Childhood-Onset Sjögren Syndrome Presenting as Pulmonary Hemorrhage

Wang, Christine; Simpkin, Charles; Vielkind, Monica; Galambos, Csaba; Lin, Clara; Liptzin, Deborah R.; Curran, Megan L.

doi:10.1542/peds.2020-042127

Cited by 5 publications

(3 citation statements)

References 19 publications

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“…Wang et al [9] published the case of a child who presented acutely with chest pain and fever after a week of fatigue and myalgia. HRCT revealed extensive bilateral airspace opacities, and initial PFTs were notable for a significantly elevated DLCO and reduced total lung capacity.…”

Section: Pulmonary Complications Present At Diagnosismentioning

confidence: 99%

New developments related to lung complications in pediatric rheumatic disease

Rai

Schulert

Towe

2023

Current Opinion in Rheumatology

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Purpose of review While substantial progress has been made understanding lung disease in adult patients with rheumatic disease, pediatric lung disease has not been well addressed. Several recent studies provide new insights into diagnosis, management and treatment of lung disease in children with rheumatic disease. Recent findings Building on previous research, newly diagnosed patients may have abnormalities in pulmonary function tests and chest computed tomography imaging even when asymptomatic. New guidelines for screening for rheumatic-associated lung disease provide important recommendations for clinicians. New theories have been proposed about immunologic shifts leading to the development of lung disease in children with systemic juvenile idiopathic arthritis. Additionally, there are new antifibrotic agents that are being explored as treatments in pediatric patients with fibrotic lung diseases. Summary Patients appear to have frequent lung function abnormalities while being clinically asymptomatic, emphasizing importance for rheumatologists to refer for pulmonary function tests and imaging at diagnosis. New advances are helping define optimal approaches to treatment of lung disease, including use of biologic agents and antifibrotic medicines for pediatric patients with rheumatologic diseases.

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Section: Pulmonary Complications Present At Diagnosismentioning

confidence: 99%

New developments related to lung complications in pediatric rheumatic disease

Rai

Schulert

Towe

2023

Current Opinion in Rheumatology

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“…Extraglandular manifestations of SS occur in up to one third of pediatric and adult patients and include, but are not limited to, arthritis, myositis, rash, cytopenias, pulmonary involvement including interstitial lung disease (ILD), airway disease, and pulmonary hemorrhage, renal tubular acidosis, tubulointerstitial nephritis, and CNS involvement including optic neuritis, transverse myelitis, and vasculitis [6,[9][10][11]. ILD is common in adult patients with autoimmune connective tissue disease (CTD), however prevalence varies depending on type of CTD: rheumatoid arthritis (RA; 10-20%), systemic sclerosis (SSc; > 70%), SLE (1-15%), idiopathic inflammatory myopathies (20-78%), mixed connective tissue disease (MCTD; 53%) [10].…”

Section: Introductionmentioning

confidence: 99%

Pulmonary manifestations of childhood-onset primary Sjogren’s syndrome (SS) masquerading as reactive airways disease in a male patient and review of interstitial lung disease associated with SS

et al. 2022

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Background Sjogren’s syndrome (SS) is a rare chronic autoimmune disease involving exocrine glands presenting with sicca syndrome, recurrent parotitis and other extraglandular stigmata. SS is well characterized in the adult population with classification criteria; however, primary SS presenting in childhood is poorly defined and rare in males. Recurrent parotitis is the most common presenting symptom in children with primary SS; however, clinical phenotype in children appears more variable than in adults. The lungs are a common extraglandular location for manifestations of primary SS. However, interstitial lung disease (ILD) is rare in children with primary SS. There are only four published reports of ILD associated with primary SS in female children. Here, we present a very rare case of primary SS in a pediatric male with pulmonary manifestations and review of the literature on ILD in childhood-onset primary SS. Case presentation A 14-year-old White male with a history of chronic severe asthma, recurrent parotitis and idiopathic intracranial hypertension was referred to pediatric rheumatology for evaluation of a positive ANA. In early childhood, he was diagnosed with persistent asthma recalcitrant to therapy. At age 8, he developed recurrent episodes of bilateral parotitis despite multiple treatments with sialoendoscopy. At age 14, respiratory symptoms significantly worsened prompting reevaluation. Lab workup was notable for positive ANA and Sjogren’s Syndrome A and B antibodies. Pulmonary function tests showed only a mild obstructive process. Computed tomography of chest was significant for small airway disease, and lung biopsy was positive for mild interstitial lymphocytic inflammation presenting a conflicting picture for ILD. The constellation of findings led to the diagnosis of primary SS with associated pulmonary manifestations. He was treated with hydroxychloroquine, mycophenolate mofetil and oral corticosteroids with resolution of symptoms. Conclusions Primary SS is a rare disease in the pediatric population that is poorly characterized. This case is the very rare presentation of childhood-onset primary SS with pulmonary manifestations in a male patient. ILD associated with primary SS is also very rare with only four pediatric patients reported in the literature. Collaborative effort is needed to develop pediatric specific diagnostic and treatment guidelines in this rare condition.

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“…Diverse clinical features include recurrent parotitis, constitutional symptoms, and extraglandular manifestations, such as arthralgias/arthritis, and neurological and renal disease. Several recent case reports have broadened the scope of diverse clinical presentations with descriptions of pulmonary hemorrhage [2] and psychosis [3]. As gland dysfunction develops over time, the characteristic sicca symptoms of dry eyes and dry mouth, may be a later feature, and thus more common in adults compared with children [1].…”

Section: Introductionmentioning

confidence: 99%

Updates in childhood Sjogren's syndrome

Wright

2021

Current Opinion in Pediatrics

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Purpose of reviewChildhood Sjogren's syndrome (cSS) is a rare, chronic autoimmune disease characterized by inflammation of the exocrine glands. cSS is underrecognized because of differences in clinical presentation compared with adults. Until recently, publications describing clinical manifestations in cSS were limited to case reports and case series with small numbers of patients. Diagnostic studies to assess glandular symptoms in adults, are less commonly obtained in children.Recent findingsRecent cohort studies describe presenting diagnostic clinical features in large populations of cSS and demonstrate how current classification criteria, used in adults, are not applicable to children. Recurrent parotitis is the consistent predominant manifestation that is inversely correlated with age. Novel salivary biomarkers and salivary gland ultrasonography are important objective measure, which may improve diagnosis and disease monitoring. Standardized treatment recommendations are needed.SummaryFindings from large cohort studies provide a framework for the future development of diagnostic criteria for cSS. Such criteria should incorporate objective measures that are easily obtained in children. Future research to improve understanding of the application of novel biomarkers and imaging and developing consensus on treatment recommendations is needed.

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Childhood-Onset Sjögren Syndrome Presenting as Pulmonary Hemorrhage

Cited by 5 publications

References 19 publications

New developments related to lung complications in pediatric rheumatic disease

New developments related to lung complications in pediatric rheumatic disease

Pulmonary manifestations of childhood-onset primary Sjogren’s syndrome (SS) masquerading as reactive airways disease in a male patient and review of interstitial lung disease associated with SS

Updates in childhood Sjogren's syndrome

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