2011년 the Boards of Directors of the American College of Rheumatology (ACR), the American Society of Nephrology (ASN), the European League Against Rheumatism (EULAR)는 "Wegener's granulomatosis"라는 용어를 "granulomatosis with polyangiitis", 약자로 GPA로 변경할 것을 권고하였습니다.
이전 블로그 내용을 보면 다음과 같은 내용이 나옵니다.
https://blog.naver.com/sjloveu2/222067706358
Granulomatosis with polyangiitis (GPA)와 microscopic polyangiitis (MPA)는 antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) 내에서 구분되는 범주로 간주되지만 현저하게 중복된 임상 발현을 가지고 있어서 때때로 한 환자에서 이 두 질환을 구분하는 것은 어려우며 ANCA type (anti-MPO 또는 anti-PR3)은 질병 유형보다는 좀 더 예후와 임상적 의미를 지니고 있다고 언급되어 있습니다.
따라서 granulomatosis with polyangiitis (GPA)와 microscopic polyangiitis (MPA)의 증상은 다음과 같이 함께 언급되어 있습니다.
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Ear, nose, and throat Ear, nose, and throat (ENT) manifestations can occur in patients with either GPA or MPA. However, they are much more common in patients with GPA (estimated frequency is 90 percent versus 35 percent in MPA). ENT manifestations include nasal crusting, sinusitis, otitis media, earache, otorrhea, persistent rhinorrhea, purulent/bloody nasal discharge, oral and/or nasal ulcers, and polychondritis. Patients frequently develop conductive and/or sensorineural hearing loss, either of which can lead to severe permanent hearing impairment. Patients with GPA more typically have evidence of bone and cartilage destruction resulting in a saddle nose deformity , upper airway and retro-orbital masses, and cranial nerve entrapment. |
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Tracheal and pulmonary disease Patients with either GPA or MPA may present with involvement of airways or pulmonary parenchyma causing hoarseness, cough, dyspnea, stridor, wheezing, hemoptysis, or pleuritic pain. These symptoms may be accompanied by signs of tracheal or subglottic stenosis, pulmonary consolidation, and/or pleural effusion. Patients may develop pulmonary fibrosis and pulmonary arterial hypertension. The chest radiograph findings are variable. Common manifestations include nodules, patchy or diffuse opacities and fleeting pulmonary infiltrates, and hilar adenopathy. Although parenchymal lung nodules are a well-recognized manifestation, antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) occasionally presents with tumor-like masses outside the lung. Among 20 such reported cases, the most common extrathoracic locations were the breast and kidney. Failure to consider vasculitis in the differential diagnosis in such cases may lead to unnecessary surgery, including nephrectomy. Sometimes, a diagnosis of sarcoidosis or pulmonary tuberculosis will be made mistakenly. |
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Renal manifestations Renal involvement is common in GPA and MPA. In studies from the National Institutes of Health (NIH) in the United States, evident glomerulonephritis was present in only 18 percent of patients at presentation, but glomerulonephritis subsequently developed in 77 to 85 percent of patients, usually within the first two years of disease onset. The manifestations of the glomerulonephritis in AAV are similar to those that occur in other causes of glomerulonephritis, including: ● Asymptomatic hematuria, which may remit and relapse, in association with normal renal function. The diagnosis of ANCA-positive glomerulonephritis may be delayed in such patients in favor of a clinical diagnosis of thin basement membrane disease or IgA nephropathy. However, the risk of progression to end-stage renal disease is considerably higher in ANCA-positive disease. ● A rise in serum creatinine occurring over days or weeks with hematuria and cellular casts. Rapidly rising serum creatinine with hematuria, hypertension, and edema is a medical emergency that requires urgent therapy. ● A variable degree of proteinuria that is usually subnephrotic. Proteinuria in patients with AAV is most likely the consequence of fibrosed glomeruli or tubular fibrosis in an individual who may or may not be in remission. Higher amounts of proteinuria, such as proteinuria above 3 g/day, may be more common in patients who present later in the course of disease and who have had previous necrotizing glomerulonephritis (and therefore have more focal and segmental glomerulosclerosis/fibrosis at the time of presentation). Alternatively, patients with AAV who have high amounts of proteinuria may have a second concurrent glomerular disease (such as membranous nephropathy) or have an atypical histologic pattern characterized by glomerular immune complex deposition. ● Rapidly progressive glomerulonephritis, which is common in this group of diseases. |
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Cutaneous manifestations About one-half of patients with GPA or MPA have cutaneous manifestations. The most common skin lesion is leukocytoclastic angiitis, which causes purpura involving the lower extremities that may be accompanied by focal necrosis and ulceration. Skin lesions may also include urticaria, livedo reticularis, and nodules. Occasional patients with erythema nodosum, pyoderma gangrenosum, and Sweet syndrome may also have ANCA-positive disease. |
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Ophthalmic and orbital manifestations Patients with AAV may develop conjunctivitis, corneal ulceration, episcleritis/scleritis, optic neuropathy, retinal vasculitis, and uveitis. In addition, retro-orbital pseudotumor and nasolacrimal duct obstruction may occur. Affected patients can present with a variety of symptoms and signs including eye pain, foreign body sensation, visual disturbance, diplopia, and proptosis. |
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Neurologic manifestations Patients with AAV may develop clinical manifestations involving the nervous system, including mononeuritis multiplex, sensory neuropathy, cranial nerve abnormalities, central nervous system mass lesions, external ophthalmoplegia, and sensorineural hearing loss. Meningeal disease is most commonly associated with granulomatous inflammation of the central nervous system. Peripheral nervous system involvement is noted in approximately 15 percent of patients with GPA and 70 percent of those with MPA. Patients with ANCA-associated vasculitis and mononeuritis multiplex have been found to have a worse prognosis than without this feature. This was demonstrated in a large multicenter study of 193 patients with eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss), polyarteritis nodosa, and MPA without poor prognostic factors (including creatinine >1.6 mg/dL [140 micromol/L], proteinuria >1 g/day, severe gastrointestinal involvement, cardiomyopathy, and/or central nervous system involvement) at the time of diagnosis who were initially treated with glucocorticoids alone [42]. Patients with mononeuritis multiplex at the time of diagnosis were more likely to fail initial treatment with glucocorticoids alone and require additional immunosuppressive therapy. |
Approximately 82 to 94 percent of patients with either GPA or MPA have a positive ANCA, depending upon severity of disease. GPA is primarily associated with PR3-ANCA, while MPA is primarily associated with MPO-ANCA. However, 20 percent of patients with GPA or MPA have the alternative ANCA, and at least 10 percent of patients are ANCA negative
REF. UpToDate 2020.08.22
