vasculitis

Consultant Rheumatology & Internal Medicine

Assistant ProfessorDr.Khudair Al-bedri

Learning Objectives

Definition Causes Classification When to Suspect Systemic Vasculitis General Approach to Diagnosis IX, Rx Overview on types of vascultis Quiz: Review Q

Definition

Vasculitis are a heterogeneous group of disease that are categorized by inflammation of blood vessels, leading to compromise of the vascular lumen and ischaemia . the commonest form of vasculitis is giant cell arteritis . Other less common vasculitis include Takayasu’s arteritis , polyarteritis nodosa (PAN),Wegener’s granulomatosis (WG), Churg-Strauss syndrome (CSS) and Henoch – Scho”nlein purpura (HSP).

Pathophysiology

Vasculitides can affect small ,medium or large vessels . Disease Aetiology can be classified into either : Primary (idiopathic ,which are autoimmune disorders and account for 45-55% of vasculitides ) Secondary , mainly due to : infection (15-20%) such as hepatitis B & C, tuberculosis (TB) and syphilis. connective tissue disease (15-20%) such as SLE ,mixed connective tissue disease (MCTD) and RA. drugs (10-15%) e.g. hydralazine ,propylthiouracil , sulphonamides ,beta-lactams and quinolones.

Risk factors for vasculitis

RA and SLE can cause secondary vasculitis. A history of asthma and/or nasal allergies is associated with CSS.
Other disorders
WG and CSS occur mainly in those aged> 40 HSP occurs mainly in children and young adults.
Age
Large vessel vasculitis is more common in women. PAN affects men more than women .
Gender
Syphilis ,TB and hepatitis B and C are associated with secondary vasculitis.
Infection
Many forms of vasculitis are more common in Caucasian patients compared to other ethnicities.Behcet’s disease common in those of Turkish descent. Ethnicity
WG is more common amongst northern Europeans and commonly presents in winter following respiratory infection. Microscopic polyangiitis is more common in southern Europe.
Geographical and environmental factors

Causes

1⁰: autoimmune disorder 2 ⁰: to infection, drugs, malignancy or CTD (SLE or RA)

Vasculitis Classification

Size and Type
Histopathologic
Clinical Features


Identifying the type of vasculitis is important Determining the extent of visceral organ involvement is more important

Clinical suspicion Systemic Vasculitis

Unexplained ischemia in : a young patient or a patient without risk factors for atherosclerosis. Multiple organ dysfunction in a systemically ill patient.

Clinical Features

Constitutional symptoms (fever, fatigue, malaise, anorexia, weight loss)  Any type of vasculitisPolymyalgia rheumatica (proximal muscle pain with morning stiffness)  Giant cell arteritisNondestructive oligoarthritis  Polyarteritis, Wegener's granulomatosis, Churg-Strauss vasculitis

Skin lesions Livedo reticularis, necrotic lesions, ulcers, nodules, digital tip infarcts, Palpable purpura Mononeuritis multiplex (Injury to 2 or more separate peripheral nerves (e.g., patient presents with both right foot drop and left wrist drop) Renal involvement Ischemic renal failure Glomerulonephritis


General Approach to Diagnosis
Exclude DDxAge, sex , and ethnic origin.Organs:-- size & type

Conditions That Can Mimic Primary Systemic VasculitisEmbolic disease, Endocarditis, atrial myxomaCholesterol embolization, Vessel stenosis or "spasm“, atherosclerosis, Drug-induced vasospasm (e.g., ergots, cocaine)Vessel thrombosis, DIC, TTP, APA syndrom, Systemic infection Malignancy

Demographic Associations Child, M = F, any ethnicity  Henoch-Schцnlein purpuraChild, M , Asian  Kawasaki diseaseYoung adult, M = F, Middle Eastern  Behзet's diseaseYoung adult, F, Asian  Takayasu's arteritisMiddle age, M, any ethnicity  Wegener's granulomatosis, Microscopic polyangiitis, Churg-Strauss vasculitisElderly, F, Caucasian  Giant cell arteritis

Investigation

Blood: -Hematological :CBC - Biochemical: KFT,LFT, -Immunological: RF,ANA,ANCA, complements(C3,C4,CH50) - Blood culture -SPE,Virology(HBV,HCV,HIV)

2) GUE : cells, cast, protein 3) Imaging: CXR, EMG&NCS 4) Angiography& tissue biopsy

Arteriography and Biopsy
Confirmation of a clinical suspicion of vasculitis usually requires arteriography, biopsy, or both. If a patient is over age 50 and presents with a new, unexplained headache and elevated ESR, with or without a tender or abnormal temporal artery, a temporal artery biopsy would be indicated.

In a patient who presents with a multisystem illness and testicular pain and swelling, a testicular biopsy should be considered. Sural nerve biopsy may be indicated in a patient with numbness and tingling in a lower extremity. If the urine sediment is abnormal, a renal biopsy might be obtained.

If a biopsy is impractical, an angiogram may be diagnostic. An angiogram should be obtained in cases of suspected large-vessel vasculitis. Examples: Branches of the aorta. mesenteric or renal vasculitis. (visceral angiogram) & perhaps, hepatic arteries should be performed.

Vasculitis of Small Vessels

Hypersensitivity vasculitis Affects postcapillary venules and arterioles of the skin. Palpable purpura, lesions may be urticarial or ulcerative.



Cryoglobulinemic vasculitis Arthritis, Raynaud's phenomenon, glomerulonephritis, palpable purpura Associated with hepatitis C.

Microscopic polyangiitis Pulmonary hemorrhage and glomerulonephritis .

Wegener's granulomatosis

Typically manifested by recurrent sinusitis or epistaxis, mucosal ulcerations, otitis media, cough, hemoptysis and dyspnea, pulmonary infiltrates and/or nodules. Glomerulonephritis may also be present

Churg-Strauss vasculitis Occurs in the setting of allergic rhinitis, asthma & eosinophilia.

Medium Vessel vasculitis Polyarteritis nodosa Middle-aged male Associations with hepatitis B Peripheral neuropathy, mononeuritis multiplex, intestinal ischemia, renal ischemia, testicular pain and livedo reticularis

Polymyalgia rheumatica

Definition Inflammatory disorder that causes muscle pain and stiffness, primarily in the neck, shoulders, upper arms, hips and thighs. Symptoms of polymyalgia rheumatica usually begin quickly over a few days. older than 65 (rarely affects people younger than 50) Women are two times more likely to develop the disorder. Related to and may coexist with giant cell arteritis.

Differential diagnoses

Fibromyalgia Hypothyroidisim Cervical spondylosis Rheumatoid arthritis Systemic vasculitis Inflammatory myopathy Malignancy Investigations High ESR & CRP Anemia

Treatment

Low dose of oral corticosteroid, such as prednisone: 10-20 mg. for 2-3 years.


Vasculitis of Large Vessels
Giant cell arteritis Women> 50 C/P New onset headache, scalp tenderness, vision disturbances , jaw claudication, polymyalgia rheumatica. Fever of unknown origin in the elderly The diagnosis is confirmed by temporal artery biopsy High dose corticosteroid(40-60 mg daily) are treatment of choice and should be commenced urgently because of the risk of visual loss +/- steroid-sparing agents, such as methotrexate or azathioprine Osteoporosis prophylaxis should be given

Takayasu's arteritis young women Malaise, arthralgias and the gradual onset of extremity claudication. Asymmetrically reduced pulses, usually along with a blood pressure different in the arms. The diagnosis is made by arteriography. High-dose corticosteroids and immunosuppressives

Behcet’s Disease Involve blood vessels of nearly all sizes and types, ranging from small arteries to large ones, and involving veins as well as arteries. HLA–B51 is a risk factorOther: infections and other environmental exposures.

Treatment of BD

Mucocutaneous regions : topical steroids and colchicineErythema nodosum and arthralgia: Colchicine, MTX . Resistant oral and genital ulceration:Thalidomide (teratogenic and neurotoxic.)Systemic disease: Steroids and immunosuppressives. Interferon–alpha has demonstrated some promise in Behcet’s.Biological Tx( Anti-TNFα)

Review Questions

Q1. Systemic vasculitis could occur secondary to: Infection Drugs Malignancy CTD All of the above

Q2. Vasculitis may be classified by: Size of vessel involvement Type of vessel involvement Histopathologic features Pattern of clinical features All of the above

Q3. Glomerulonephritis is a feature of WG. True False

Q4. Differential diagnoses of PMR include: A. Fibromyalgia B. Hypothyroidisim C. Rheumatoid arthritis D. All of the above E. None of the above



Q5. High-dose corticosteroids is the standard initial therapy for Giant cell arteritis True False

Q6. Recurrent aphthus ulcers are universal feature of Behcet’s disease.True False

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