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Avvedimento EV, Krieg T. Scleroderma. N Engl J Med. 2009;360(19):1989–2003.
AUTOIMMUNE VASCULITIS
See Vasculitis (Chapter 3 , Cardiovascular Disorders).
EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (CHURG-STRAUSS SYNDROME)
Definition
Eosinophilic granulomatosis with polyangiitis (EGPA), also referred to as Churg-Strauss syndrome (CSS) or allergic granulomatosis and angiitis, is a multisystem vasculitis affecting small and medium-sized blood vessels and characterized by asthma, allergic rhinitis and sinusitis, eosinophil-rich inflammation, and peripheral blood eosinophilia.
EGPA has three phases:
Prodromal (allergic) phase: characterized by airway inflammation (asthma and allergic rhinitis).
Eosinophilic phase: peripheral eosinophilia and eosinophilic infiltration of multiple organs, especially the lungs and gastrointestinal tract.
Vasculitic phase: systemic vasculitis that can be life threatening and is often associated with vascular and extravascular granulomatosis.
See Table 2-1 .
TABLE 2–1. The American College of Rheumatology 1990 Criteria for the Classification of Vasculitis
Adapted from the website of the American College of Rheumatology ( www.rheumatology.org ).
Who Should Be Suspected?
Candidates are patients with asthma that is poorly controlled and a necrotizing eosinophilic vasculitis. In addition to pulmonary and sinus involvement, organs commonly affected by EGPA include the skin, kidneys, peripheral nervous system, gastrointestinal tract, and cardiovascular system. Cardiac involvement accounts for approximately one half of deaths attributable to EGPA.
EGPA frequently occurs in patients between 40 and 60 years of age.
Laboratory Findings
Diagnosis relies on a combination of tissue biopsy (eosinophilic infiltrates, necrosis, and eosinophilic giant cell vasculitis) and laboratory testing.
Leukocytosis with peripheral blood eosinophilia (>1,500 cells/μL; usually in the 5,000–9,000 range) is found in approximately 90% of patients. Eosinophilia may be missed in some cases due to spontaneous fluctuations or corticosteroid therapy preceding the diagnosis.
Circulating ANCAs are present in 40–60% of EGPA cases. The majority of ANCA-positive patients have antibodies against myeloperoxidase (MPO), with a perinuclear staining pattern (p-ANCA).
Elevated serum IgE during the vasculitic phase.
Hypergammaglobulinemia and markedly elevated ESR and CRP.
Complement components (C3, C4, CH50) may be normal or elevated.
Normochromic, normocytic anemia.
Proteinuria and microscopic hematuria can be present.
GIANT CELL ARTERITIS
Definition
Giant cell (temporal) arteritis is a chronic systemic vasculitis of the large- and medium-sized vessels, involving especially the cranial branches of the arteries that originate from the aortic arch.
Visual impairment is a major complication of the disease, and failure to make the diagnosis may lead to irreversible visual loss.
See Table 2-1 .
Who Should Be Suspected?
Candidates are patients with severe bitemporal headache, visual disturbances (primarily partial, transient monocular visual loss), jaw claudication, and symptoms of polymyalgia rheumatica (30–50% of cases). Other frequent manifestations of systemic inflammation can be present and they include fatigue, general malaise, fever, anorexia, weight loss, and night sweats.
This disorder mainly affects individuals >50 years or age and is extremely rare in younger people. The incidence rises markedly with increasing age, peaking in the eighth decade of life.
Laboratory Findings
Diagnosis is mainly clinical. Laboratory studies are pertinent, but not specific.
Increased ESR (≥50 mm/h, mean 88 mm/h).
Thrombocytosis with platelet counts >400,000/μL and CRP levels >2.45 mg/dL have been found to be to the strongest laboratory predictors of a positive temporal artery
AUTOIMMUNE VASCULITIS
See Vasculitis (Chapter 3 , Cardiovascular Disorders).
EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (CHURG-STRAUSS SYNDROME)
Definition
Eosinophilic granulomatosis with polyangiitis (EGPA), also referred to as Churg-Strauss syndrome (CSS) or allergic granulomatosis and angiitis, is a multisystem vasculitis affecting small and medium-sized blood vessels and characterized by asthma, allergic rhinitis and sinusitis, eosinophil-rich inflammation, and peripheral blood eosinophilia.
EGPA has three phases:
Prodromal (allergic) phase: characterized by airway inflammation (asthma and allergic rhinitis).
Eosinophilic phase: peripheral eosinophilia and eosinophilic infiltration of multiple organs, especially the lungs and gastrointestinal tract.
Vasculitic phase: systemic vasculitis that can be life threatening and is often associated with vascular and extravascular granulomatosis.
See Table 2-1 .
TABLE 2–1. The American College of Rheumatology 1990 Criteria for the Classification of Vasculitis
Adapted from the website of the American College of Rheumatology ( www.rheumatology.org ).
Who Should Be Suspected?
Candidates are patients with asthma that is poorly controlled and a necrotizing eosinophilic vasculitis. In addition to pulmonary and sinus involvement, organs commonly affected by EGPA include the skin, kidneys, peripheral nervous system, gastrointestinal tract, and cardiovascular system. Cardiac involvement accounts for approximately one half of deaths attributable to EGPA.
EGPA frequently occurs in patients between 40 and 60 years of age.
Laboratory Findings
Diagnosis relies on a combination of tissue biopsy (eosinophilic infiltrates, necrosis, and eosinophilic giant cell vasculitis) and laboratory testing.
Leukocytosis with peripheral blood eosinophilia (>1,500 cells/μL; usually in the 5,000–9,000 range) is found in approximately 90% of patients. Eosinophilia may be missed in some cases due to spontaneous fluctuations or corticosteroid therapy preceding the diagnosis.
Circulating ANCAs are present in 40–60% of EGPA cases. The majority of ANCA-positive patients have antibodies against myeloperoxidase (MPO), with a perinuclear staining pattern (p-ANCA).
Elevated serum IgE during the vasculitic phase.
Hypergammaglobulinemia and markedly elevated ESR and CRP.
Complement components (C3, C4, CH50) may be normal or elevated.
Normochromic, normocytic anemia.
Proteinuria and microscopic hematuria can be present.
GIANT CELL ARTERITIS
Definition
Giant cell (temporal) arteritis is a chronic systemic vasculitis of the large- and medium-sized vessels, involving especially the cranial branches of the arteries that originate from the aortic arch.
Visual impairment is a major complication of the disease, and failure to make the diagnosis may lead to irreversible visual loss.
See Table 2-1 .
Who Should Be Suspected?
Candidates are patients with severe bitemporal headache, visual disturbances (primarily partial, transient monocular visual loss), jaw claudication, and symptoms of polymyalgia rheumatica (30–50% of cases). Other frequent manifestations of systemic inflammation can be present and they include fatigue, general malaise, fever, anorexia, weight loss, and night sweats.
This disorder mainly affects individuals >50 years or age and is extremely rare in younger people. The incidence rises markedly with increasing age, peaking in the eighth decade of life.
Laboratory Findings
Diagnosis is mainly clinical. Laboratory studies are pertinent, but not specific.
Increased ESR (≥50 mm/h, mean 88 mm/h).
Thrombocytosis with platelet counts >400,000/μL and CRP levels >2.45 mg/dL have been found to be to the strongest laboratory predictors of a positive temporal artery
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