Pulmonary Hypertension From Prominent Vascular Involvement in Diffuse Amyloidosis

Shiue, Shyi-Tang; McNally, Daniel P.

doi:10.1001/archinte.1988.00380030193030

Cited by 31 publications

(12 citation statements)

References 15 publications

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“…In contrast to reported cases of amyloidosis and pulmonary hypertension where the average survival has been less than two months [2], the patient described in this paper is doing well after 1 year from the diagnosis and no other manifestations of either multiple myeloma or amyloidosis are evident. After a consideration by hematologists, patient has been started on melphalan-prednison protocol for multiple myeloma.…”

Section: Discussioncontrasting

confidence: 93%

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Pulmonary hypertension as a dominant clinical picture in a case of amyloidosis and smoldering multiple myeloma

Lehtonen¹,

Kettunen²

2007

International Journal of Cardiology

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Section: Discussioncontrasting

confidence: 93%

“…In addition to amyloid fibril deposition into cardiac tissue, deposition may occur also in the coronary arteries and pulmonary blood vessels [1]. Pulmonary hypertension is a rare late manifestation of amyloidosis [1,2]. We describe a patient with AL amyloidosis and pulmonary hypertension without any other clinical manifestation.…”

Section: Introductionmentioning

confidence: 96%

Pulmonary hypertension as a dominant clinical picture in a case of amyloidosis and smoldering multiple myeloma

Lehtonen¹,

Kettunen²

2007

International Journal of Cardiology

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“…Vascular deposits are common, but rarely clinically relevant, although they can give rise to pulmonary hypertension [45,52,53]. In patients with AL amyloidosis, the most common aetiologies of pulmonary hypertension are left-sided restrictive cardiomyopathy from amyloid deposition (group II pulmonary hypertension) or diffuse lung disease (group III pulmonary hypertension) [54,55].…”

Section: Diffuse Alveolar-septal Amyloidosismentioning

confidence: 99%

The lung in amyloidosis

et al. 2017

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Amyloidosis is a disorder caused by misfolding of autologous protein and its extracellular deposition as fibrils, resulting in vital organ dysfunction and eventually death. Pulmonary amyloidosis may be localised or part of systemic amyloidosis.Pulmonary interstitial amyloidosis is symptomatic only if the amyloid deposits severely affect gas exchange alveolar structure, thus resulting in serious respiratory impairment. Localised parenchymal involvement may be present as nodular amyloidosis or as amyloid deposits associated with localised lymphomas. Finally, tracheobronchial amyloidosis, which is usually not associated with evident clonal proliferation, may result in airway stenosis.Because the treatment options for amyloidosis are dependent on the fibril protein type, the workup of all new cases should include accurate determination of the amyloid protein. Most cases are asymptomatic and need only a careful follow-up. Diffuse alveolar-septal amyloidosis is treated according to the underlying systemic amyloidosis. Nodular pulmonary amyloidosis is usually localised, conservative excision is usually curative and the long-term prognosis is excellent. Tracheobronchial amyloidosis is usually treated with bronchoscopic interventions or external beam radiation therapy.

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“…With time, this leads to interference with gas exchange and respiratory failure. [7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25] Thirdly, extensive amyloid deposits in the walls of capillaries, arterioles, and medium-sized muscular pulmonary arteries may overshadow the interstitial involvement leading to the early appearance of cor pulmonale, as was the case in this report. 12 ' 22 Table 1 summarizes the reported cases of alveolar septal amyloidosis from either primary amyloidosis or multiple myeloma.…”

Section: Discussionmentioning

confidence: 97%