Pulmonary manifestations of systemic vasculitis in childhood

Lawton, Adam; Machta, Joseph; Semple, Thomas; Gupta, Atul

doi:10.1183/20734735.0211-2020

Cited by 4 publications

(3 citation statements)

References 36 publications

(38 reference statements)

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“…The most common age of onset is in early teenage years, although some children present in infancy. Lung involvement, considered to be a hallmark of MPA and GPA, is associated with increased morbidity and mortality -in one cohort of 28 patients, half of children required ICU admission and one third of these admissions were for respiratory failure, with five patient requiring mechanical ventilation [131][132][133].…”

Section: Anca Associated Vasculitismentioning

confidence: 99%

“…In pediatric GPA, pulmonary involvement can range from asymptomatic with radiographic abnormalities (a prior report noted 41% of radiographic abnormalities found in patients with pediatric GPA were observed in asymptomatic patients [135]) to presentation with fulminant DAH [133]. In the ARChiVe cohort, including 183 patients with pediatric GPA, 74% had pulmonary involvement, with 54% reporting chronic cough and 42% with massive hemoptysis or alveolar hemorrhage [136,137].…”

Section: Anca Associated Vasculitismentioning

confidence: 99%

“…PFTs may show abnormal DLCO (related to parenchymal disease or hemorrhage), restrictive lung disease, and/or obstructive disease (related to airway stenosis). CT findings may include nodular lung lesions (secondary to necrotizing, granulomatous inflammation, which may demonstrate cavitation and/or a "halo sign" related to adjacent hemorrhage), fixed pulmonary infiltrates, and more rarely (< 10% of cases), fibrosis, septal thickening and pneumothorax [133,136]. Bronchoalveolar lavage (BAL) with serial aliquots can be useful in evaluation for pulmonary hemorrhage.…”

Section: Anca Associated Vasculitismentioning

confidence: 99%

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Innovations in the Care of chILD Associated with Connective Tissue Disease and Immune Mediated Disorders

Samad,

Wobma,

Casey

2023

Preprint

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Childhood interstitial lung disease associated with connective tissue disease and immune mediated is the second most common chILD diagnostic category. As knowledge of the molecular and genetic underpinnings of these rare disorders advances, the recognized clinical spectrum of pulmonary manifestations that can be associated with them continues to broaden. This review will focus on chILD and other pulmonary complications associated with primary immune disorders, namely monogenic inborn errors of immunity as well as acquired systemic autoimmune and autoinflammatory diseases. Pulmonary complications, including ILD in these diseases can confer increased risk for morbidity and mortality and can be complex to manage due to the multiple organ systems that can be impacted in these systemic disorders. Thankfully, pulmonologists do not have to work alone. These diseases often have stereotypical patterns of extra-pulmonary features which aid in their recognition. In collaboration with a multidisciplinary team of subspecialists, the pulmonary and other systemic manifestations can be managed effectively together. The goal of this review is to familiarize the reader with the distinct patterns of ILD and associated systemic/immunologic features that are characteristic of monogenic inborn errors of immunity and systemic autoimmune and autoinflammatory diseases. In addition, this review will highlight current, emergent and innovative therapeutic strategies and will underscore the important role of multidisciplinary management to improving outcomes for these patients.

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Section: Anca Associated Vasculitismentioning

confidence: 99%

Section: Anca Associated Vasculitismentioning

confidence: 99%

Section: Anca Associated Vasculitismentioning

confidence: 99%

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Innovations in the Care of chILD Associated with Connective Tissue Disease and Immune Mediated Disorders

Samad,

Wobma,

Casey

2023

Preprint

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Innovations in the care of childhood interstitial lung disease associated with connective tissue disease and immune‐mediated disorders

Samad,

Wobma,

Casey

2024

Pediatric Pulmonology

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Childhood interstitial lung disease (chILD) associated with connective tissue and immune mediated disorders is the second most common chILD diagnostic category. As knowledge of the molecular and genetic underpinnings of these rare disorders advances, the recognized clinical spectrum of associated pulmonary manifestations continues to expand. Pulmonary complications of these diseases, including ILD, confer increased risk for morbidity and mortality and contribute to increased complexity for providers tasked with managing the multiple organ systems that can be impacted in these systemic disorders. While pulmonologists play an important role in diagnosis and management of these conditions, thankfully they do not have to work alone. In collaboration with a multidisciplinary team of subspecialists, the pulmonary and other systemic manifestations of these conditions can be managed effectively together. The goal of this review is to familiarize the reader with the classic patterns of chILD and other pulmonary complications associated with primary immune‐mediated disorders (monogenic inborn errors of immunity) and acquired systemic autoimmune and autoinflammatory diseases. In addition, this review will highlight current, emerging, and innovative therapeutic strategies and will underscore the important role of multidisciplinary management to improving outcomes for these patients.

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Systemic diseases involving the lung

Dobler

2020

Breathe

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Pulmonary manifestations of systemic vasculitis in childhood

Cited by 4 publications

References 36 publications

Innovations in the Care of chILD Associated with Connective Tissue Disease and Immune Mediated Disorders

Innovations in the Care of chILD Associated with Connective Tissue Disease and Immune Mediated Disorders

Innovations in the care of childhood interstitial lung disease associated with connective tissue disease and immune‐mediated disorders

Systemic diseases involving the lung

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