Case Of Nodular Pulmonary Amyloidosis Research Articles

A 69-YEAR-OLD MAN WITH HEAVY ASBESTOS EXPOSURE, DYSPNEA, AND PLEURAL SOFT-TISSUE THICKENING

SESSION TITLE: Invasion of the PleuraSESSION TYPE: Case ReportsPRESENTED ON: 10/18/2022 11:15 am - 12:15 pmINTRODUCTION: Amyloidosis is a disease caused by autologous protein deposition in tissues in the form of fibrils. It can affect any organ and can be fatal if left untreated. Most common pulmonary involvement with amyloid is nodular amyloidosis. Pleural disease is very rare and presents most commonly as recurrent pleural effusion (1). It can mimic multiple diseases including malignant mesothelioma.CASE PRESENTATION: A 69-year-old male patient with a history of asbestos exposure presented for worsening dyspnea on exertion over 3 months accompanied with a dry cough. He worked installing asbestos from age 18-23. Physical exam was pertinent for decreased bibasilar breath sounds. Laboratory studies were unremarkable. PFTs showed severe restriction with severely reduced DLCO. CT chest showed bilateral pleural effusions and worsening multifocal pleural plaques compared to a prior CT chest. It was associated with pleural and soft tissue thickening invading the left upper mediastinum. PET CT showed low FDG uptake (Fig1A&B). Pleural fluid was exudative per Light's criteria. Microscopic evaluation of two pleural nodules showed extensive amyloid infiltration with a positive Congo red stain. Immunohistochemical stains were positive for CD20 and CD138 specific for small B-cells and plasma cells respectively (Fig1C&D). He was diagnosed with systemic amyloidosis and started on chemotherapyDISCUSSION: Our case represents a very rare presentation of pleural amyloidosis with disseminated amyloid lesions on the parietal, visceral and diaphragmatic pleura. Similar cases were only rarely reported in the literature. Whether extensive asbestos exposure can lead to pleural amyloidosis is unclear. Hiroshima et al described a case of nodular pulmonary amyloidosis in a patient with a history of asbestos exposure and suggested asbestos fibers as an etiology (2). The differential diagnosis of amyloidosis is broad including interstitial lung disease, malignant mesothelioma, pleural infection and malignancy. It has been thought to be strongly associated with lymphoproliferative disorders including plasma cell dyscrasias. A pathological diagnosis is crucial and evaluation for second organ involvement is vital for prognostication and gauging therapy. Furthermore, the finding of significantly reduced DACO suggests a process in addition to restriction affecting diffusion such as parenchymal involvement with amyloidosis or asbestosisCONCLUSIONS: In brief, the diagnosis of amyloidosis can be challenging as it can mimic other diseases including malignant mesothelioma. Our patient had a history of asbestos exposure and malignant mesothelioma was highly suspected. It is essential to keep a broad differential when approaching pleural diseases and recommend a thorough workup. First line therapy is stem cell transplant, Berk et al shows a significant survival rate in patients treated with SCT or chemotherapy compared to untreated patients (3).Reference #1: Dacic S, Colby TV, Yousem SA. Nodular Amyloidoma and Primary Pulmonary Lymphoma with Amyloid Production: A Differential Diagnostic Problem. Mod Pathol. 2000 Sep;13(9):934–40.Reference #2: Hiroshima K, Ohwada H, Ishibashi M, Yamamoto N, Tamiya N, Yamaguchi Y. Nodular pulmonary amyloidosis associated with asbestos exposure. Pathol Int. 1996 Jan;46(1):66–70.Reference #3: Berk JL, Keane J, Seldin DC, Sanchorawala V, Koyama J, Dember LM, et al. Persistent Pleural Effusions in Primary Systemic Amyloidosis. Chest. 2003 Sep;124(3):969–77.DISCLOSURES: No relevant relationships by Ahmed AlhusseinyNo relevant relationships by Jean-Pierre AssakerNo relevant relationships by Douglas Johnson SESSION TITLE: Invasion of the Pleura SESSION TYPE: Case Reports PRESENTED ON: 10/18/2022 11:15 am - 12:15 pm INTRODUCTION: Amyloidosis is a disease caused by autologous protein deposition in tissues in the form of fibrils. It can affect any organ and can be fatal if left untreated. Most common pulmonary involvement with amyloid is nodular amyloidosis. Pleural disease is very rare and presents most commonly as recurrent pleural effusion (1). It can mimic multiple diseases including malignant mesothelioma. CASE PRESENTATION: A 69-year-old male patient with a history of asbestos exposure presented for worsening dyspnea on exertion over 3 months accompanied with a dry cough. He worked installing asbestos from age 18-23. Physical exam was pertinent for decreased bibasilar breath sounds. Laboratory studies were unremarkable. PFTs showed severe restriction with severely reduced DLCO. CT chest showed bilateral pleural effusions and worsening multifocal pleural plaques compared to a prior CT chest. It was associated with pleural and soft tissue thickening invading the left upper mediastinum. PET CT showed low FDG uptake (Fig1A&B). Pleural fluid was exudative per Light's criteria. Microscopic evaluation of two pleural nodules showed extensive amyloid infiltration with a positive Congo red stain. Immunohistochemical stains were positive for CD20 and CD138 specific for small B-cells and plasma cells respectively (Fig1C&D). He was diagnosed with systemic amyloidosis and started on chemotherapy DISCUSSION: Our case represents a very rare presentation of pleural amyloidosis with disseminated amyloid lesions on the parietal, visceral and diaphragmatic pleura. Similar cases were only rarely reported in the literature. Whether extensive asbestos exposure can lead to pleural amyloidosis is unclear. Hiroshima et al described a case of nodular pulmonary amyloidosis in a patient with a history of asbestos exposure and suggested asbestos fibers as an etiology (2). The differential diagnosis of amyloidosis is broad including interstitial lung disease, malignant mesothelioma, pleural infection and malignancy. It has been thought to be strongly associated with lymphoproliferative disorders including plasma cell dyscrasias. A pathological diagnosis is crucial and evaluation for second organ involvement is vital for prognostication and gauging therapy. Furthermore, the finding of significantly reduced DACO suggests a process in addition to restriction affecting diffusion such as parenchymal involvement with amyloidosis or asbestosis CONCLUSIONS: In brief, the diagnosis of amyloidosis can be challenging as it can mimic other diseases including malignant mesothelioma. Our patient had a history of asbestos exposure and malignant mesothelioma was highly suspected. It is essential to keep a broad differential when approaching pleural diseases and recommend a thorough workup. First line therapy is stem cell transplant, Berk et al shows a significant survival rate in patients treated with SCT or chemotherapy compared to untreated patients (3). Reference #1: Dacic S, Colby TV, Yousem SA. Nodular Amyloidoma and Primary Pulmonary Lymphoma with Amyloid Production: A Differential Diagnostic Problem. Mod Pathol. 2000 Sep;13(9):934–40. Reference #2: Hiroshima K, Ohwada H, Ishibashi M, Yamamoto N, Tamiya N, Yamaguchi Y. Nodular pulmonary amyloidosis associated with asbestos exposure. Pathol Int. 1996 Jan;46(1):66–70. Reference #3: Berk JL, Keane J, Seldin DC, Sanchorawala V, Koyama J, Dember LM, et al. Persistent Pleural Effusions in Primary Systemic Amyloidosis. Chest. 2003 Sep;124(3):969–77. DISCLOSURES: No relevant relationships by Ahmed Alhusseiny No relevant relationships by Jean-Pierre Assaker No relevant relationships by Douglas Johnson

Monoclonal gammopathy of clinical significance : about a case of nodular pulmonary amyloidosis.

Amyloid proteins can infiltrate virtually all organs. Diagnosis is usually difficult owing to its diverse clinical presentation. Involvement of the lung is relatively common, but rarely symptomatic, and most commonly observed in localized (primary) forms of amyloidosis. We report here a case of nodular pulmonary amyloidosis that evolved over a 10-year period. A 61 year-old woman was referred for a suspicion of pulmonary amyloidosis. She has been diagnosed at the age of 30 with rheumatoid arthritis (RA) that required for years, hydroxychloroquine.

PRIMARY PULMONARY NODULAR AMYLOIDOSIS IN A FEMALE WITH UNDIAGNOSED SJOGREN'S SYNDROME

SESSION TITLE: Tuesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Pulmonary amyloidosis is characterized by extracellular deposition of insoluble, misfolded fibrils within the lung. Isolated pulmonary amyloidosis is a rare condition especially in association with Sjogren’s syndrome. Frequently asymptomatic, we report a case of nodular pulmonary amyloidosis in a young, Hispanic female who presented with dyspnea. CASE PRESENTATION: A 45 year-old female presented with shortness of breath and chest tightness for 3 years. Her symptoms progressed in frequency, functional status deteriorated and she experienced a 20-pound weight loss in the past year. Initial imaging 2 years prior revealed multiple lung nodules with a thin-walled cyst in the right middle lobe (RML). Quantiferon-TB Gold and coccidioidomycosis serology was negative. Pulmonary function testing (PFT) demonstrated a minimal obstructive lung defect with mild response to bronchodilator. Repeat imaging demonstrating increasing size of multiple pulmonary nodules, a now solid nodule of RML, and a 2.6 cm left suprasternal lymph node. Computed tomography (CT)-guided biopsy revealed nodular pulmonary amyloidosis confirmed with congo red stain. The patient was placed on a prednisone taper with improvement of symptoms. She continued to endorse multiple recurring subcutaneous nodules of both upper and lower extremities, dry mouth, dry eyes, cold fingers, and hair loss. Lab studies were notable for positive anti-SSA/Ro antibodies and rheumatoid factor (208 IU/mL), and negative cyclic citrullinated peptide antibody and antinuclear antibody. Serum protein electrophoresis identified a mild elevation of gamma globulins. Plaquenil, Rituximab and Methotrexate therapy was initiated. Repeat PFT had normal spirometry, lung volume and diffusion capacity. DISCUSSION: Pulmonary amyloidosis appears in three forms: pulmonary nodules, tracheobronchial, and diffuse alveolar septal. Nodular pulmonary amyloidosis is often an incidental finding and is indistinguishable from neoplasm or granulomatous lesions. Nodules are slowly enlarging and may cavitate or calcify. Histological examination is necessary to determine the diagnosis. In this case, untreated Sjögren’s syndrome with gammaglobinemia likely led to deposition of amyloid in lung. In literature, PFT are mostly normal with a small subset demonstrating restrictive pattern and even fewer with reduced diffusion. Our patient initially presented with obstructive pattern likely due to the large cyst compromising elastic recoil of the lungs. CONCLUSIONS: In primary nodular pulmonary amyloidosis, nodules are radiographically nonspecific and require biopsy for diagnosis. Amyloidosis should be included in the differential diagnosis of multiple pulmomary nodules. Management is not well defined due to the low incidence of the disease. When associated with Sjögren’s syndrome, there is reported success with treatment of steroids and immunosuppression. Reference #1: Xian, Jonathan Z. et all. "A 45-Year-Old Woman With Multiple Pulmonary Nodules and Sjogren Syndrome.” CHEST 2019;155(2):e51-154. Reference #2: Heraganahally, Subash et all. "Pulmonary AL - amyloidosis masquerading as lung malignancy in an Australian Indigenous patient with Sjogren's syndrome.” Respiratory Medicine Case Reports 2019;26:94-97 Reference #3: Garcia-Sanchez, Aldara et all. "Amyloidosis as a Cause of Cystic Pulmonary Fibrosis Associated With Pulmonary Nodules.” Arch Bronconeumol 2018;54(9): 481-494. DISCLOSURES: No relevant relationships by Aarti Mittal, source=Web Response No relevant relationships by Erin Wiltchik, source=Web Response

A Case of Nodular Pulmonary Amyloidosis in a Patient With Mixed Connective Tissue Disease (MCTD)

A Case of Nodular Pulmonary Amyloidosis in a Patient With Mixed Connective Tissue Disease (MCTD)

Single Nodular Pulmonary Amyloidosis: Case Report.

Amyloidosis is defined as the presence of extra-cellular deposits of an insoluble fibrillar protein, amyloid. The pulmonary involvement of amyloidosis is usually classified as tracheobronchial, parenchymal nodular, or diffuse alveolar septal. A single nodular lesion can mimic various conditions, including malignancy, pulmonary tuberculosis, and fungal infection. To date, only one case of nodular pulmonary amyloidosis has been reported in Korea, a case involving multiple nodular lesions. Here, we report and discuss the case of a patient having single nodular amyloidosis.

A Case of Nodular Pulmonary Amyloidosis in a Patient With Mixed Connective Tissue Disease (MCTD)

A Case of Nodular Pulmonary Amyloidosis in a Patient With Mixed Connective Tissue Disease (MCTD)

Nodular pulmonary amyloidosis and Sjögren's syndrome in a patient treated with intermittent hemodialysis

In the available literature, we have found the descriptions of 5 cases of nodular pulmonary amyloidosis associated with Sjögren's syndrome. In our practice, such a case has occurred in a patient with chronic renal failure. A 53-year-old woman underwent nephrological, rheumatological, and pulmonological examinations because of end-stage renal disease with a small cirrhotic kidney in renal ultrasound examination, pulmonary nodules, and xerophthalmia. Serological data revealed a slightly positive rheumatoid factor, antinuclear antibodies, anti-SS-A, anti-SS-B, and anti-RNP/Sm antibodies. Schirmer's test was positive on both sides and Sjögren's syndrome was recognized. Pulmonological examinations (the chest radiograph and CT scan, bronchofiberoscopy, culture of bronchial washings, bronchial biopsy, pleural effusion analysis, and a thick-needle biopsy) failed to determine the etiology of nodular changes in lungs. Immunofluorescence studies in the skin biopsy specimen showed IgM-positive staining. After 2 years of treatment with IHD, a toracoscopy was performed with enucleation of the nodules from the right lung. Histological examination showed massive deposits of amyloid, which allowed for a diagnosis of diffusive nodular pulmonary amyloidosis.

Three Cases of the Nodular Pulmonary Amyloidosis with A longterm Observation

Longterm observation with chest radiograph and computed tomography (CT) scan was performed for pulmonary amyloidosis. There are few reports of primary pulmonary amyloidosis with a longterm observation. We encountered three cases of nodular pulmonary amyloidosis observed by intermittent chest radiograph or CT for 5 years or more. The patients were a 54-year-old man, and 67- and 68-year old women. For diagnosis, transbronchial biopsy and percutaneous lung biopsy were performed. Amyloid nodules grew slowly and two cases showed findings of cavity and calcification.

Clonal Light Chain Restricted Primary Intrapulmonary Nodular Amyloidosis

Primary intrapulmonary nodular amyloidosis is a rare form of immunoglobulin associated amyloidosis, also falling under the alternative appellation of amyloidoma. Although amyloidomas in other organ sites may be reflective of a localized or more generalized plasma cell dyscrasia, in the context of its presentation in the lung the presumptive basis has long been held to be one of chronic inflammation. We encountered 2 patients with nodular amyloidosis in whom the pathologic examination disclosed the basis to be one of a light chain restricted clonal lymphocytic plasma cell infiltrate, although without morphologic features of coexisting pulmonary lymphoma. These 2 patients serve to underscore the potential categorization of some cases of pulmonary nodular amyloidosis as a form of low grade B cell lymphoproliferative disease.

Nodular pulmonary amyloidosis: a case report and review of literature

Since 1974, 20 new cases of nodular pulmonary amyloidosis have been reported in the English literature. We present a new case and a review of the literature.

Nodular pulmonary amyloidosis with extrapulmonary involvement

Although approximately 50 cases of nodular pulmonary amyloidosis have been described in the literature, few of them have involved bilateral confluent disease. An exceptional case with disease extensive enough to cause death by respiratory insufficiency is reported. A remarkable degree of calcification in the subpleural areas, with osseous and chondroid metaplasia, characterized the amyloid deposits. Amyloid was confined to the respiratory tract except for contiguous areas (vertebral body cortex, epicardial fat, and hilar lymph nodes). A review and update of the literature since 1974 is included.