalp tenderness, jaw claudication, and blindness. |
|
| Kawasaki disease | Fever, conjunctivitis, , palmoplantar erythema, cervical lymphadenopathy, and mucosal enanthema. |
|
| Granulomatosis with polyangiitis | Crusting rhinorrhea, sinusitis, chronic otitis media, Nasal congestion, shortness of breath, and chronic cough. |
| Eosinophilic granulomatosis with polyangiitis | Asthma, allergic rhinitis, sinusitis, , peripheral neuropathy, pulmonary infiltrates, and abdominal pain. |
|
| Cryoglobulinemic vasculitis | Palpable purpura, Raynaud syndrome, Arthralgia, and peripheral neuropathy. |
| IgA vasculitis | Palpable purpura, arthralgia, abdominal pain, nephritis, and Hematuria. |
| Hypocomplementemic urticarial vasculitis | Hives, arthralgia, membranoproliferative glomerulonephritis, and chronic obstructive pulmonary disease. |
|
| Cogan syndrome | Interstitial keratitis, ocular redness, vertigo, and tinnitus. |
|
| Cutaneous arteritis | Nodules, livedo reticularis, ulcers, and gangrene. |
| Primary central nervous system vasculitis | Headache, cognitive impairment, stroke, encephalopathy, and . |
| Retinal vasculitis | Visual impairments, , and macular edema. |
|
| Rheumatoid vasculitis | Purpura, focal digital lesions, ulcers, digital necrosis, pyoderma, distal sensory or motor neuropathy, and mononeuritis multiplex. |
Causes
There are several different etiologies for vasculitides. Although infections usually involve vessels as a component of more extensive tissue damage, they can also directly or indirectly cause vasculitic syndromes through immune-mediated secondary events. Simple
Thrombosis usually only affects the luminal process, but through the process of thrombus organization, it can also occasionally cause a more chronic vasculitic syndrome. The
Autoimmunity etiologies, a particular family of diseases characterized by dysregulated immune responses that produce particular pathophysiologic signs and symptoms, are more prevalent.
Classification
Primary systemic, secondary, and single-organ vasculitides are distinguished using the highest classification level in the 2012 Chapel Hill Consensus Conference nomenclature.
Primary systemic vasculitis
Primary systemic vasculitis is categorized by the size of the vessels mainly involved. Primary systemic vasculitis includes large-vessel vasculitis, medium-vessel vasculitides, small-vessel vasculitides, and variable-vessel vasculitides.
Large vessel vasculitis
The 2012 Chapel Hill Consensus Conference defines large vessel vasculitis (LVV) as a type of vasculitis that can affect any size artery. It usually affects the aorta and its major branches more frequently than other vasculitides.
Takayasu arteritis (TA) and giant cell arteritis (GCA) are the two main forms of LVV.
Medium vessel vasculitis
Medium vessel vasculitis (MVV) is a type of vasculitis that mostly affects the medium arteries, which are the major arteries that supply the viscera and their branches. Any size artery could be impacted, though.
The two primary types are polyarteritis nodosa (PAN) and
Kawasaki disease (KD).
Small vessel vasculitis
Small vessel vasculitis (SVV) is separated into immune complex SVV and antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a necrotizing vasculitis linked to MPO-ANCA or PR3-ANCA that primarily affects small vessels and has few or no immune deposits. AAV is further classified as eosinophilic granulomatosis with polyangiitis (EGPA), granulomatosis with polyangiitis (GPA), and microscopic polyangiitis (MPA).
Immune complex small vessel vasculitis (SVV) is a vasculitis that primarily affects small vessels and has moderate to significant immunoglobulin and complement component deposits on the vessel wall.
Variable vessel vasculitis
Variable vessel vasculitis (VVV) is a kind of vasculitis that may impact vessels of all sizes (small, medium, and large) and any type (arteries, veins, and capillaries), with no particular type of vessel being predominantly affected.
This category includes Behcet's disease (BD) and
Cogan syndrome (CS).
Secondary vasculitis
The subset of illnesses known as secondary vasculitides is believed to be triggered by an underlying ailment or exposure. Systemic illnesses (such as rheumatoid arthritis), cancer, drug exposure, and infection are the primary causes of vasculitis; however, there are still a few factors that have a conclusively shown pathogenic relationship to the condition.
Vasculitis frequently coexists with infections, and several infections, including
hepatitis B and
Hepatitis C,
HIV, infective endocarditis, and
tuberculosis, are significant secondary causes of vasculitis.
Except for rheumatoid vasculitis, the majority of secondary vasculitis forms are exceedingly rare.
Single-organ vasculitis
Single-organ vasculitis, formerly known as "localized", "limited", "isolated", or "nonsystemic" vasculitis, refers to vasculitis that is limited to one organ or organ system. Examples of this type of vasculitis include gastrointestinal, cutaneous, and peripheral nerve vasculitides.
Diagnosis
-
Laboratory tests of blood or body fluids are performed for patients with active vasculitis. Their results will generally show signs of inflammation in the body, such as increased erythrocyte sedimentation rate (ESR), elevated C-reactive protein (CRP), anemia, leukocytosis and eosinophilia. Other possible findings are elevated antineutrophil cytoplasmic antibody (ANCA) levels and hematuria.
-
Other organ functional tests may be abnormal. Specific abnormalities depend on the degree of organ involvement. A brain SPECT can show decreased blood flow to the brain and brain damage.
-
The definite diagnosis of vasculitis is established after a biopsy of involved organ or tissue, such as skin, sinuses, lung, nerve, brain, and kidney. The biopsy elucidates the pattern of blood vessel inflammation.
- *Some types of vasculitides display leukocytoclasis, which is vascular damage caused by nuclear debris from infiltrating neutrophils.
[ Updated: 25 October 2018] It typically presents as palpable purpura.[ Conditions with leucocytoclasis mainly include hypersensitivity vasculitis (also called leukocytoclastic vasculitis) and cutaneous small-vessel vasculitis (also called cutaneous leukocytoclastic angiitis).]
-
An alternative to biopsy can be an angiogram (x-ray test of the blood vessels). It can demonstrate characteristic patterns of inflammation in affected blood vessels.
-
18F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT)has become a widely used imaging tool in patients with suspected Large Vessel Vasculitis, due to the enhanced glucose metabolism of inflamed vessel walls.
-
Acute onset of vasculitis-like symptoms in small children or babies may instead be the life-threatening purpura fulminans, usually associated with severe infection.
+ Laboratory Investigation of Vasculitic Syndromes |
|
| Leukopenia, thrombocytopenia, Coombs' test, complement activation: low serum concentrations of C3 and C4, positive immunofluorescence using Crithidia luciliae as substrate, antiphospholipid antibodies (i.e. anticardiolipin, lupus anticoagulant, false-positive VDRL) |
| |
| |
| Elevated CRP |
| Elevated CRP |
| Elevated CRP and eosinophilia |
| |
| Cryoglobulins, rheumatoid factor, complement components, hepatitis C |
| |
| Elevated CRP and eosinophilia |
| Elevated CRP and ESR |
In this table: ANA = antinuclear antibodies, CRP = C-reactive protein, ESR = erythrocyte sedimentation rate, dsDNA = double-stranded DNA, ENA = extractable nuclear antigens, RNP = ribonucleoproteins; VDRL = Venereal Disease Research Laboratory
Treatment
Treatments are generally directed toward stopping the inflammation and suppressing the immune system. Typically,
such as
prednisone are used. Additionally, other immune suppression medications, such as
cyclophosphamide, are considered. In case of an infection, antimicrobial agents including
cephalexin may be prescribed. Affected organs (such as the heart or lungs) may require specific medical treatment intended to improve their function during the active phase of the disease.
See also
External links
