Amyloidosis presenting as bilateral transudative pleural effusions with normal cardiac investigations: a case report

Our plain-English summary of Amyloidosis presenting as bilateral transudative pleural effusions with normal cardiac investigations: a case report, by J.H. Briggs, W.G. Singleton, M.M. Burke, et al (Cases Journal, 2009).

The study

• Authors: J.H. Briggs, W.G. Singleton, M.M. Burke, L.A. Hart, R.J. Parker
• Journal: Cases Journal
• Published: 2009;2:6963
• Type of article: Case report
• Cited by: 5 articles, as counted by NIH iCite
• Read it: PubMed · Publisher · Free full text

Abstract

A 66-year-old man with a diagnosis of monoclonal gammopathy of unknown significance was referred for investigation of bilateral transudative pleural effusions by the cardiology team. Echocardiography, myocardial perfusion scanning and left heart catheterisation were all normal or non diagnostic. Given significant occupational asbestos exposure in his twenties he underwent thoracoscopic pleural biopsy. This showed fibrous inflammation only. He subsequently developed proteinuria and peripheral oedema. Reanalysis of the pleural biopsy specimen for amyloidosis was positive. Pleural disease is an uncommon presentation of systemic amyloidosis. The aetiology of the pleural effusions is unclear and is not simply a consequence of cardiac or renal impairment.

In plain English

This case report described one patient, a 66-year-old white British man referred by cardiologists to a respiratory clinic with fluid around both lungs (bilateral pleural effusions). For 5 years he had had IgG kappa monoclonal gammopathy of undetermined significance (MGUS), a blood protein abnormality. He had strong occupational asbestos exposure in the building trade in his twenties. Heart tests showed good heart function and only a minor (30%) narrowing of one coronary artery. The fluid was of the transudative type and contained no cancer cells. A chest CT scan showed only the effusions, with no evidence of prior asbestos exposure or cancer. A pleural biopsy showed chronic inflammation with fibrosis and hyalinized collagenous plaques, described as in keeping with his asbestos history, but no cancer. He later developed protein in his urine and marked swelling. The original biopsy was then re-examined with Congo red stain, which the authors do not use routinely, and showed amyloid. He was diagnosed with systemic AL amyloidosis and died 4 months after diagnosis. The authors concluded that systemic amyloidosis should be considered as a possible cause of unexplained pleural effusions.

We wrote this plain-English summary from the full article with the help of AI, and a second AI model checked each sentence against the article. It is not the authors' wording; the abstract and the article are their own account.

What the study did and found

• Design: case report (single patient)
• Who was studied: One 66-year-old white British man referred from cardiology to a respiratory out-patient clinic in the UK, with hypertension and IgG kappa MGUS for 5 years
• Exposure: Strong occupational asbestos exposure while working in the building trade in his twenties; fiber type not stated
• Main results:
  • Heart investigations showed good left and right ventricular function, no significant valve disease and normal cardiac structure, with no reversible ischaemia on perfusion scanning
  • Pleural fluid tested on two occasions was transudative by Light's criteria (pleural protein 30 g/l, serum protein 91 g/l, lactate dehydrogenase 61 IU/l), with no malignant cells
  • Chest CT showed only bilateral pleural effusions, with no evidence of prior asbestos exposure or of malignancy
  • The thoracoscopic pleural biopsy showed chronic inflammatory change with fibrosis and several hyalinized collagenous plaques described as in keeping with asbestos exposure, with no malignant disease
  • After surgery he developed marked oedema, a serum albumin of 22 g/l and proteinuria of 1.3 g per 24 hours
  • Re-analysis of the original biopsy with Congo red stain, which the authors do not use routinely on pleural biopsies, showed amyloid in the areas of fibrosis, and systemic AL amyloidosis was diagnosed
  • He was treated with oral dexamethasone and melphalan and died 4 months after diagnosis
• The authors' conclusion: The authors concluded that systemic amyloidosis should be considered as a potential cause of unexplained pleural effusions. They said it can be diagnosed on pleural biopsy, but only if the diagnosis is suspected so that the correct stain is used.

Topics

Medical terms in this study, explained in our Lexicon: Bilateral, Pleural Effusion, Monoclonal, Asbestos Exposure, Pleural Biopsy, Inflammation.

Related studies

• Diffuse pulmonary mesothelioma presenting as bilateral lymphangitis — A.P. Chahinian (1986)
• An investigation of the penetration of ingested asbestos into the normal and abnormal intestinal mucosa of the rat — M.E. Meek, P. Grasso (1983)
• Calretinin. A selective marker of normal and neoplastic mesothelial cells in serous effusions — M.C. Barberis, M. Faleri, et al. (1997)
• Defect in recruiting effector memory CD8+ T-cells in malignant pleural effusions compared to normal pleural fluid — A. Scherpereel, B.D. Grigoriu, et al. (2013)
• The value of anti-calretinin antibody in the differential diagnosis of normal and reactive mesothelia versus metastatic tumors in effusion cytology — H. Nagel, B. Hemmerlein, et al. (1998)
• Investigation of pleural effusion: comparison between fibreoptic thoracoscopy, needle biopsy and cytology — W.M. Edmondstone (1990)

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