Multinodular pulmonary amyloidosis in primary Sjögren's syndrome

Adzic, Tatjana; Stojšić, Jelena; Radosavljevic-Asic, G.; Bouros, Demosthenes

doi:10.1016/j.ejim.2008.07.029

Cited by 9 publications

(3 citation statements)

References 3 publications

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“…1,2,7,10,11 Nodular amyloidosis is usually seen as bilateral, variable-sized, peripheral subpleural localizations. 3,7,10,14 In pulmonary amyloidosis, calcifications or cavitations may occur inside the nodule, but often nodules usually show calcifications without cavitation. 1,5,9 It may be associated with lymphocytic interstitial pneumonia and cystic lesions.…”

Section: Discussionmentioning

confidence: 99%

Coexistence of Sjögren's syndrome and pulmonary nodular amyloidosis

Tastekin,

Kerim,

Sen

et al. 2023

Int J of Rheum Dis

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There are very few cases in the literature on the coexistence of Sjögren's syndrome and pulmonary nodular amyloidosis being treated with rituximab. When nodules with central calcification and cystic lesions are seen on computed tomography, amyloid lung should be considered. Biopsy is recommended as it can be confused with malignancies. In this article, we present a 66‐year‐old female patient who has been followed up for Sjögren's syndrome for 26 years. Multiple cystic lesions with central calcification in the lung were detected and it was evaluated as amyloid nodule in the biopsy performed. The patient is being followed and is stable under rituximab treatment. Pulmonary noduler amyloidosis is very rare in Sjögren patients and there are very few cases where rituximab is used for treatment. We decided to publish in order to guide clinicians who will encounter similar cases.

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Section: Discussionmentioning

confidence: 99%

Coexistence of Sjögren's syndrome and pulmonary nodular amyloidosis

Tastekin,

Kerim,

Sen

et al. 2023

Int J of Rheum Dis

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“…70 A heavy female predominance is observed among SjD patients with amyloidosis. 68,[71][72][73][74][75][76][77] Disease onset is typically in the fifth decade, and most patients have a pre-existing diagnosis of SjD when amyloidosis is diagnosed. Common symptoms include cough and dyspnea in over half of all patients, with a smaller proportion of asymptomatic incidental diagnoses.…”

Section: Amyloidosismentioning

confidence: 99%

Pulmonary Manifestations of Sjögren's Disease

Byrne,

McCarthy,

Fabre

et al. 2024

Semin Respir Crit Care Med

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Sjögren's disease (SjD) is a chronic, progressive autoimmune condition of exocrine and extraglandular tissues. It can present with isolated disease characterized by lymphocytic infiltration of salivary or lacrimal glands, but in approximately one-third of the patients, lymphocytic infiltration extends beyond exocrine glands to involve extraglandular organs such as the lungs. Pulmonary complications have been reported to occur between 9 and 27% of patients with SjD across studies. Respiratory manifestations occur on a spectrum of severity and include airways disease, interstitial lung disease, cystic lung disease, and lymphoma. Lung involvement can greatly affect patients' quality of life, has a major impact on the overall prognosis, and frequently leads to alteration in the treatment plans, highlighting the importance of maintaining a high index of clinical suspicion and taking appropriate steps to facilitate early recognition and intervention.

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“…More recent studies in selected patients with Sjögren's syndrome using surgical biopsies have also described a number of ILDs such as f-NSIP (the most frequently occurring), cellular NSIP, UIP, COP and lung amyloidosis in addition to the already described LIP [214][215][216] . However, this is not part of our clinical experience in very wellselected pSs patients, especially the development of fibrotic ILDs, raising thoughts about the coexistence of a second CTD although not yet fulfilling undisputed diagnostic criteria [29] .…”

Section: Other Interstitial Pneumoniasmentioning

confidence: 99%

Investigation of Lung Involvement in Connective Tissue Disorders

Papiris¹,

Manali²,

Kolilekas³

et al. 2015

Respiration

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Lung involvement in connective tissue disorders (CTDs) may present as pleomorphic since any lung compartment may be involved such as airways, exocrine secretory and alveolar epithelia, interstitial lung structure, pulmonary vasculature and pleura as well as, in specific disorders, several tissues of the thoracoabdominal ventilator pump. Any combination of the above anatomic structures may be involved concomitantly although some specific combinations may include a determinant of rheumatic disorders. The diagnosis of a specific CTD requires the fulfilment of clearly defined clinical and laboratory criteria including in most cases positivity for autoantibodies, mostly specific serologic combinations. In this setting, serologic investigation targets mainly, although not exclusively, the detection of antinuclear antibodies. A specific serologic positivity or a combination of autoantibodies constitutes not only a diagnostic criterion for a specific CTD, but may also characterize the pattern of respiratory manifestation in a determinant rheumatic disorder. Therefore, the investigation of lung involvement in CTDs requires adequate skills in the ambit of a multidisciplinary approach and an extended spectrum of diagnostic tools and modalities able to detect both early clinical clues and serologic conversion as well as any pathophysiologic alteration that regards the complexity of respiratory functional status. Although many patients with CTDs suffer from a ‘vicious' combination of lung involvement, lung drug toxicity and infections related to the above two as well as to the ‘mater' disease, for space reasons this review will focus on the established lung manifestations that regard the 7 major CTDs.

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Multinodular pulmonary amyloidosis in primary Sjögren's syndrome

Cited by 9 publications

References 3 publications

Coexistence of Sjögren's syndrome and pulmonary nodular amyloidosis

Coexistence of Sjögren's syndrome and pulmonary nodular amyloidosis

Pulmonary Manifestations of Sjögren's Disease

Investigation of Lung Involvement in Connective Tissue Disorders

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