Pulmonary Vascular Disease Due to Plasma Cell Dyscrasia

Rajapreyar, Indranee; Joly, Joanna M.; Tallaj, José; Pamboukian, Salpy V.; Assad, A.; Lenneman, Carrie; Litovsky, Silvio; Chatterjee, Arka; Hoopes, Charles W.; Lenneman, Andrew

doi:10.1016/j.mayocpiqo.2020.09.004

Cited by 5 publications

(6 citation statements)

References 19 publications

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“…In SSc, B-cells produce angiogenic mediators (such as angiogenin and angiopoietin 1) in greater proportion than healthy controls, but without difference in patients with and without PAH [ 45 ]. Various plasma cell proliferative disorders are characterised by elevated VEGF levels and vascular manifestations, including PAH [ 121 ].…”

Section: Effector Mechanisms Of B-cells In Pahmentioning

confidence: 99%

B-cells in pulmonary arterial hypertension: friend, foe or bystander?

Sanges,

Tian,

Dubucquoi

et al. 2024

Eur Respir J

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There is an unmet need for new therapeutic strategies that target alternative pathways to improve the prognosis of patients with pulmonary arterial hypertension (PAH). As immunity has been involved in the development and progression of vascular lesions in PAH, we review the potential contribution of B cells in its pathogenesis and evaluate the relevance of B cell-targeted therapies. Circulating B cell homeostasis is altered in PAH patients, with total B-cell lymphopenia, abnormal subset distribution (expansion of naïve and antibody-secreting cells, reduction of memory B cells) and chronic activation. B cells are recruited to the lungs through local chemokine secretion, and activated by several mechanisms: 1) interaction with lung vascular auto-antigens through cognate B cell receptors; 2) co-stimulatory signals provided by T follicular helper (Tfh) cells (IL-21), T helper 2 (Th2) cells and mast cells (IL-4, IL-6 and IL-13); and 3) increased survival signals provided by B cell activating factor (BAFF) pathways. This activity results in the formation of germinal centres within perivascular tertiary lymphoid organs and in the local production of pathogenic autoantibodies that target the pulmonary vasculature and vascular stabilization factors (including angiotensin-II/endothelin-1 receptors and bone morphogenetic protein receptors). B cells also mediate their effects through enhanced production of pro-inflammatory cytokines, reduced anti-inflammatory properties by regulatory B cells, IgG-induced complement activation, and IgE-induced mast cell activation. Precision-medicine approaches targeting B cell immunity are a promising direction for select PAH conditions, as suggested by the efficacy of anti-CD20 therapy in experimental models and a trial of rituximab in systemic sclerosis-associated PAH.

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Section: Effector Mechanisms Of B-cells In Pahmentioning

confidence: 99%

B-cells in pulmonary arterial hypertension: friend, foe or bystander?

Sanges,

Tian,

Dubucquoi

et al. 2024

Eur Respir J

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“…It may contain a fluid level Pneumatocele-a thin-walled, air-filled space in the lung often associated with acute pneumonia, trauma, or aspiration of hydrocarbon fluid which is often transient. It may contain fluid (e.g., blood products in traumatic pneumatoceles) Sources: Harknett et al, 49 Zamora and Aeddula, 153 Waele et al, 154 Kil et al, 7 Vassallo et al, 26 Qin et al, 155 Watanabe et al, 81 Parambil et al, 156 Ito et al, 157 Natalini et al, 158 Lohrmann et al, 159 Martínez-Balzano et al, 160 Toro et al, 96 Dal Sasso et al, 161 Kyle et al, 162 Quock et al, 163 Zamora et al, 164 Berk et al, 165 Rajapreyar et al, 166 Baqir et al, 167 Sheard et al, 86 Zamora et al, 115 Willey et al, 168 Levy and Feingold, 169 Parvinian et al, 123 Kaul et al, 170 Remy-Jardin et al, 171 Hansell et al 172 Fig. 2 Axial chest CT in a 61-year-old female shows multiple bilateral parenchymal lucencies in the upper and lower lobes.…”

Section: Cysts and Cystic Lung Diseases Mimic/ Pitfallsmentioning

confidence: 99%

High-Resolution Computed Tomography of Cystic Lung Disease

Kusmirek

Meyer

2022

Semin Respir Crit Care Med

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The cystic lung diseases (CLD) are characterized by the presence of multiple, thin-walled, air-filled spaces in the pulmonary parenchyma. Cyst formation may occur with congenital, autoimmune, inflammatory, infectious, or neoplastic processes. Recognition of cyst mimics such as emphysema and bronchiectasis is important to prevent diagnostic confusion and unnecessary evaluation. Chest CT can be diagnostic or may guide the workup based on cyst number, distribution, morphology, and associated lung, and extrapulmonary findings. Diffuse CLD (DCLDs) are often considered those presenting with 10 or more cysts. The more commonly encountered DCLDs include lymphangioleiomyomatosis, pulmonary Langerhans' cell histiocytosis, lymphoid interstitial pneumonia, Birt–Hogg–Dubé syndrome, and amyloidosis/light chain deposition disease.

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“… 4 , 5 Consequently, patients with MM are at an increased risk of cardiovascular complications including PH, due to older age at disease onset, increased survival, and treatment‐related adverse effects. 6 , 7 , 8 , 9 , 10 , 11 …”

Section: Introduction Definitions and Terminologymentioning

confidence: 99%

“…Five‐year survival for patients with MM has significantly improved from 27% (1987–1989) and 49% (2005–2011) to 58% (2012–2018) based on cancer statistics and SEER data 4,5 . Consequently, patients with MM are at an increased risk of cardiovascular complications including PH, due to older age at disease onset, increased survival, and treatment‐related adverse effects 6–11 …”

Section: Introduction Definitions and Terminologymentioning

confidence: 99%

Pulmonary hypertension in patients with multiple myeloma: A comprehensive review

et al. 2023

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Multiple myeloma (MM) is a common hematological malignancy resulting from clonal proliferation of plasma cells and is defined by criteria set forth by the international myeloma working group. Pulmonary hypertension (PH) is defined by an elevated mean pulmonary artery pressure >20 mmHg measured during right heart catheterization. Echocardiography‐diagnosed PH is relatively common in patients with MM and has been associated with increased mortality, morbidity, and poor stem cell transplant outcomes. PH in patients with MM (PH‐MM) is usually multifactorial in origin. MM disease‐specific factors, host comorbidities, and treatment‐related adverse effects are the key factors for the development of PH‐MM. Pragmatically, patients with PH‐MM can be grouped into either (i) PH in patients with a new diagnosis of MM or (ii) PH that develops or worsens along the way of MM treatment. In the latter group, drug‐induced PH, venous thromboembolism, pulmonary veno occlusive disease, and cardiotoxicity should be considered as possible causes. PH‐MM should be evaluated and managed in a multidisciplinary setting. Select individuals with PH‐MM could be considered for pulmonary vasodilators at PH‐specialized centers.

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Pulmonary Vascular Disease Due to Plasma Cell Dyscrasia

Cited by 5 publications

References 19 publications

B-cells in pulmonary arterial hypertension: friend, foe or bystander?

B-cells in pulmonary arterial hypertension: friend, foe or bystander?

High-Resolution Computed Tomography of Cystic Lung Disease

Pulmonary hypertension in patients with multiple myeloma: A comprehensive review

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