Pulmonary Nodular Amyloidosis in Sjögren Syndrome

Carbone, Roberto; Cosso, C.; Cimmino, Marco A.

doi:10.3899/jrheum.140964

Cited by 3 publications

(2 citation statements)

References 2 publications

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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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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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“…Other sites are reported including the tracheobronchial walls, kidneys, lacrimal glands, tongue, and mammary glands. When it affects the lungs or the tracheobronchial wall, symptoms may ensue, such as cough, dyspnea, pleuritic pain, and hemoptysis [193][194][195][196][197][198][199][200][201][202][203]. Amyloid composition in SS is usually of AL type (lambda or kappa) light chains, or less commonly, AA amyloid type [192].…”

Section: Amyloidosis In Pulmonary Sjogren's Syndromementioning

confidence: 99%

Perspective Chapter: Pulmonary System and Sjogren’s Syndrome

Ehtesham¹,

Tiwari²,

George³

et al. 2022

Idiopathic Pulmonary Fibrosis

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Sjogren’s syndrome (SS) is a connective tissue disease targeting the exocrine glands with subsequent sicca symptoms mainly in eyes and mouth. Respiratory symptoms may be the most frequent extraglandular manifestation following fatigue and pain. Mucosal dysfunction may affect the upper and lower airways, being the small airways more frequently involved. Parenchymal disease carries most of the morbidity and mortality. Nonspecific interstitial pneumonia (NSIP) is the most common radiographic feature, whereas the fibrotic NSIP type is the most reported finding in biopsies. Pulmonary lymphoma may arise from bronchial-associated lymphoid tissue lesions, and although rare, it is prevalent in SS. Chronic hypertrophic bronchial wall changes may ascribe to the various cystic lesions. Under their presence, possible lymphocytic interstitial pneumonia, amyloidosis, and lymphoma should be explored. Pulmonary arterial hypertension may present as frequently as in lupus, especially in Asian populations. Advanced knowledge in the pathogenesis has helped in understanding the various presentations within the respiratory system, contrasting with the scarce therapeutic options to treat both the airway and parenchymal disease. Anti-fibrotic parenchymal lung therapy offers promising outcomes. The pulmonary involvement in SS may associate with a decline in quality of life and reduced life expectancy. Subsequently, clinicians should know these facts for a timely intervention.

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