Amyloidosis and Pleural Disease

doi:10.1378/chest.98.1.20

Chest

Volume 98, Issue 1, July 1990, Pages 20-23

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Pleural involvement with systemic amyloidosis has been reported rarely in the literature. Diagnosis of this entity by percutaneous needle biopsy of the pleura has been described only in two prior case reports. We describe five patients in whom the diagnosis of pleural amyloidosis was established by Cope needle biopsy during evaluation of pleural effusions of indeterminate cause. Three patients presented with a history suggestive of multiorgan disease and a pleural biopsy performed despite a transudative effusion demonstrated amyloid infiltration of the pleura, obviating the need for other organ biopsies. We conclude that in patients with pleural effusions, if history suggests multiorgan involvement and there is suspicion for amyloidosis, then a closed pleural biopsy with special stains for amyloid should be performed even if the effusions are transudative. This may be the diagnostic procedure of choice in such patients.

Section snippets

Case 1

A 71-year-old woman presented in April 1987 for evaluation of a two-year history of progressive dyspnea on exertion and right pleural effusion. She had a two-year history of seronegative rheumatoid arthritis and Sjögren's syndrome. In August 1986 she underwent a small bowel biopsy for evaluation of chronic diarrhea and was diagnosed as having amyloidosis of the bowel. A thoracentesis was performed and the fluid characteristics are summarized in Table 2. A Cope needle biopsy of the pleura was

Discussion

Pleural effusions appear to occur not infrequently in patients with systemic amyloidosis, 30 percent in one series.¹⁰ The cause for the pleural effusions is most often congestive heart failure. Indeed, a large review of 333 patients with systemic amyloidosis from 13 studies revealed that the incidence of histologic involvement of the heart was 66 percent, with 46 percent incidence of clinically evident congestive heart failure, with or without pleural efiusions.¹⁰ Other data suggest that there

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Cited by (44)

Pulmonary manifestations of amyloidosis
2023, Respiratory Medicine
Amyloidosis is caused by abnormal protein deposition in various tissues, including the lungs. Pulmonary manifestations of amyloidosis may be categorized by areas of involvement, such as parenchymal, large airway and pleural involvement. We describe four distinct manifestations of amyloidosis involving the lung and review their clinical, radiological and pathological features and summarize the evidence for treatment in each of these presentations. We describe alveolar-septal amyloidosis, cystic amyloid lung disease, endobronchial amyloidosis and pleural amyloidosis.
The spectrum of pulmonary amyloidosis
2023, Respiratory Medicine
Amyloidosis is a disease caused by misfolded proteins that deposit in the extracellular matrix as fibrils, resulting in the dysfunction of the involved organ. The lung is a common target of Amyloidosis, but pulmonary amyloidosis is uncommonly diagnosed since it is rarely symptomatic. Diagnosis of pulmonary amyloidosis is usually made in the setting of systemic amyloidosis, however in cases of localized pulmonary disease, surgical or transbronchial tissue biopsy might be indicated. Pulmonary amyloidosis can be present in a variety of discrete entities. Diffuse Alveolar septal amyloidosis is the most common type and is usually associated with systemic AL amyloidosis. Depending on the degree of the interstitial involvement, it may affect alveolar gas exchange and cause respiratory symptoms. Localized pulmonary Amyloidosis can present as Nodular, Cystic or Tracheobronchial Amyloidosis which may cause symptoms of airway obstruction and large airway stenosis. Pleural effusions, mediastinal lymphadenopathy and pulmonary hypertension has also been reported.
Treatment of all types of pulmonary amyloidosis depends on the type of precursor protein, organ involvement and distribution of the disease. Most of the cases are asymptomatic and require only close monitoring. Diffuse alveolar septal amyloidosis treatment follows the treatment of underlying systemic amyloidosis. Tracheobronchial amyloidosis is usually treated with bronchoscopic interventions including debulking and stenting or with external beam radiation. Long-term prognosis of pulmonary amyloidosis usually depends on the type of lung involvement and other organ function.
A 54-Year-Old Man With Anasarca, Dyspnea, and Recurrent Bilateral Pleural Effusions
2017, Chest
A 54-year-old African-American man presented with 2 years of progressively worsening dyspnea and anasarca. Over the past 6 months he gained 30 lbs with worsening lower extremity, abdominal wall, and scrotal edema. A recent workup for cardiac, renal, and liver disease, including two-dimensional echocardiogram, liver and renal function tests, and abdominal ultrasound, was unremarkable. He reported a 15-pack year history of smoking and quit 3 years ago. Chest radiograph at that time revealed bilateral pleural effusions that were both reportedly milky in appearance when drained by thoracenteses.
Primary systemic amyloidosis: A rare cause for pleural effusion
2014, Respiratory Medicine Case Reports
Citation Excerpt :
Pleural effusions in patients with amyloidosis which is a rare cause for massive effusion can cause dyspnoea and may require repeated thoracentesis to relieve symptoms as was the situation in our case which finally required intercostal tube drainage for dyspnoea management. One of the first reports of amyloid-associated pleural effusion included five patients by Kavuru et al. [10]. These authors found that pleural effusions in the majority of their patients (60% in their series) were believed to be related to either congestive heart disease or nephrotic syndrome, and only 40% of pleural effusions were “idiopathic”.
Pleural effusion is a common problem dealt by most of the practicing clinicians. Some causes for pleural effusion are less often considered as a differential diagnosis owing to its rarity. Here we report a case of renal amyloidosis on alternate day haemodialysis for about two months time presenting with left sided pleural effusion. On evaluation this turned out to be a case of amyloidosis on thoracoscopic pleural biopsy suggesting the possibility of Primary systemic amyloidosis.
A 71-year-old woman with an unusual cause for pleural effusions
2011, Chest
Citation Excerpt :
Furthermore, Berk et al7 found that aggressive diuretic therapy was unsuccessful at resolving persistent AL amyloidosis effusions, which is likely due to an impaired ability of the lymphatic system to reabsorb excess fluid. Multiple case reports have described a plethora of amyloid nodules measuring up to 5 mm in diameter on patients' parietal pleura.9–12 The mechanical occlusion of the parietal stomata is thought to be the cause for the persistence of pleural effusions in AL amyloidosis.
Pulmonary amyloidosis and non-amyloid immunoglobulin deposits
2008, Revue des Maladies Respiratoires
Les amyloses sont caractérisées à l’histopathologie par des dépôts tissulaires de substance amyloïde fibrillaire spécifiquement colorée par le rouge Congo avec une biréfringence en lumière polarisée. Cette propriété est liée à une structure physique en feuillet torsadé bêta-plissé, dénominateur commun des amyloses dont plus de vingt protéines différentes peuvent être les précurseurs.
La plus fréquente des amyloses est l’amylose AL, d’origine immunoglobulinique, qui peut être limitée à un organe ou systémique (avec atteinte cardiaque prédominante).
L’amylose limitée à l’appareil bronchopulmonaire s’exprime sous forme de dépôts trachéobronchiques en plaques diffuses pouvant entraîner des sténoses, ou de nodules ou masses parenchymateuses. L’amylose interstitielle pulmonaire diffuse symptomatique est très rare, survenant dans le cadre d’une amylose systémique AL avec des dépôts amyloïdes atteignant la zone d’échanges gazeux alvéolocapillaire.
L’amylose peut se manifester également par une hypertension pulmonaire, des adénopathies amyloïdes hilaires et médiastinales, une atteinte pleurale.
L’amylose AL peut également être associée localement à un lymphome pulmonaire.
Plus rarement, des dépôts immunoglobuliniques non fibrillaires, non colorés par le rouge Congo, peuvent se produire au niveau pulmonaire sous forme de nodules ou de lésions kystiques. Exceptionnellement des dépôts immunoglobuliniques peuvent prendre une structure cristalline tissulaire ou intrahistiocytaire.
J.-F. Cordier
The amyloidoses are characterised histopathologically by the tissue deposition of fibrillar amyloid, specifically stained by Congo red and birefringent under polarised light. This characteristic is linked to a β-folded structural configuration that is the common denominator of the amyloidoses which may have more than twenty distinct protein precursors.
The most common is AL amyloidosis which is of immunoglobulin origin. It may be organ limited, or systemic (with predominant cardiac involvement). Limited bronchopulmonary amyloidosis, usually AL, may manifest itself as either tracheobronchial deposits or parenchymal nodules or masses. Diffuse interstitial pulmonary amyloidosis with clinical manifestations is rare and usually associated with systemic AL amyloidosis and deposits involving the alveolar-capillary gas exchange zone. Amyloidosis may also manifest itself as pulmonary hypertension, amyloid hilar and mediastinal adenopathy or pleural involvement. AL amyloidosis may be associated locally with pulmonary lymphoma.
Occasionally, non-fibrillar, Congo red-negative, immunoglobulin deposits may occur, presenting as parenchymal pulmonary nodules or cysts. Exceptionally immunoglobulin deposits may show a cellular or extra-cellular crystalline structure.

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: Manuscript received August 24; revision accepted November 10.

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Chest

Introducing New Members of the Editorial Board Clinical InvestigationAmyloidosis and Pleural Disease

Section snippets

Case 1

Discussion

Am J Med

Chest

Chest

Am J Cardiol

Chest

Chest

The lung in generalized amyloidosis

Acta Pathol Jpn

Amyloidosis: review of 236 cases

Medicine

Amyloidosis

N Engl J Med

Introducing New Members of the Editorial Board Clinical Investigation
Amyloidosis and Pleural Disease