Pulmonary Amyloidosis Research Articles

Two cases of monoclonal nodular pulmonary amyloidosis and review of the literature

Nodular pulmonary amyloidosis (NPA) is an uncommon pathology of insoluble protein depositing in pulmonary parenchyma. This localized pulmonary form of amyloidosis is most often found to contain combinations of kappa and lambda immunoglobulin light chain and immunoglobulin heavy chain proteins with a polyclonal lymphoplasmacystic infiltrate. Herein we present two cases of NPA of the rarely reported monoclonal (light-chain restricted) form with review of the literature and discussion of the clinical, radiographic, and histologic features of NPA.

Two cases of monoclonal nodular pulmonary amyloidosis and review of the literature

Nodular pulmonary amyloidosis (NPA) is an uncommon pathology of insoluble protein depositing in pulmonary parenchyma. This localized pulmonary form of amyloidosis is most often found to contain combinations of kappa and lambda immunoglobulin light chain and immunoglobulin heavy chain proteins with a polyclonal lymphoplasmacystic infiltrate. Herein we present two cases of NPA of the rarely reported monoclonal (light-chain restricted) form with review of the literature and discussion of the clinical, radiographic, and histologic features of NPA.

Intralobular septal thickening on chest CT in a patient with pulmonary amyloidosis: a rare case study

A 54-year-old female presented with chronic dry cough and dyspnoea over 3 months and was referred to our outpatient clinic. She had no history of smoking, allergy or respiratory disorders....

A Rare Case of PET Scan Positive Amyloidosis in Sjögren Syndrome

SESSION TITLE: Fellow Case Report Poster - Procedures SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM INTRODUCTION: Sjogren syndrome(SS), is a chronic inflammatory disorder characterized by lymphocytic infiltration of exocrine glands. Pulmonary amyloidosis secondary to Sjogren Syndrome is rare with a paucity of data on its clinical and radiological manifestations. CASE PRESENTATION: A 65 year old female with past medical history of COPD and Sjogren’s syndrome was found to have 1.6 cm right lower lobe and 1.4 cm left upper lobe irregular nodules as an incidental CT scan finding after a traumatic injury to her chest.Her review of systems was significant for dry mouth and non productive cough. A PET scan obtained as a follow up showed moderate FDG uptake with SUV of 4.0 and 2.3 respectively. Due to intermediate risks with a history of smoking and family history of breast cancer , tissue specimen via transbronchial biopsy were obtained to rule out cancer . Histology showed amorphous tissue material that stained positive for Congo red consistent with diagnosis of amyloidosis. She subsequently opted to have the right lower lobe nodule resected via a VATS procedure that conformed the diagnosis of amyloidosis. DISCUSSION: Sjogren syndrome is an autoimmune disease associated with a multitude of pulmonary lympho proliferative disorders. The extracellular deposition of its insoluble amyloid proteins can present with an interstitial and nodular pattern. The most common manifestation is the bilateral pulmonary nodules.The nodules have a peripheral lower lobe distribution and tend to have no FDG activity on PET scan . The transthoracic resection is the recommended approach for diagnosis, however advanced bronchoscopic techniques including electromagnetic navigation bronchoscopy could be utilized to biopsy and diagnose the condition. Bleeding remains a significant risk secondary to pulmonary vascular involvement. We report a rare case of bilateral pulmonary nodules in a patient with Sjogren syndrome that had significant FDG uptake and was diagnosed as amyloidosis. CONCLUSIONS: Amyloidosis should be considered in the differential diagnosis of bilateral pulmonary nodule in patients with Sjogren’s syndrome. Tissue samples sould be obtained from nodules with increased FDG activity or intermediate risks to rule out active malignancy Reference #1: FDG PET Scanning in Pulmonary Amyloidosis; Baqir et al ; J Nucl Med 2014; 55:565-568 Reference #2: Pulmonary amyloidosis in Sjogren Syndrome: A case report and systemic review of the literature; Rajagopala et al; Respirology 2010; 15:860-868 DISCLOSURE: The following authors have nothing to disclose: Hammad Arshad, Meilin Young, Rajashekar Adurty No Product/Research Disclosure Information

A Rare Case of Pulmonary Amyloidosis Secondary to Multiple Myeloma

A Rare Case of Pulmonary Amyloidosis Secondary to Multiple Myeloma

TBLB で診断し得た肺アミロイドーシスの 1 例

TBLB で診断し得た肺アミロイドーシスの 1 例

TBLB にて AL-κ蛋白が証明された原発性結節性肺アミロイドーシスの 1 例

TBLB にて AL-κ蛋白が証明された原発性結節性肺アミロイドーシスの 1 例

Loculated Secondary Spontaneous Pneumothorax: A Very Rare Complication of Nodular Pulmonary Amyloidosis

Loculated Secondary Spontaneous Pneumothorax: A Very Rare Complication of Nodular Pulmonary Amyloidosis

Lymphome du MALT pulmonaire et syndromes paranéoplasiques

Primary pulmonary lymphoma is a rare disease; diagnosis is often delayed because of atypical clinical presentation and slow progression. A 42-year-old woman consulted because of haemoptysis. Chest CT-scan showed multiple nodular calcified masses. A lung biopsy led to the diagnosis of pulmonary amyloidosis with pulmonary MALT lymphoma (mucosa-associated lymphoid tissue). The patient developed two paraneoplastic syndromes: a hypertrophic osteoarthropathy and mucinosis. Multiple nodular amyloidosis can be a mode of presentation for pulmonary lymphoma. Paraneoplastic syndromes must be systematically considered and can help in early diagnosis of the disease and its relapse.

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)

Amyloid-associated Cystic Lung Disease.

Amyloid-associated cystic lung disease is rare. It can be associated with collagen vascular disease (CVD). We aimed to describe the clinical, radiology, and pathology findings of this entity. We reviewed the records of subjects having biopsy-proven pulmonary amyloidosis with cystic lung disease demonstrated at high-resolution computed tomography (HRCT). Demographic characteristics, association with CVD and lymphoproliferative disorders, pulmonary function, and pathology results were reviewed. HRCT appearance was analyzed for number, size, distribution, and morphology of cysts and nodules. Twenty-one subjects (13 female, eight male; median age, 61 years) with cystic pulmonary amyloidosis were identified. The most common pulmonary function patterns were normal (42%) and obstructive (32%). The most common associated CVD was Sjögren syndrome (10 of 12). Nine subjects had no CVD. Cysts tended to be multiple (≥ 10 in 14 of 21, 67%), round (21 of 21, 100%), or lobulated (20 of 21, 95%); thin-walled (< 2mm in 17 of 21, 81%); and of small (< 1cm in 21 of 21, 100%) to moderate (1-2cm in 17 of 21, 81%) size. Peribronchovascular (19 of 21, 90%) and subpleural (19 of 21, 90%) cysts were typically present. Seventeen (81%) subjects had lung nodules, which tended to be numerous (≥ 10 in 10 of 17, 59%; 4-9 in six of 17, 35%). At least one calcified nodule was present in 14 of 17subjects (82%). Pulmonary mucosa-associated lymphoid tissue lymphoma (MALToma) was diagnosed in seven subjects (33%). Amyloid-associated cystic lung disease can occur with or without underlying CVD. Cystic lesions in the lung are commonly numerous, often are peribronchovascular or subpleural, and are frequently associated with nodular lesions that are often calcified. MALToma was a relatively frequent association.

Long-term observation of a case of localized nodular pulmonary amyloidosis

Long-term observation of a case of localized nodular pulmonary amyloidosis

膀胱癌精査中に発見された限局性結節性肺アミロイドーシスの1例

症例は75歳，男性．肉眼的血尿を主訴に当院泌尿器科を受診し，精査にて多発膀胱癌と診断された．精査中に胸部X線で右中肺野に異常陰影を指摘された．胸部CTで右肺S6に径2cm大の結節影を認め，転移性肺腫瘍を疑い当科紹介となった．膀胱癌からの転移か否かで治療方針が変わるため，確定診断目的に胸腔鏡下右肺部分切除を施行した．病理組織学検査で，結節性肺アミロイドーシスと診断した．確定診断後の全身検索では，多発性骨髄腫の合併は否定的で，また他臓器にアミロイド沈着を認める所見も認めず，限局性結節性肺アミロイドーシスと最終診断した．膀胱癌精査中に発見された限局性結節性肺アミロイドーシスの1例を経験し，膀胱癌との関連も否定できないため報告する．

Amylose pulmonaire AL révélée par une toux : à propos d’une observation

IntroductionPulmonary lesions may be a presenting feature of AL amyloidosis. ObservationWe report a 49-year-old male with AL amyloidosis secondary to a multiple myeloma with symptomatic interstitial pulmonary lesions and chronic cough as the presenting feature. ConclusionLung involvement is relatively frequent during AL amyloidosis but most of the time it remains asymptomatic. Interstitial pneumonia is the rarest condition of pulmonary amyloidosis. It is related to a large diffusion of the disease as demonstrated by the usual concurrent presence of cardiac lesions.

Amiloidose Pulmonar: Um Desafio Diagnóstico

Amyloidosis is characterized by amyloid extracellular deposition in organs and tissues. Pulmonary involvement is a rare manifestation of the disease and it can be focal or as part of systemic amyloidosis. We report two cases. Case 1: 71 year-old female with bronchiectasis and Sjogrenâ syndrome, who complained of anorexia, weight loss and a productive cough. The diagnostic study included a surgical lung biopsy and histological examination demonstrated pulmonary amyloidosis. Case 2: 83 year-old male patient, ex-smoker, asymptomatic, whose routine chest x-ray showed a nodular opacity in the right lung field. A transthoracic biopsy revealed an amyloid lung tumor. These cases illustrate a rare disease which in Case 1 also coexisted with Sjögrenâs syndrome and bronchiectasis. The most important differential diagnosis is cancer and so a definitive diagnosis is essential, as amyloidosis is usually benign and indolent.

Medical image of the week: pulmonary amyloidosis

Medical image of the week: pulmonary amyloidosis

Recurrent Pleural Effusions Occurring in Association with Primary Pulmonary Amyloidosis.

Recurrent pleural effusions occurring in association with immunoglobulin light chain amyloidosis and not associated with amyloid cardiomyopathy are rare. These portend an overall poor prognosis with mean survival time of approximately 1.8 months. We hereby report a case of a 59-year-old Caucasian female with recurrent pleural effusions and an ultimate diagnosis of pulmonary amyloidosis in association with plasma cell myeloma. The optimal treatment for recurrent pleural effusions in amyloidosis has not been determined; however, our patient responded to therapy with Cyclophosphamide-Bortezomib- (Velcade-) Dexamethasone (CyBorD) and had no repeat hospitalizations or recurrence of pleural effusion at four-month follow-up after initiation of therapy.

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.

Primary pulmonary amyloidosis misdiagnosed as malignancy on dual-time-point fluoro-deoxyglucose positron emission tomography/computed tomography: A case report and review of the literature

Primary pulmonary amyloidosis is an uncommon manifestation, characterized by amyloid deposition in the lungs and other associated tissue. The clinical presentation of amyloidosis is variable, with non-specific symptoms. The current study reports the case of a 59-year-old female presenting with primary pulmonary amyloidosis, indistinguishable from lung malignancy based on 18F-fluoro-deoxyglucose (18F-FDG) accumulation on dual-time-point (DTP) FDG-positron emission tomography/computed tomography (PET/CT) imaging and the similarities in morphological changes. A percutaneous CT-guided thoracoscopic biopsy was subsequently performed. Histological examination revealed that the specimens contained amorphous, homogeneous material with a number of polyclonal plasma cells, lymphocytes and giant cells. A diagnosis of primary nodular parenchymal pulmonary amyloidosis was determined, and the patient was discharged without chemotherapy. The patient remained in good clinical condition during follow-up. The present case indicated that localized nodular amyloidosis with increased FDG uptake on DTP FDG PET must be considered in the differential diagnosis of growing lung nodules, and that a histological examination must be conducted to distinguish this condition from malignancies of the lung.

Multiple Bullae and Pulmonary Nodular Amyloidosis: A Rare Complication of Primary Sjögren's Syndrome

We report the case of a 48 year-old woman with primary Sjögren's syndrome (pSS) who developed a rare but important pulmonary disorder associated with this condition. This case highlights the difficulty in making the diagnosis of this rare pulmonary disorder and the treatment challenges it poses.