Vasculitis Approach to Diagnose

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By Chest Department Ainshams University: 

By Chest Department Ainshams University Classification of and approach to the Vasculitides in adults

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The vasculitides are defined by the presence of leukocytes in the vessel wall with : Reactive damage to mural structures. Loss of integrity leads to bleeding. Compromise of the lumen leads to tissue ischemia and necrosis. In general, affected vessels vary in size, type, and location in association with the specific vasculitic disorder. Vasculitis may occur as a primary process or may be secondary to another underlying disease

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The exact mechanisms underlying these disorders are unclear. Three different pathogenic models of disease have been advanced to explain why the lesions of a particular vasculitic syndrome are only found in specific vessels : The distribution of the antigen responsible for the Vasculitis determines the pattern of vessel involvement.

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The recruitment and accumulation of the inflammatory infiltrate is determined by the endothelial cell, including the expression of adhesion molecules, the secretion of peptides and hormones, and the specific interaction with inflammatory cells. Some endothelial cells are therefore able to attract inflammatory cells, while others are not

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Nonendothelial structures of the vessel wall are involved in controlling the inflammatory process. In addition to the endothelial cells which provide costimulatory function, other cellular components serve as antigen-presenting cells and contribute proinflammatory mediators. It is likely that elements of each model contribute to the pathogenesis of these diseases.

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The vasculitides are often serious and sometimes fatal diseases that require prompt recognition and therapy. Symptomatic involvement of affected organs may either occur in isolation or in combination with multiple organs. The distribution of affected organs may suggest a particular vasculitic disorder, but significant overlap is observed.

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Fortunately, available treatments are helpful, particularly in the acute phase. However, during maintenance therapy with glucocorticoids and immunosuppressive agents, the adverse effects of drugs and superimposed infections may assume increasing importance. Mortality data suggest that while early deaths in Vasculitis are due to the active disease, late deaths may be due to the complications of therapy

CLASSIFICATION : 

CLASSIFICATION Classically, the vasculitic syndromes have been categorized by the predominant sizes of the blood vessels and types of vessels most commonly affected among patients with the disorder. The presence or absence of antineutrophil cytoplasmic antibodies (ANCA) is a more recent addition to proposed classification criteria

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Large vessel vasculitis Takayasu arteritis — Takayasu arteritis primarily affects the aorta and its primary branches. The inflammation may be localized to a portion of the thoracic or abdominal aorta and branches, or may involve the entire vessel. Giant cell arteritis — Giant cell or temporal arteritis is a chronic vasculitis of large and medium size vessels. Although it may be generalized, vessel inflammation most prominently involves the cranial branches of the arteries originating from the aortic arch.

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Medium sized vessel vasculitis Polyarteritis nodosa — Polyarteritis nodosa is a systemic necrotizing vasculitis that typically affects the small and medium-sized muscular arteries . Kawasaki disease — Kawasaki disease is an arteritis of large, medium, and small arteries, particularly the coronary arteries. The disease usually occurs in children, and is often associated with a mucocutaneous lymph node syndrome. . Isolated central nervous system vasculitis — Isolated central nervous system vasculitis affects medium and small arteries over a diffuse area of the central nervous system, without symptomatic involvement of extracranial vessels.

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Small vessel vasculitis Churg -Strauss arteritis — Churg -Strauss arteritis (also called allergic granulomatosis and angiitis ) is a vasculitis of the medium and small sized muscular arteries, and is often found with vascular and extravascular granulomatosis . The vasculitis classically involves the arteries of the lung and skin, but may be generalized

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Wegener's granulomatosis — Wegener's granulomatosis is a systemic vasculitis of the medium and small arteries, as well as the venules and arterioles. It typically produces granulomatous inflammation of the upper and lower respiratory tracts and necrotizing, pauci -immune glomerulonephritis in the kidneys. Wegener's granulomatosis is usually associated with antineutrophil cytoplasmic antibodies (ANCA).

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Microscopic polyarteritis — Microscopic polyarteritis or polyangiitis is a vasculitis which primarily affects capillaries, venules , or arterioles. Involvement of small and medium-sized arteries may also be present. This disorder is thought by some investigators to represent part of a clinical spectrum that includes Wegener's granulomatosis , since both are associated with the presence of ANCA and similar histologic changes outside the respiratory tract.

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Henoch-Schönlein purpura —  (HSP) is a systemic vasculitis that is characterized by the tissue deposition of IgA -containing immune complexes. Biopsy of lesions reveals inflammation of the small blood vessels (most prominent in the postcapillary venules ). This disorder is considered a form of hypersensitivity vasculitis, but is distinguished from other forms of hypersensitivity by the finding of prominent deposits of IgA .

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Essential cryoglobulinemic vasculitis is characterized by the presence of cryoglobulins , which are serum proteins that precipitate in the cold and dissolve upon rewarming . Cryoglobulins typically are composed of a mixture of immunoglobulins and complement components. In this disorder, which is most often due to hepatitis C virus infection, immune complexes are deposited in the walls of capillaries, venules or arterioles, thereby resulting in small vessel inflammation.

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hypersensitivity vasculitis is most properly used to refer to vasculitis that occurs as a hypersensitivity reaction to a known or suspected substance such as a vasculitic drug reaction. In hypersensitivity vasculitis, the presence of skin vasculitis with palpable petechiae or purpura is typically a major finding. Biopsy of these lesions reveals inflammation of the small blood vessels, called leukocytoclastic vasculitis, that is most prominent in the postcapillary venules .

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Vasculitis secondary to connective tissue disorders — A subset of patients with systemic lupus erythematosus , rheumatoid arthritis, relapsing polychondritis , Behcet's disease, and other connective tissue disorders may have or develop an associated vasculitis. The vasculitic process in this setting most frequently involves the small muscular arteries, arterioles, and venules . A propensity for involvement of particular organs varies with the underlying autoimmune disorder

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Vasculitis secondary to viral infection — A number of viral infections may cause a vasculitis of medium and/or small vessels . This association is most commonly observed with hepatitis B and C viruses, but may also be seen with HIV, cytomegalovirus, Epstein-Barr virus, and Parvovirus B19. The clinical presentation may be similar to that of polyarteritis nodosa or microscopic polyangiitis . Although the disorder is probably immune complex mediated, it must be distinguished from non-viral associated vasculitides since treatment consists of the administration of anti-viral, and not anti-inflammatory

CLINICAL MANIFESTATIONS AND APPROACH TO DIAGNOSIS: 

CLINICAL MANIFESTATIONS AND APPROACH TO DIAGNOSIS . The presence of vasculitis should be considered in patients who present with systemic symptoms in combination with evidence of single and/or multiorgan dysfunction. Although neither sensitive or specific, common complaints and signs of vasculitis include fatigue, weakness, fever, arthralgias , abdominal pain, hypertension, renal insufficiency (with an active urine sediment containing red and white cell and occasionally red cell casts), and neurologic dysfunction.

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The diagnosis of vasculitis is often delayed because the clinical manifestations can be mimicked by a number of other disorders. However, the presence of certain signs is strongly suggestive. As examples: Mononeuritis multiplex (or asymmetric polyneuropathy ) is highly suggestive of vasculitis (particularly polyarteritis nodosa ), since diabetic neuropathy is the only other common cause of this problem in developed countries.

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Palpable purpura — Patients with palpable purpura alone are likely to have cutaneous leukocytoclastic vasculitis of the hypersensitivity vasculitis type ; if the purpura is present with systemic organ involvement, such patients are likely to have HSP or microscopic polyarteritis . Pulmonary-renal involvement — The combination of hemoptysis and renal involvement suggestive of glomerulonephritis suggests the diagnosis of Wegener's granulomatosis or microscopic polyangiitis .

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However, certain other disorders, particularly anti- glomerular basement membrane (anti-GBM) antibody disease, may cause an identical combination of findings. One report of 97 such patients, for example, found that 48 were ANCA-positive and presumably had Wegener's granulomatosis or microscopic polyangiitis , 7 had both ANCA and anti-GBM antibodies, and six had anti-GBM antibody disease alone . The remaining patients had a variety of disorders including pulmonary emboli, infection, and lupus

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Because of the variability of the manifestations of vasculitic syndromes, it has not been possible to outline a single uniform method of evaluating patients suspected of having one of these conditions. Nevertheless, there are shared elements in the history, physical examination, and laboratory evaluation of the patient suspected of having a vasculitis. In addition, specific procedures are essential in some forms of vasculitis, such as arch angiogram for Takayasu's arteritis and temporal artery biopsy for giant cell arteritis .

Diagnostic approach : 

Diagnostic approach History — A detailed history is important to assess whether the patient has recently been administered drugs (which may produce hypersensitivity vasculitis), has a history of hepatitis (hepatitis C virus is responsible for most cases of mixed cryoglobulinemia and some cases of polyarteritis ), or has been diagnosed with any disorder known to be associated with a vasculitis (such as systemic lupus erythematosus ).

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The propensity of certain disorders to occur among certain age groups and/or in women may favor the diagnosis of a specific vasculitic . In a review of 807 patients from the American College of Rheumatology cohort, the mean age at onset was between 45 and 50 for Wegener's granulomatosis and polyarteritis nodosa compared with 17 and 26 years of age for HSP and Takayasu's arteritis , and 69 years for giant cell arteritis

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Physical examination — A careful physical examination helps to determine the extent of vascular lesions, the distribution of affected organs, and the presence of additional disease processes. As mentioned above, certain findings, such as mononeuritis multiplex and palpable purpura , are highly suggestive of an underlying vasculitic process.

Laboratory tests : 

Laboratory tests Laboratory tests help ascertain the type of vasculitis, and the degree and types of organs affected. Basic laboratory analysis should include serum creatinine muscle enzyme concentrations liver function studies erythrocyte sedimentation rate, hepatitis serologies Urinalysis chest x-ray electrocardiogram.

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Other tests that may be warranted include cerebrospinal fluid analysis central nervous system imaging pulmonary function testing and blood and tissue culture. Although many of these tests are nonspecific, they permit the initial assessment of organ involvement, and may suggest, include, or exclude additional diagnostic possibilities, particularly infection

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ANA — A positive antinuclear antibody test suggests the presence of an underlying connective tissue disorder, particularly systemic lupus erythematosus . Complement — Low serum complement levels may be present in mixed cryoglobulinemia and lupus but not most other vasculitis disorders. Among those with cryoglobulins , infection with hepatitis C virus must be considered. ANCA — Although not diagnostic, the presence of ANCA directed against protease 3 strongly suggests a diagnosis of Wegener's granulomatosis , while ANCA directed against myeloperoxidase favors a diagnosis of microscopic polyarteritis . Other ANCA associated disorders include drug-induced vasculitis and Churg -Strauss vasculitis..

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Electromyography — Electromyography is useful if a systemic vasculitis is suspected and neuromuscular symptoms are present, such as a mononeuritis multiplex. Tissue biopsy — Biopsy examination of the most clinically involved tissue is essential for diagnosis. The chances of obtaining a diagnostic specimen are greatest when a specimen is taken from an involved portion of an affected organ and when the tissue sample is of adequate size. A "blind" biopsy is less likely to be fruitful. Histologic findings evolve as lesions progress and may vary from an acute inflammation to those due to healing and repair

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Arteriography — are helpful in identifying and characterizing a vasculitis of large and medium-sized arteries, such as polyarteritis nodosa , Takayasu's arteritis , and giant cell arteritis with an aortic arch syndrome. In these conditions, angiographic abnormalities may not be pathognomonic , but usually support a diagnosis when combined with other clinical data.

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Angiograms of mesenteric or renal arteries in polyarteritis nodosa may show aneurysms, occlusions, and vascular wall irregularities .In the patient suspected of having polyarteritis nodosa in whom an obvious area for biopsy is absent, a mesenteric angiogram should be considered, particularly in the presence of abdominal pain. In contrast, angiography is unlikely to be helpful in assessing a small vessel vasculitis, such as microscopic polyangiitis . The affected vessels in this disorder are below the resolution of usual angiograms.

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The decision to biopsy and/or perform angiography in a patient suspected of a vasculitis is based upon an overall clinical assessment and the relative complications associated with each procedure in a particular clinical setting. As an example, arteriography alone is usually performed to confirm the diagnosis of Takayasu's arteritis . The arteriographic changes tend to be most pronounced in the region of the aortic arch and its primary branches, areas relatively inaccessible for biopsy.

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In contrast, involvement of the skin is characteristic of patients with a leukocytoclastic vasculitis; the skin is easily accessible for biopsy, a procedure not associated with significant morbidity. However, the observed changes are not specific unless there is IgA deposition, establishing the diagnosis of HSP.

CLASSIFICATION CRITERIA  : 

CLASSIFICATION CRITERIA Criteria for the classification of most of the major forms of vasculitis have been established by the American College of Rheumatology in 1990 and are presented below . These criteria are based upon prospective data from patients with vasculitis; they do not include all characteristics of a particular disorder, only those that help to distinguish the disorder from other vasculitides . ,the diagnosis of a particular form of vasculitis is virtually always confirmed by tissue biopsy.

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Takayasu's arteritis .The following classification criteria for Takayasu's arteritis were primarily designed to distinguish this disorder from other forms of vasculitis : - Age at disease onset ≤ 40 years - Claudication of the extremities - Decreased pulsation of one or both brachial arteries - Difference of at least 10 mmHg in systolic blood pressure between the arms - Bruit over one or both subclavian arteries or the abdominal aorta – - Arteriographic narrowing or occlusion of the entire aorta, its primary branches, or large arteries in the proximal upper or lower extremities.Patients are said to have Takayasu's arteritis if at least three of the six criteria are present.

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Giant cell arteritis .In a patient with vasculitis, the finding of three of the following five criteria was associated with a 94 percent sensitivity and a 91 percent specificity for the diagnosis of GCA: Age ≥ 50 years at time of disease onset Localized headache of new onset Tenderness or decreased pulse of the temporal artery Erythrocyte sedimentation rate (ESR) greater than 50 mm/h Biopsy which includes an artery, and reveals a necrotizing arteritis with a predominance of mononuclear cells or a granulomatous process with multinucleated giant cells If an elevated ESR is excluded, but scalp tenderness and claudication of the jaw, tongue, or with deglutition are added as criteria, sensitivity for the diagnosis is now 95 percent with a specificity of 91 percent

Polyarteritis nodosa : 

Polyarteritis nodosa The ACR has established ten criteria for the classification PN Unexplained weight loss > 4 kg Livedo reticularis Testicular pain or tenderness Myalgias (excluding that of the shoulder and hip girdle), weakness Polyneuropathy Mononeuropathy or polyneuropathy New onset diastolic blood pressure > 90 mmHg Elevated levels of urea nitrogen (>40 mg/ dL ) or creatinine (>1.5 mg/ dL ) Evidence of hepatitis B virus infection via serum antibody or antigen serology Characteristic arteriographic abnormalities A biopsy of small- or medium-sized artery containing polymorphonuclear cells

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Kawasaki disease Diagnosis requires the presence of fever lasting five days or more without any other explanation, combined with at least four of the five following physical findings : Bilateral conjunctival injection Oral mucous membrane changes, including injected or fissured lips, injected pharynx, or strawberry tongue Peripheral extremity changes, including erythema of palms or soles or edema of hands or feet (acute phase), and periungual desquamation (convalescent phase) Polymorphous rash Cervical lymphadenopathy (at least one lymph node >1.5 cm in diameter)

Churg-Strauss arteritis : 

Churg -Strauss arteritis The following six criteria have been formulated for the diagnosis of the Churg -Strauss arteritis in a patient with documented vasculitis - Asthma (a history of wheezing or the finding of diffuse high pitched wheezes on expiration) - Eosinophilia of >10 percent on differential white blood cell count - Mononeuropathy (including multiplex) or polyneuropathy - Migratory or transient pulmonary opacities detected radiographically - Paranasal sinus abnormality Biopsy containing a blood vessel showing the accumulation of eosinophils in extravascular areas . The presence of four or more of these criteria yields a sensitivity of 85 percent and a specificity of 99.7 percent for the Churg -Strauss arteritis

Wegener's granulomatosis : 

Wegener's granulomatosis The ACR proposed four clinical criteria for the classification of Wegener's granulomatosis in 1990, before the availability of ANCA testing : Nasal or oral inflammation (painful or painless oral ulcers or purulent or bloody nasal discharge). Abnormal chest radiograph showing nodules, fixed infiltrates, or cavities. Abnormal urinary sediment (microscopic hematuria or red cell casts). Granulomatous inflammation on biopsy of an artery or perivascular area.

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The presence of two or more of these four criteria yielded a sensitivity of 88 percent and a specificity of 92 percent. As a result, an abnormal chest radiograph or the findings of granulomatous inflammation on biopsy are not absolute requirements to distinguish patients with Wegener's granulomatosis from those with other forms of vasculitis.

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Antineutrophil cytoplasmic antibodies — At present, the diagnosis of Wegener's granulomatosis is suggested from the clinical and laboratory findings and from the presence of circulating antineutrophil cytoplasmic antibodies (ANCA) that are usually directed against proteinase 3 (C-ANCA) . Almost all patients with active systemic Wegener's granulomatosis have a positive ANCA . However, ANCA alone, including the presence of antiproteinase 3 antibodies which are more specific for Wegener's granulomatosis , does not appear to be sufficiently accurate to preclude the need for tissue biopsy.

Microscopic polyangitis: 

Microscopic polyangitis Some patients have equivalent vasculitic lesions to those observed in Wegener's granulomatosis , but, at least at presentation, do not have symptomatic or histologic respiratory involvement; such individuals are considered to have microscopic polyarteritis . However, two observations suggest that this disorder is closely related to Wegener's granulomatosis and should be considered similarly: some patients subsequently develop classic respiratory tract lesions and ANCA are typically present

Hypersensitivity vasculitis : 

Hypersensitivity vasculitis The ACR proposed the following five criteria for the classification of hypersensitivity vasculitis in a patient with vasculitis : Age >16 Use of a possible offending drug in temporal relation to the symptoms Palpable purpura . Maculopapular rash Biopsy of a skin lesion showing neutrophils around an arteriole or venule

Henoch-Schönlein purpura : 

Henoch-Schönlein purpura HSP is characterized by the deposition of IgA on skin or renal biopsy.There are also clinical criteria : Palpable purpura Bowel angina Gastrointestinal bleeding Hematuria Age at onset ≤ 20 years lesion No new medications The presence of three or more of the six criteria yielded a correct classification of HSP in 87 percent of. The presence of two or fewer criteria yielded a correct classification of hypersensitivity vasculitis in 74 percent

Essential cryoglobulinemic vasculitis: 

Essential cryoglobulinemic vasculitis Specific criteria for a diagnosis of essential cryoglobulinemic vasculitis have not yet been formulated. The diagnosis is typically made from the history, skin purpura , low complement levels, demonstration of circulating cryoglobulins , and histology showing small vessel inflammation with immune deposits found in the vascular walls.

Vasculitis secondary to connective tissue disorders : 

Vasculitis secondary to connective tissue disorders The diagnostic criteria for vasculitis secondary to an underlying connective tissue disorder, and not a primary disorder, rests upon histologic evidence of a vasculitis in combination with a firm diagnosis of a connective tissue disorder. Discussions of the criteria for the diagnosis of those connective tissue disorders in which vasculitis may emerge is presented in the appropriate topic reviews.

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DIFFERENTIAL DIAGNOSIS — Patients with nonvasculitic disease processes may present with symptoms and findings which closely mimic those of the various vasculitides . Perhaps most common are systemic rheumatic diseases such as systemic lupus erythematosus . Other disorders that should be considered in selected patients include: Fibromuscular dysplasia (predominantly affects the aorta and its primary branches) Cholesterol emboli Atrial myxoma with emboli Infective endocarditis Malignancies, such as lymphomatoid granulomatosis /polymorphic reticulosis and angioimmunoblastic T cell lymphoma Mycotic aneurysm with embolization (predominantly with arteriole, capillary, and venule involvement) Bacteremia Rickettsial infection Ergotism (principally affects medium and small sized muscular arteries) Thrombocytopenia and other processes associated with purpura Radiation fibrosis (radiation-induced vasculopathy ) Neurofibromatosis Congenital coarctation of the aorta Amyloidosis Livedoid vasculopathy Malignant atrophic papulosis ( Degos disease or syndrome) Necrotic arachnidism ( Loxosceles species spider bites)