Vasculitis Flashcards
When to suspect vasculitis?
You have to suspect vasculitis when a patient presents with:
- Unexplained systemic illness
- Symptoms of organ system ischaemia (multiple organ dysfunctions in a systemically ill patient should raise the possibility of vasculitis)
- Nephritic syndrome
What are mimics of vasculitis?
Mimics of vasculitis:
Infections
Embolic disorders
Malignancy
Drugs
Vasculitis mimics, frequently entitled pseudo-vasculitis or vasculitis-like syndromes, should be excluded first. Infections deserve careful attention, as they are major mimics of vasculitis and would be aggravated by medication aimed to suppress vascular inflammation. Infective endocarditis should always be considered. Although the endocardium may be the primary site of infection, it often results in multisystem manifestations, involving several organs. Bacteraemia and peripheral embolic events are common. Circulating immune complexes may lead to inflammatory responses most often affecting the skin, the kidney, and the central nervous system.
Differential diagnosis is not always simple.
An embolism from an atrial myxoma or cholesterol emboli from an atheroma are examples of embolic diseases that can mimic vasculitis. Thrombotic disorders such as antiphospholipid syndrome, thrombotic thrombocytopenic purpura and sickle cell disease can cause thrombosis and thus mimic vasculitis. Amyloid angiopathy and fibromuscular dysplasia are non-inflammatory vessel wall disorders causing ischaemic organ damage. Some conditions like end-stage renal failure and hyperparathyroidism can be associated with a livedo reticularis-like rash which can be mistaken as a sign of vasculitis.
Drugs can induce vasoconstriction and ischaemia. These include ergots, cocaine, and phenylpropanolamine. Some drugs can cause coagulopathy and, in this way, mimic vasculitis ,e.g. Warfarin.
Malignancy will often have B-symptoms and basic laboratory findings such as normocytic normochromic anaemia, lymphocytosis, thrombocytosis, elevated ESR and raised CRP. Radiological findings of multi nodular lung shadows due to metastasis can suggest GPA.
Many infections can result in secondary vasculitis; the majority are viral. Hepatitis B and C, Parvovirus B19, Human immunodeficiency virus (HIV), Cytomegalovirus (CMV), and Epstein-Barr virus (EBV) are examples of viral conditions which are known to cause secondary vasculitis. Cryoglobulins can be found in many of primary or secondary vasculitis.
Infections with Salmonella, Streptococcus pneumoniae, Clostridium septicum, Chlamydia pneumoniae and Mycobacterium tuberculosis can also result in vasculitis. Even parasites (e.g. Ascaris) and fungi (e.g. Aspergillus) have been associated with secondary vasculitis.
Malignant diseases such as solid tumours, myeloproliferative and lymphoproliferative disorders can be associated with secondary vasculitis.
Connective tissue diseases, especially SLE and primary Sjögren’s syndrome, as well as rheumatoid arthritis (RA) are known to cause secondary vasculitis. An important clue is that the vasculitis usually appears late in the disease, which makes the diagnosis easier.
Inflammatory bowel diseases can also originate secondary vasculitis.
What further work-up do you require to determine if you have a patient with primary vs. secondary vasculitis?
- Expand the history taken
- Travel history
- Drug history
- Full clinical examination
- Urine protein: creatinine ratio and urine microscopy
- Chest x-ray
- Basic blood screening (complete blood cell count, liver enzymes, CRP, ESR, creatinine)
- Electrocardiogram
Laboratory testing is only occasionally helpful in classifying vasculitis, but it is most important in excluding other diseases and determining organ involvement. Here you must have all the former conditions in mind and undertake laboratory testing based on the history and clinical examination.
You may consider:
- Blood cultures
- Hepatitis B and C screening, CMV and Parvovirus B19
- HIV test
- Relevant testing for bacteria, fungi, or parasites
- Serological tests
- ANA, anti-double stranded DNA, rheumatoid factor, ACPA, glomerular basement membrane antibody, creatine phosphokinase, complement factors (C3, C4)
- ANCA (PR3/MPO)
- Antiphospholipid antibodies & Lupus anticoagulan
- Cryoglobulins
- Echocardiogram
Histological confirmation of vasculitis should be sought in most cases by undertaking a tissue biopsy. If there is an indication of kidney involvement (proteinuria), a kidney biopsy will be preferred. Other favoured sites are skin, temporal artery, muscle, nasal mucosa, lung, sural nerve, and testis. Although pathological proof of vasculitis should be looked for, it is not mandatory for the final diagnosis. In the context of a clinical picture suggestive of GCA, a negative temporal artery biopsy does not rule out GCA. Likewise, a full-blown picture of ANCA associated vasculitis does not necessarily require pathological confirmation.
What would a MRA of the thoracic aorta potentially show in a patient with Takayasu arteritis?
MRA of the thoracic aorta may show stenosis, occlusion, or aneurysm formation in patients with large vessel vasculitis such as Takayasu´s arteritis.
What classification criteria is used for vasculitis?
Chapel Hill classification (2012). t builds on microscopic findings, but also considers the size of vessels involved, and immunological markers (e.g. ANCA in GPA), and immunohistological findings (e.g. IgA-dominant immune deposit in Henoch-Schönlein purpura)
Re: Takayasu’ Arteritis, what is the typical:
1. Clinical symptoms
2. Age
3. Sex ratio (M:F)
4. Ethnic Origin
5. Vessel size
Re: Takayasu’ Arteritis, what is the typical:
1. Clinical symptoms? Arm or leg claudication, decreased pulses, subclavian/aortic bruit
2. Age? 15-40yo
3. Sex ratio (M:F)? 1:9
4. Ethnic Origin? Asian >others
5. Vessel size? Large
Re: GCA what is the typical:
1. Clinical symptoms
2. Age
3. Sex ratio (M:F)
4. Ethnic Origin
5. Vessel size
Re: GCA what is the typical:
1. Clinical symptoms? Headache, jaw claudication, shoulder girdle/hip pain. diplopia
2. Age? >50yo
3. Sex ratio (M:F)? 1:3
4. Ethnic Origin? Caucasian >others
5. Vessel size? Large
Re: PAN what is the typical:
1. Clinical symptoms
2. Age
3. Sex ratio (M:F)
4. Ethnic Origin
5. Vessel size
Re: PAN what is the typical:
1. Clinical symptoms? Weight loss, livedo reticularis, mono/polyneuropathy, HTN
2. Age? 40-60yo
3. Sex ratio (M:F)? 2:3
4. Ethnic Origin? Any
5. Vessel size? Medium
Re: Kawasaki Disease what is the typical:
1. Clinical symptoms
2. Age
3. Sex ratio (M:F)
4. Ethnic Origin
5. Vessel size
Re: Kawasaki Disease what is the typical:
1. Clinical symptoms? Fever, conjunctivitis, cervical lymphadenopathy, mucositis, polymorphous exanthema
2. Age? 1-5yo
3. Sex ratio (M:F)? 1.5 : 1
4. Ethnic Origin? Asian > white > others
5. Vessel size? Medium
Re: GPA what is the typical:
1. Clinical symptoms
2. Age
3. Sex ratio (M:F)
4. Ethnic Origin
5. Vessel size
Re: GPA what is the typical:
1. Clinical symptoms? Sinusitis, oral ulcers, otitis media, haemoptysis, active urinary sediment
2. Age? 30-50 yo
3. Sex ratio (M:F)? 1:1
4. Ethnic Origin? Any
5. Vessel size? Small
Re: eGPA what is the typical:
1. Clinical symptoms
2. Age
3. Sex ratio (M:F)
4. Ethnic Origin
5. Vessel size
Re: eGPA what is the typical:
1. Clinical symptoms? Asthma, atopic history, mono/polyneuropathy, pulmonary infiltrates, eosinophilia
2. Age? 40-60 yo
3. Sex ratio (M:F)? 2:1
4. Ethnic Origin? Any
5. Vessel size? Small
Re: HSP what is the typical:
1. Clinical symptoms
2. Age
3. Sex ratio (M:F)
4. Ethnic Origin
5. Vessel size
Re: HSP what is the typical:
1. Clinical symptoms? Palpable purpura, abdominal pain, bloody diarrhoea
2. Age? 5-20 yo
3. Sex ratio (M:F)? 1:1
4. Ethnic Origin? Any
5. Vessel size? Small
Re: Behcet’s syndrome what is the typical:
1. Clinical symptoms
2. Age
3. Sex ratio (M:F)
4. Ethnic Origin
5. Vessel size
Re: Behcet’s syndrome what is the typical:
1. Clinical symptoms? Oral and genital ulcers, folliculitis, uveitis, thrombophelbitis
2. Age? 20-35yo
3. Sex ratio (M:F)? 1:1
4. Ethnic Origin? Middle Eastern > others
5. Vessel size? Small
Re: Leukocytoclastic vasculitis what is the typical:
1. Clinical symptoms
2. Age
3. Sex ratio (M:F)
4. Ethnic Origin
5. Vessel size
Re: Leukocytoclastic vasculitis what is the typical:
1. Clinical symptoms? Palpable purpura, maculopapular rash
2. Age? 30-50yo
3. Sex ratio (M:F)? 1:1
4. Ethnic Origin? Any
5. Vessel size? Small
