Vasculitis - Rheumatology Flashcards
Vasculitis definition, pathogenesis and Clinical characteristics ?
Definition:
б It is an inflammation and subsequent necrosis of blood vessels leading to tissue ischemia or infarction of any organ system.
б It is a Complex, very variable, serious group of chronic disease.
б Clinical signs and symptoms are common to many other diseases.
б The results of laboratory and other investigations are often not specific to vasculitis and can be misleading, so differential diagnosis is difficult.
Pathogenesis may be:
б Immune complex deposition (Type 3) OR
б Direct endothelial cell infection OR
б Anti-endothelial cell antibodies (Type 2) OR
б ANCA-mediated neutrophil activation (Type 2) OR
б T cell-dependent injury (Type 4)
Clinical Characteristics:
1. General manifestations:
б Clinical presentation often includes nonspecific constitutional symptoms including
1. fever,
2. malaise,
3. headache,
4. weight loss.
- Systemic manifestations:
- Signs and symptoms
» are generally dictated by the tropism of involved vessels. - Skin manifestations of vasculitis include
» petechiae,
» palpable purpura,
» subcutaneous nodules,
» livedo reticularis (Spasm of B.Vs)
» ulcerations,
» digital ischemia. - Kidney involvement of
» medium-sized and large vessel vasculitis is often in the form of renovascular hypertension.
» Glomerulonephritis may be seen in small vessel vasculitis. - Pulmonary small vessel involvement
» can cause alveolar hemorrhage, which can present with
a. cough, b. dyspnea, c. alveolar hemorrhage. - Mononeuritis multiplex
» is the characteristic finding of vasculitis affecting the vasa nervorum of the peripheral nervous system. (Peripheral neuropathy) - Gastrointestinal involvement of
» the mesenteric vasculature can cause
a. postprandial pain, b. bleeding, c. perforation. - Testicular pain or tenderness
» can be seen with hepatitis B infection in PAN. (Polyarteritis Nodosa) - Cardiac involvement: can include
» chest pain secondary to ischemic infarcts in the coronary arteries,
» pericarditis,
» cardiomyopathy,
» arrhythmias. - Arthritis,
» while nonspecific, can be present. - Significant clinical variability
» exists between the various vasculitis,
» although overlapping symptoms may be seen.
Giant cell arteritis definition, histological features and C/P?
❖ Giant cell arteritis (GCA)
» is inflammatory granulomatous arteritis of large cerebral arteries. Occurs in association with PMR (Polymyalgia rheumatica).
» Extremely rare under 50 years of age.
❖ The histological features of GCA:
1. Cellular infiltrates of CD4+ T lymphocytes, macrophages, and giant cells in the vessel wall.
2. Granulomatous inflammation of the intima and media.
3. Breaking up of the internal elastic lamina.
4. Giant cells, lymphocytes, and plasma cells in the internal elastic lamina.
❖ Clinical presentation
1. Severe headaches.
2. Tenderness of the scalp or of the temple.
3. Claudication, and tenderness of the jaw when eating.
4. Swelling of one or more temporal or occipital arteries.
» The most feared manifestation is sudden,
1. painless,
2. temporary, or permanent loss of vision in one eye due to involvement of the ophthalmic artery
Polymyalgia rheumatica short note?
б Polymyalgia rheumatica (PMR)
» causes a sudden onset of severe pain and stiffness of the shoulders and neck, and of the hips and lumbar spine: a limb girdle pattern.
б These symptoms are worse in the morning, lasting from 30 minutes to several hours.
б The clinical history is usually diagnostic, and the patient is always over 50 years old.
б Temporal artery biopsy
» shows giant cell arteritis in 10–30% of cases but is rarely performed unless giant cell arteritis is also suspected.
б Management of PMR or GCA
1. Corticosteroids
» significantly reduce the risk of irreversible visual loss and other focal ischemic lesions.
2. PMR: 10–15 mg prednisolone as a single dose in the morning
3. GCA: 60–100 mg prednisolone, usually in divided doses.
Takayasu’s arteritis short note?
б It is a granulomatous inflammation of the aorta and its major branches.
б This is rare, except in Japan.
б It is known as the pulseless disease or aortic arch syndrome.
б It is of unknown etiology and occurs in females.
б There is a vasculitis involving the aortic arch, as well as other major arteries.
б Absent peripheral pulses and hypertension are common.
б Management of Takayasu’s arteritis
1. Corticosteroids
» Help to improve constitutional symptoms.
» Eventually, heart failure and strokes may occur, but most patients survive for at least 5 years.
2. Treatment
» may involve a surgical bypass to improve perfusion of the affected areas.
Polyarteritis nodosa C/P and management?
б Classical polyarteritis nodosa (PAN) is rare.
б Usually occurs in middle-aged men.
б Clinical presentation
1. Constitutionals include
» fever, malaise, weight loss and myalgia.
» These initial symptoms are followed by Dramatic acute features that are due to organ infarction.
2. Neurological.
» Mononeuritis multiplex is due to arteritis of the vasa nervorum.
3. Abdominal.
» Pain is due to arterial involvement of the abdominal viscera, mimicking
1. acute cholecystitis, 2. pancreatitis, 3. appendicitis.
4. Gastrointestinal hemorrhage
» occurs because of mucosal ulceration.
5. Renal.
» Presentation is with hematuria and proteinuria.
» Hypertension and acute/chronic kidney disease occur.
6. Cardiac.
» Coronary arteritis causes myocardial infarction and heart failure.
» Pericarditis also occurs.
7. Skin.
» Subcutaneous hemorrhage and gangrene occur.
» A persistent livedo reticularis is seen in chronic cases.
8. Cutaneous and subcutaneous palpable nodules
» occur but are uncommon.
9. Lung. Involvement is rare
-
б Investigations: Angiography.
» Microaneurysms in hepatic, intestinal renal vessels
б Management:
» Treatment is with corticosteroids,
» usually in combination with immunosuppressive drugs such as azathioprine.
Kawaskis disease definition, clinical signs, complications and diagnosis?
б Kawasaki disease (KD), also known as Kawasaki syndrome,
» is an acute febrile illness of unknown cause that primarily affects children younger than 5 years of age.
б Clinical signs include
1. fever,
2. rash,
3. swelling of the hands and feet,
4. irritation,
5. redness of the sclera,
6. swollen cervical lymph glands in the neck,
7. irritation and inflammation of the mouth, lips, and throat.
8. Up to 50 percent may develop myocarditis, which may be associated with
a. tachycardia
b. decreased ventricular functioning,
c. and, in severe cases, heart failure.
9. In addition, in some cases, heart involvement may include
a. pericarditis,
b. or other abnormalities.
10. The most serious cardiac complication is
a. inflammation of coronary arteritis
b. and possible weakening, widening (dilation),
c. and bulging (aneurysms) of affected arterial walls.
d. Dilation and aneurysm formation occur in approximately three to 20 percent of patients.
б In severe cases, complications may include
1. the development of blood clots in the ballooned area with obstruction of blood flow,
2. (rupture) of an aneurysm,
3. or heart attack, leading to potentially life-threatening complications.
б Diagnosis:
» Diagnosis of complete KD requires fever of at least 5 days’ duration along with 4 or 5 of the principal clinical features.
» The principal clinical features are as follows:
1. Extremity changes
2. Polymorphous rash
3. Oropharyngeal changes
4. Bilateral, nonexudative, limbic sparing, painless bulbar conjunctival injection
5. Acute unilateral nonpurulent cervical lymphadenopathy with lymph node diameter greater than 1.5 cm.
Kawaskis disease investigations, DD and management?
б Laboratory evaluation:
1. Complete blood count (CBC), normochromic anemia, thrombocytosis
2. Electrolyte panel,
3. Renal function testing,
4. Liver enzymes,
5. Hypoalbuminemia
6. Erythrocyte sedimentation rate (ESR)
7. C-reactive protein (CRP),
8. Urinalysis.
б Diagnostics for Cardiac Complications:
» Serial echocardiograms should be obtained,
» preferably at the time of KD diagnosis, at 1-2 weeks, and at 5-6 weeks after the onset of the illness
б Differential Diagnosis of Kawasaki Disease:
1. Drug hypersensitivity
2. Juvenile idiopathic arthritis
3. Staphylococcal scalded skin syndrome
4. Stevens-Johnson syndrome
5. Streptococcal scarlet fever
6. Toxic shock syndrome
7. Viral infection
б Management:
A. The principal goal of treatment for KD
» is to prevent coronary artery aneurysms and other cardiac complications.
B. The mainstays of treatment for KD
» are IVIG and aspirin.
C. All patients with KD
» should be admitted to the hospital for administration of
a. IVIG,
b. echocardiography,
c. initiation of aspirin,
d. and for observation until fevers have resolved.
-
1. Intravenous immunoglobulin (IVIg):
a. 2 g/kg as a single IV infusion on diagnosis
b. IVIg
» should be given within the first 10 days of illness
» but should also be given to children diagnosed after 10 days if there is evidence of ongoing fever and/or inflammation
c. A second dose of 2 g/kg IVIg
» should be given to children who do not respond to the first dose,
» as demonstrated by persistent or recurrent fevers 36 hours after the end of the first IVIg infusion.
2. Aspirin:
a. 3-5 mg/kg orally as a daily dose until normal echo on follow up (minimum 6 weeks)
b. There is minimal risk of Reye syndrome with low-dose aspirin
c. Avoid non-steroid anti-inflammatory medications whilst on aspirin
3. Additional treatments:
» Several therapies are available for consideration in patients who are not responsive to initial IVIg including biological medicines such as infliximab (TNF inhibitor).
Small vessel vasculitis classifications ?
A. Circulating anti neutrophilic cytoplasmic antibody (ANCA associated): (Type 2)
1. Wegener’s granulomatosis
2. Churg-Strauss syndrome
3. Microscopic polyangiitis
B. Immune complex associated vasculitis: (Type 3)
1. Cryoglobulinemic vasculitis
2. Henoch-Schönlein purpura
3. Essential mixed cryoglobulinemia
4. Cutaneous leukocytoclastic vasculitis
5. Vasculitis 2nry to RA, SLE, Sjögren’s syndrome
6. Drugs
7. Infection (e.g. HIV, Hepatitis B, C)
Small vessel vasculitis presentation and treatment?
- Systemic
» malaise,
» fever,
» weakness,
» weight loss. - Pulmonary
» cough,
» hemoptysis,
» granulomatous upper respiratory tract masses,
» tracheal and bronchial stenosis. - Renal
» hematuria,
» proteinuria,
» elevated creatinine,
» glomerulonephritis - Other
» joint,
» skin,
» eye complaints,
» vasculitic neuropathy
Treatment:
For severe, life or organ threatening disease:
1. Pulse: methylprednisolone ( 0.5 - 1.0g /d x 1-3 d )
2. followed by
» prednisone (1 mg/kg/d PO) + cyclophosphamide (2 mg/kg/d PO)for 3-6 mo
» OR rituximab (375 mg/m2 x 4 weekly infusions )
3. followed by
» high dose MTX (20-25 mg PO/SC weekly) or azathioprine (2 mg/kg/d PO OD)
Condition that mimics vasculitis in presentation “ Differential Diagnosis”?
- Infective endocarditis
- Atrial myxoma
- Cholesterol emboli
- Malignancy
- Hypercoagulopathy
- Congenital collagen vascular disorder
- Leukocytoclastic vasculitis, palpable purpura.
