Idiopathic Isolated Pulmonary Arteritis with Chronic Cor Pulmonale

Okubo, Shumpei; Kunieda, Takeyoshi; Ando, Motomi; Nakajima, Nobuyuki; Yutani, Chikao

doi:10.1378/chest.94.3.665

Cited by 22 publications

(5 citation statements)

References 4 publications

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“…In those cases, the interval between detection of pulmonary arterial stenosis and diagnosis of Takayasu's arteritis ranged between 1 and 16 years. Our case is similar to two reported cases of idiopathic pulmonary arteritis with respect to the 99m Tc‐MAA perfusion defect in the entire unilateral lung, severe stenosis of the main PA, and pulmonary hypertension 5,6 . Uchiyama et al 6 reported a patient with idiopathic pulmonary arteritis who was positive for MPO‐ANCA.…”

Section: Discussionsupporting

confidence: 89%

“…Our case is similar to two reported cases of idiopathic pulmonary arteritis with respect to the 99m Tc-MAA perfusion defect in the entire unilateral lung, severe stenosis of the main PA, and pulmonary hypertension. 5,6 Uchiyama et al 6 reported a patient with idiopathic pulmonary arteritis who was positive for MPO-ANCA. They suspected that their case had polyangitis overlap syndrome with Takayasu's arteritis and MPO-ANCA-associated vasculitis, although there were no signs or symptoms of systemic arterial lesions.…”

Section: Discussionmentioning

confidence: 99%

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A case of pulmonary arteritis with stenosis of the main pulmonary arteries with positive myeloperoxidase‐antineutrophil cytoplasmic autoantibodies

Nakayama¹,

Uchida²,

Sim³

et al. 2000

Respirology

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A 53-year-old woman was referred to our hospital with the main symptoms of productive cough, fever and exertional dyspnoea. Chest X-ray revealed enlargement of the left hilar shadow and cavitary infiltration in the right upper lobe. 99mTechnetium-macroaggregated albumin (99mTc-MAA) perfusion scintigram showed complete hypoperfusion through the entire right lung. A pulmonary angiogram revealed stenotic lesions in the right and left main pulmonary arteries. Right cardiac catheterization showed an elevated right ventricular systolic pressure. There was no evidence of systemic arterial lesions nor vasculitis. The patient was positive for myeloperoxidase (MPO)-antineutrophil cytoplasmic autoantibodies (ANCA) (168 EU). The Mycobacterium avium complex sputum culture was positive. The pulmonary stenotic lesions were surgically resected. The resected pulmonary arterial lesions were pathologically diagnosed as non-specific vasculitis. The cavitary lesion disappeared 6 months after the surgery. Two years after the surgery, although the MPO-ANCA level had decreased to 12 EU, stenosis of the pulmonary arteries reappeared. It is suggested that the patient became positive for MPO-ANCA in association with the Mycobacterium avium complex infection, and that the presence of MPO-ANCA may not be related to the development of pulmonary stenosis of the main pulmonary arteries.

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Section: Discussionsupporting

confidence: 89%

Section: Discussionmentioning

confidence: 99%

A case of pulmonary arteritis with stenosis of the main pulmonary arteries with positive myeloperoxidase‐antineutrophil cytoplasmic autoantibodies

Nakayama¹,

Uchida²,

Sim³

et al. 2000

Respirology

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“…These disorders lack specific features of the better defined syndromes such as GCA (temporal), Takayasu's arteritis, WG, and CS syndrome [33]. The adult forms of IGV include: (1) disseminated visceral giant cell arteritis [3,4]; (2) juvenile systemic granulomatosis (which has also been noted in adults) [34]; (3) granulomatous phlebitis [35], and (4) isolated or localized variants [5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23]. These isolated IGVs have been described most frequently in the breast and female genital tract.…”

Section: Discussionmentioning

confidence: 99%

“…In a few cases idiopathic granulomatous vasculitis (IGV) presents confined to one organ involving smallto medium-sized arteries. This form has been described in the breast [5][6][7], female genital tract [8][9][10][11], liver [12,13], lung [14][15][16], small bowel [17] central nervous system [18], epididymis and spermatic cord [19][20][21], testis and epididymis [22], and the prostate [23].…”

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confidence: 99%

Isolated Idiopathic Granulomatous (Giant Cell) Vasculitis of the Prostate

Val‐Bernal¹,

Garijo

1999

Int J Surg Pathol

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Vasculitis involving the prostate is uncommon and usually reflects systemic disease. Isolated prostatic vasculitis is very rare. Most of these cases are morphologically categorized as polyarteritis nodosa. We describe a 72-year-old man with idiopathic granulomatous vasculitis limited to the prostate. The vasculitis was identified incidentally upon microscopic examination of the suprapubic prostatectomy specimen removed for nodular hyperplasia. The transmural inflammation was centered on muscular arteries and veins and it was accompanied by narrowing of the lumen. Vasculitis was not associated with any secondary changes such as infarct or postinfarct fibrosis. Immunophenotyping of the vascular inflammatory infiltrate disclosed abundant macrophages and T-lymphocytes and virtual absence of B-lymphocytes. The patient is alive and well on no treatment for a follow-up period of 14 months. To our knowledge this is the second case report of prostatic involvement in localized idiopathic granulomatous vasculitis. It is important to distinguish cases of isolated granulomatous vasculitis in the prostate from systemic disease, because the latter implies a poor prognosis and requires an aggressive treatment. Int J Surg Pathol 7(1): [53][54][55][56][57][58] 1999

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“…Idiopathic isolated pulmonary GCA is a rare condition in which clinical features are absent outside the lung. This condition may lead to dyspnea and peripheral pulmonary infarcts, but the diagnosis is most often made postmortem [67,68].…”

Section: Giant Cell Arteritismentioning

confidence: 99%

Pulmonary manifestations of large, medium, and variable vessel vasculitis

Adams

Zhang

Batra

et al. 2018

Respiratory Medicine

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The hallmark of vasculitis is autoimmune inflammation of blood vessels and surrounding tissues, resulting in an array of constitutional symptoms and organ damage. The lung is commonly targeted in the more familiar ANCAassociated small vessel vasculitidies, but large and medium vessel vasculitides, including Takayasu arteritis, giant cell arteritis, polyarteritis nodosa, Behcet's disease, and necrotizing sarcoid granulomatosis, may also feature prominent pulmonary involvement. Pulmonary manifestations of these conditions include pulmonary arterial aneurysms, pulmonary hypertension, diffuse alveolar hemorrhage, pulmonary nodules, and parenchymal infiltrates. An understanding of the diverse manifestations of vasculitis and a high index of clinical suspicion are essential to avoid delays in disease recognition that may result in permanent or life threatening morbidity. In this review, we outline the general clinical manifestations, pulmonary manifestations, diagnostic workup, imaging findings, and treatment of medium, large, and variable vessel vasculitides.

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Idiopathic Isolated Pulmonary Arteritis with Chronic Cor Pulmonale

Cited by 22 publications

References 4 publications

A case of pulmonary arteritis with stenosis of the main pulmonary arteries with positive myeloperoxidase‐antineutrophil cytoplasmic autoantibodies

A case of pulmonary arteritis with stenosis of the main pulmonary arteries with positive myeloperoxidase‐antineutrophil cytoplasmic autoantibodies

Isolated Idiopathic Granulomatous (Giant Cell) Vasculitis of the Prostate

Pulmonary manifestations of large, medium, and variable vessel vasculitis

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