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Query: UMLS:C0010200 (cough)
 23,843 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Amyloidosis is a heterogeneous group of disorders characterized by misfolding of extracellular proteins. The two most common forms are light-chain (AL) and reactive (AA) amyloidosis. Pulmonary amyloidosis secondary to Sjogren's syndrome (SS) is rare, and there is a paucity of data on its clinical and radiological features. We describe the case of a 41-year-old woman with diffuse septal pulmonary AL (lambda) amyloidosis related to SS, in the absence of systemic amyloidosis. A systematic search for pulmonary amyloidosis in SS yielded 37 cases. Amyloidosis occurred almost exclusively in women (96.5%). Cough and dyspnoea (56%) were the most common symptoms. The diagnosis of pulmonary amyloidosis was made subsequent to that of SS, with a median delay of 7 years. Radiologically, diffuse nodules with (45.5%) or without (33.3%) multiple cysts were the most common pattern. Most cases were related to nodular AL amyloidosis (both kappa and lambda). Diffuse septal AA amyloidosis has been reported in SS. The index patient is the first documented case of diffuse septal pulmonary AL amyloidosis in SS, without systemic amyloidosis. Surgical lung biopsy (78%) is usually required to establish the diagnosis and rule out lymphoma. There is no data to support any definitive therapeutic intervention for SS-related pulmonary amyloidosis. Observation is sufficient for nodular amyloidosis. Further studies are necessary to assess the efficacy of current therapies in diffuse septal amyloidosis secondary to SS.
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PMID:Pulmonary amyloidosis in Sjogren's syndrome: a case report and systematic review of the literature. 2054 91

A 67- year old man presented with cough, weight loss and night sweats. Fiberoptic bronchoscopy did not show any abnormality. Chest computed tomography scan revealed peribronchovascular thickening, sheathing and narrowing of some bronchi. There were also mediastinal and interbronchial Lymphadenopathies. The patient became lost to follow-up. He presented 5 years later with pneumonia. Flexible bronchoscopy showed diffuse infiltration of the bronchi suggesting lung cancer. Histopathological study with histochemical staining revealed tracheobronchial tract AL amyloidosis. Chest CT-scan revealed extension of the broncho-vascular thickening and superimposed pulmonary calcified nodules and lymphadenopathies. Labial biopsy revealed AL amyloidosis. No specific treatment of amyloidosis was thought to be necessary for the patient. At 6 years follow-up the disease had not progressed. This case report highlights the fact that even very rarely, systemic AL amyloidosis can involve the tracheobronchial tract. Moreover, the lungs and the tracheobronchial tract can, although rarely, be affected in the same patient.
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PMID:Systemic Al amyloidosis of the tracheobronchial tract and lungs: a rare finding. 2519 15

Multiple myeloma is characterized by monoclonal proliferation of bone marrow plasma cells causing multiple bone lesions and overproduction of a monoclonal protein (M-protein) that could deposit in tissues (amyloidosis). Dissemination of the multiple myeloma outside the bone is rare. We present a case of a 76 years old woman presenting with dry cough. Chest X-ray showed a giant tumor of the upper right lung. Contrast enhanced CT revealed a tumor that most probably originated from the structures of the thoracic wall. The transthoracic biopsy was inconclusive. The tumor was resected and the histopathological examination showed amyloid tumor of the thoracic wall with plasmacytic elements and lambda light chains deposits. A bone marrow aspiration was performed that found diffuse plasmacytic infiltrate of 20-60% and the serum proteins electrophoresis with immunofixation revealed elevated IgA and lambda light chains. The patient was diagnosed with IgA and lambda light chains multiple myeloma with consequent AL amyloidosis presenting as thoracic mass.
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PMID:Incidental Discovery of Multiple Myeloma, Presenting as an Amyloid Tumor with Plasmacytic Elements of the Thoracic Wall. 2671 May 3

BACKGROUND Amyloidosis is a protein conformational disorder characterized by extracellular deposition of amyloid fibrils in extracellular tissue. Lung involvement is most commonly caused by secondary AL amyloidosis. The familial autosomal-dominant senile transthyretin (ATTR) disease manifests mainly as polyneuropathy and restrictive cardiomyopathy denoting the name familial amyloidotic polyneuropathy (FAP). Rarely, this form manifests with clinical and radiologically relevant respiratory tract symptoms and lung involvement. CASE REPORT A 51-year-old male former smoker presented with progressive lower-extremity weakness of several months' duration. He was ultimately diagnosed with chronic demyelinating polyneuropathy and treated with intravenous immunoglobulin therapy. Subsequently, he was admitted with heart failure symptoms and pulmonary infiltrates and his echocardiogram showed a 'myocardial speckled pattern', prompting an endomyocardial biopsy, which showed transthyretin amyloid deposition. He was started on diflunisal. Additionally, serial radiographic imaging of his chest over 3 different admissions for cough, dyspnea, hypoxemia, and lethargy demonstrated recurrent pulmonary infiltrates. A fiberoptic bronchoscopy with trans-bronchial biopsies revealed amyloid deposition in the lung tissue. CONCLUSIONS The clinical presentation of recurrent or persistent pulmonary symptoms and fleeting infiltrates on imaging in a patient with familial amyloidotic polyneuropathy is not common; when present, it should raise the suspicion of respiratory tract involvement.
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PMID:Hereditary Amyloidosis with Recurrent Lung Infiltrates. 2787 70

Primary localized endobronchial amyloidosis is a rare entity, as pulmonary amyloidosis most commonly occurs as a part of systemic AL amyloidosis. It can be asymptomatic or can present with nonspecific symptoms such as progressive dyspnea, cough, wheezing and rarely respiratory failure. It is frequently misdiagnosed as asthma, COPD or pneumonia. Solitary endobronchial amyloidosis having a nodular appearance can mimic endobronchial tumor. The diagnosis is usually delayed by 8-37 months. The average life expectancy for primary tracheobronchial amyloidosis is approximately 9 years, so the early diagnosis of this disease is very critical to improve the prognosis of patients. We are presenting a case of 65 year old male which was initially diagnosed and treated as asthma exacerbation with minimal improvement. Further workup was done with CT chest, bronchoscopy and biopsy because of persistent shortness of breath, which revealed primary localized solitary endobronchial amyloidosis.
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PMID:Primary endobronchial amyloidosis: A rare case of endobronchial tumor. 2971 8