CC May 2017 Granulomatosis with Polyangiitis (Wegener's Granulomatosis)

echoecho's version from 2017-06-14 14:56


Question Answer
What is another term for Wegener's granulomatosis?granulomatosis with polyangiitis (GPA)
GPA is the most common form of what type of vasculitis (about 2/3 of cases)?antineutrophil cytoplasm antibody (ANCA)-associated small vessel vasculitis
GPA is very rare, with an incidence of ___ to ___ cases per million per year?10; 20
It can present at any age, but is more common in what 2 decades of life?4th decade and 5th decade
It is one of several types of vasculitis with symptoms affecting ____ systems?multiple
What is the etiology?unknown
In GPA, antineutrophil cytoplasm antibody (ANCA) are directed against what?glycoprotein enzymes in neutrophil granules and monocyte lysosomes
When ANCA binds to these antigens, what happens?the associated WBCs are activated, promoting neutrophil adhesion to small artery endothelial cells resulting in inflammation.
Are complement pathways also abnormally activated when ANCA binds to the above antigens?yes
*** Patients with GPA may present with what nonspecific symptoms?weight loss, fever, fatigue, arthralgias, myalgias
*** More than ____% of patients have granulomatous upper airway disease that may present as epitaxis, sinusitis, deafness, hoarseness or stridor?90
*** List the pulmonary involvement of GPA?pulmonary nodules, pulmonary infiltrates, pleural effusions, pulmonary fibrosis
*** List the renal impairment?crescentric glomerulonephritis
Can GPA show cutaneous, nervous system, GI and cardiac involvement?yes
What other conditions can GPA mimic?neoplastic and infectious diseases
What test (in the correct clinical context) is highly suggestive of GPA?presence of cytoplasmic-staining ANCA
List 2 non-specific markers of inflammation that may also be elevated?CRP and ESR
*** What is the gold standard for GPA diagnosis?biopsy showing small vessel vasculitis (kidney is the most common biopsy site)
The American College of Rheumatology criteria for diagnosing GPA was proposed in 1990 and reported to have a sensitivity of 88% and a specificity of 92% when 2 or more criteria are positive. List these criteria?1) nasal or oral inflammation (development of painful or painless oral ulcers or purulent or bloody nasal discharge) 2) abnormal chest x-ray (showing the presence of nodules, fixed infiltrates or cavities) 3) urinary sediment (microhematuria showing > 5 red blood cells per high-power field or red cell casts in urine sediment 4) granulomatous inflammation on biopsy (histologic changes showing granulomatous inflammation within the wall of an artery or in the perivascular or extravascular area of an artery or arteriole
The above criteria in the above question were originally developed for research study design PRIOR to the common availability of ANCA testing and are _____ very reliable as diagnostic criteria?not
In 1994 Chapel Hill Consensus Conference developed new nomenclature and simplified classification of vasculitis, using what?vessel size (large, medium, small) to classify the spectrum of these diseases
*** What is the 5-year survival rate of GPA without intervention? What % of patients will go into remission with treatment? There is a very high relapse rate of ___% at 5 years?1) 75% 2) 80-90% 3) 50%
What medications are used for induction therapy?corticosteroids and cyclophosphamide
What medications are used for maintenance therapy?azathioprine, methotrexate, mycophenolate mofetil, leflunomide
*** SUMMARY = GPA is a multisystem vasculitis with symptoms often affecting what three areas of the body?1) sinuses 2) lungs 3) kidneys
*** SUMMARY = Clinical presentation ranges from what to what?chronic, nonspecific symptoms to severe manifestations of renal failure and pulmonary disease, depending on the organ system involved
*** SUMMARY = Besides the signs and symptoms characteristic of vasculitis, diagnosis involves what?1) biopsy findings 2) serologic antibody testing