A Case of Solitary Pulmonary Nodular Amyloidosis with Sj^|^ouml;gren^|^#8217;s Syndrome

Abstract

Patients with Sjögren's syndrome frequently have pulmonary involvement, but the involvement of nodular pulmonary lesions, including pulmonary amyloidosis, is rare. Most cases of pulmonary amyloidosis involve multiple nodules; solitary pulmonary nodular amyloidosis, as an associated condition of Sjögren's syndrome, is very rare. In our report, we present the case of an 80-year-old female with Sjögren's syndrome who was incidentally found to have a small solitary pulmonary nodule. The nodule showed high fluorodeoxyglucose uptake and contained areas of calcification. Because the probability that the nodular lesion was malignant could not be excluded, the tumor was excised using a thoracoscopic procedure; the final diagnosis was pulmonary nodular amyloidosis. Although most cases of pulmonary amyloidosis involve multiple nodules, amyloidosis should be considered in the differential diagnosis for a solitary pulmonary nodule in patients with Sjögren's syndrome.

Pulmonary AL- amyloidosis masquerading as lung malignancy in an Australian Indigenous patient with Sjogren's syndrome

“…Similarly, variety of pulmonary manifestations have been described in literature in association with Sjogren's syndrome, the most common of which are interstitial lung disease (ILD) and tracheobronchial disease [ 1 ]. Amyloidosis secondary to Sjogren's syndrome manifesting as nodule-cystic lung disease is infrequently reported, more so in Australian indigenous population [ [2] , [3] , [4] , [5] ]. We describe herewith a case of 52-year-old Australian Indigenous woman with a known history of primary Sjogren's syndrome, who presented with chronic cough and was found to have Pulmonary AL Amyloidosis after extensive investigation of the lung lesions which were initially masquerading as lung malignancy.…”

“…It is difficult to reliably differentiate clinico-radiologically the two despite noted associations of amyloidosis with Sjogren syndrome, Lymphoma or Myeloma and histological examination is necessary. In most reported cases as well as ours, surgical lung biopsy is required to reach a definitive diagnosis [ 4 ] and rule out lung primary or metastasis or lymphoma. This affects treatment decisions and prognostication.…”

“…Pulmonary involvement in patients with Sjogren's syndrome is varied and becoming more widely understood and is estimated to occur in between 9 and 20% of patients with Primary Sjogren's Syndrome [ 1 , 3 ], though cough can be present in upto 60% cases. The presentations described include multiple cysts or nodules, bronchiectasis and lymphoproliferative Disease [ [1] , [2] , [3] , [4] , [5] , [6] ]. These are described in Table 2 .…”

A case of wild-type transthyretin amyloidosis associated with organizing pneumonia

“…Pulmonary amyloidosis is characterized by extracellular deposition of a specific histochemical substance called amyloid in the pulmonary tissues 1 , 4 , 6 , 7 , 8 ) . Amyloid includes various proteins in a β-pleated sheet configuration that makes the proteins resistant to proteolysis 1 , 2 , 3 , 9 ) .…”

“…Consequently, pulmonary amyloid deposition is also divided into an organ-restricted type and a type that is a part of systemic amyloidosis 6 , 11 ) . Pulmonary amyloidosis may present as either a nodular parenchymal form (parenchymal nodules), a diffuse interstitial form (diffuse interstitial damage), a tracheobronchial form (submucosal deposits in the airways), or as a pleural effusion associated with pleural amyloid deposition 7 , 8 , 11 ) . The present patient may be classified as having the diffuse interstitial form of pulmonary amyloidosis, which is the least common form of pulmonary amyloidosis and is seen much more commonly as a part of systemic amyloidosis 8 ) .…”