Primary amyloidosis with multiple pulmonary nodular lesions and IgA nephropathy-like renal involvement

Ml, Onozato; Tojo, Akihiro; Ogura, Shota; Asaba, Kensuke; Goto, Atsuo; Fujita, Teruo

doi:10.5414/cnp60134

Cited by 7 publications

(3 citation statements)

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“…It is defined as one or more nodular amyloid deposits involving the lung. It usually represents localised AL (immunoglobulin light chain) or AL/AH (mixed immunoglobulin light chain/heavy chain) amyloidosis [16,17], but rare cases of systemic AL, localised AA, localised ATTRwt and localised Aβ2M/AL (mixed β2-microglobulin/immunoglobulin light chain) amyloidosis have been reported [18][19][20][21][22][23][24]. Of note, localised AL amyloidosis is not unique to the lungs and the tracheobronchial tree.…”

Section: Nodular Pulmonary 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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Section: Nodular Pulmonary Amyloidosismentioning

confidence: 99%

The lung in amyloidosis

et al. 2017

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“…[53][54][55][56][57] IgA paraproteinemias related to myeloma, macroglobulinemia and amyloidosis could present with DAH 52 or a nodular pattern in the lung. 70 Finally, an overlap syndrome between IgAN and ANCA-related vasculitis has been recently described. 71 ANCA-positive IgAN patients are older, have greater evidence of inflammation, lower hemoglobin, more fibrinoid necrosis in renal biopsies and more pulmonary disease when compared to patients with IgAN alone.…”

Section: Discussionmentioning

confidence: 99%

Diffuse alveolar hemorrhage in IgA nephropathy: case series and systematic review of the literature

et al. 2016

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These findings are similar to our previous observations of Henoch-Schonlein purpura (HSP)-related alveolar hemorrhage, highlighting the similarities of these related syndromes. Multicentric studies of IgAN and HSP-related pulmonary renal syndrome with a standard protocol are needed to define their similarities and differences, optimum suppression and its role in preventing renal progression in this setting.

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“…It usually represents localized AL or AL/AH (mixed immunoglobulin light chain/heavy chain) amyloidosis, 33,34 but rare cases of systemic AL, localized AA, localized wildtype ATTR, and localized Ab 2 M/AL (mixed b 2 -microglobulin/immunoglobulin light chain) amyloidosis have been reported (Table 2). [35][36][37][38][39][40][41] Of note, localized AL amyloidosis is not unique to the lungs and the tracheobronchial tree (see below). It can also occur at other sites including the larynx, 42 urinary bladder, 43 and colon.…”

Section: Pathologymentioning

confidence: 99%

Amyloidosis of the Lung

Khoór¹,

Colby²

2017

Archives of Pathology &Amp; Laboratory Medicine

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Context.—Amyloidosis is a heterogeneous group of diseases characterized by the deposition of congophilic amyloid fibrils in the extracellular matrix of tissues and organs. To date, 31 fibril proteins have been identified in humans, and it is now recommended that amyloidoses be named after these fibril proteins. Based on this classification scheme, the most common forms of amyloidosis include systemic AL (formerly primary), systemic AA (formerly secondary), systemic wild-type ATTR (formerly age-related or senile systemic), and systemic hereditary ATTR amyloidosis (formerly familial amyloid polyneuropathy). Three different clinicopathologic forms of amyloidosis can be seen in the lungs: diffuse alveolar-septal amyloidosis, nodular pulmonary amyloidosis, and tracheobronchial amyloidosis. Objective.—To clarify the relationship between the fibril protein–based amyloidosis classification system and the clinicopathologic forms of pulmonary amyloidosis and to provide a useful guide for diagnosing these entities for the practicing pathologist. Data Sources.—This is a narrative review based on PubMed searches and the authors' own experiences. Conclusions.—Diffuse alveolar-septal amyloidosis is usually caused by systemic AL amyloidosis, whereas nodular pulmonary amyloidosis and tracheobronchial amyloidosis usually represent localized AL amyloidosis. However, these generalized scenarios cannot always be applied to individual cases. Because the treatment options for amyloidosis are dependent on the fibril protein–based classifications and whether the process is systemic or localized, the workup of new clinically relevant cases should include amyloid subtyping (preferably with mass spectrometry–based proteomic analysis) and further clinical investigation.

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Primary amyloidosis with multiple pulmonary nodular lesions and IgA nephropathy-like renal involvement

Cited by 7 publications

References 0 publications

The lung in amyloidosis

The lung in amyloidosis

Diffuse alveolar hemorrhage in IgA nephropathy: case series and systematic review of the literature

Amyloidosis of the Lung

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