Bronchiectasis caused by common variable immunodeficiency

Divino, Paulo Henrique do Amor; Basílio, José; Fabbri, Renato; Bastos, Igor Polônio; Forte, Wilma Carvalho Neves

doi:10.1590/s1806-37132015000000095

Cited by 3 publications

(7 citation statements)

References 5 publications

(8 reference statements)

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“…Regarding the direct costs, the total average cost for ambulatory care for patients with RRTI is 1656 €/patient, according to the official report of the Spanish Ministry of Health, Consumer Affairs and Social Welfare in 2017 [ 36 ]. This amount can increase to 3962 €/patient when hospital admission is required.…”

Section: Resultsmentioning

confidence: 99%

“…With regard to indirect costs, 14 euros per hour was defined as the cost of absenteeism due to illness, as well as presenteeism due to the reduction in the percentage of productivity [ 36 ]. A median of 2 days of absenteeism (the daily workday is 8 h), equivalent to €28, added up to €308 for a cumulative 22 h of labor absenteeism, giving a total of 336 €/patient of annual indirect costs in the pre-immunization RRTI group.…”

Section: Resultsmentioning

confidence: 99%

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Trained Immunity Based-Vaccines as a Prophylactic Strategy in Common Variable Immunodeficiency. A Proof of Concept Study

et al. 2020

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Background. A major concern in the care of common variable immunodeficiency (CVID) patients is the persistence of subclinical or recurrent respiratory tract infections (RRTI) despite adequate trough IgG levels, which impacts the quality of life (QoL) and morbidity. Therefore, the development of new approaches to prevent and treat infection, especially RRTI, is necessary. Objectives. We conducted a clinical observational study from May, 2016 to December, 2017 in 20 CVID patients; ten of these patients had a history of RRTI and received the polybacterial preparation MV130, a trained immunity-based vaccine (TIbV) to assess its impact on their QoL and prognosis. Methods. Subjects with RRTI received MV130 for 3 months and were followed up to 12 months after initiation of the treatment. The primary endpoint was a reduction in RRTI at the end of the study. We analyzed the pharmacoeconomic impact on the RRTI group before and after immunotherapy by estimating the direct and indirect costs, and assessed CVID-QoL and cytokine profile. Specific antibody responses to the bacteria contained in MV130 were measured. Results. The RRTI-group treated with TIbV MV130 showed a significant decrease in infection rate (p = 0.006) throughout the 12 months after initiation of the treatment. A decrease in antibiotic use and unscheduled outpatient visits was observed (p = 0.005 and p = 0.002, respectively). Significant increases in anti-pneumococcus and anti-MV130 IgA antibodies (p = 0.039 both) were detected after 12 months of MV130. Regarding the CVID QoL questionnaire, an overall decrease in the score by more than 50% was observed (p < 0.05) which demonstrated that patients experienced an improvement in their QoL. The pharmacoeconomic analysis showed that the real annual direct costs decreased up to 4 times per patient with the prophylactic intervention (p = 0.005). Conclusion. The sublingual administration of the TIbV MV130 significantly reduced the rate of respiratory infections, antibiotic use and unscheduled visits, while increasing specific IgA responses in CVID patients. Additionally, the CVID population felt that their QoL was improved, and a decrease in expenses derived from health care was predicted.

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Trained Immunity Based-Vaccines as a Prophylactic Strategy in Common Variable Immunodeficiency. A Proof of Concept Study

et al. 2020

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“…7 En el paciente fueron descartadas causas secundarias de hipogammaglobulinemia: pérdidas renales o intestinales, leucosis, linfomas y uso de medicamentos, dado que el diagnóstico de IDCV es de exclusión, 1 así como otras IDP: CD19 normal (lo que descartó deficiencia de tirosina cinasa de Bruton), sin aumento de IgM, sin infecciones oportunistas e IgE normal (para síndrome de la hiper-IgM), serologías negativas para linfoproliferación después de infección por virus Epstein Bar, sin timoma ni deficiencia de CD19 e CD3 (síndrome de Good). 2,8 El paciente presentó neumonías de repetición después de comenzar a trabajar en el aeropuerto. Es conocida una distribución bimodal en la aparición de las manifestaciones clínicas de la IDCV de los seis a 10 años y entre los 20 y 40 años.…”

Section: Discussionunclassified

“…Los pacientes con IDCV presentan mayor riesgo de enfermedades inflamatorias y autoinmunes, en especial de la tiroides. 2,9 Por estos motivos, al principio y durante el seguimiento se realizaron exámenes para búsqueda intencionada de enfermedades autoinmunes: anticuerpos antitiroideos, antinucleares, factor reumatoide, complemento total.…”

Section: Discussionunclassified

“…1 Actualmente, la IDCV es considerada la más prevalente entre las inmunodeficiencia primaria (IDP) graves. 2 La IDCV se caracteriza por disminución de la inmunoglobulina (Ig) G, A o M, después de la exclusión de otras causas de hipogammaglobulinemia. 1 Puede observarse compromiso celular, caída de linfocitos CD4+, aumento no bien esclarecido de CD8+ e inversión de la relación CD4/CD8.…”

Section: Antecedentesunclassified

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Manifestaciones clínicas y diagnóstico tardío de la inmunodeficiencia común variable

Forte

Morad

Oliveira

et al. 2019

RAM

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Antecedentes: La inmunodeficiencia común variable (IDCV) es la inmunodeficiencia primaria (IDP) grave más frecuente. Las manifestaciones clínicas surgen en cualquier edad, pero son más frecuentes entre los seis y 10 años y entre los 20 y 40 años. En la literatura, casi no hay informes de diagnóstico de IDCV después de los 50 años.Caso clínico: Hombre, 58 años de edad, con historia clínica de infecciones de repetición desde los 35 años. Los exámenes mostraron disminución de IgG, IgA, anticuerpos específicos, sin otras causas de hipogammaglobulinemia. Se realizó el diagnóstico de IDCV y recibió reposición de inmunoglobulina humana, así como refuerzo de la higiene personal y ambiental. El paciente dejó de presentar infecciones de repetición.Conclusión: Los diagnósticos después de los 50 años, a pesar de ser tardíos, son fundamentales para la recuperación de los pacientes. En el caso referido, la reposición con inmunoglobulina humana permitió mejorar la calidad de vida.

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Posterior Cord Syndrome and Trace Elements Deficiency as an Uncommon Presentation of Common Variable Immunodeficiency

Macedo

Mota

Monteiro

et al. 2017

Case Reports in Medicine

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Diarrhea is one of the most common symptoms in common variable immunodeficiency, but neurologic manifestations are rare. We presented a 50-year-old woman with recurrent diarrhea and severe weight loss that developed a posterior cord syndrome. Endoscopy found a duodenal villous blunting, intraepithelial lymphocytosis, and lack of plasma cells and magnetic resonance imaging of the spine was normal. Laboratory assays confirmed common variable immunodeficiency syndrome and showed low levels of trace elements (copper and zinc). Treatment was initiated with parenteral replacement of trace elements and intravenous human immunoglobulin and the patient improved clinically. In conclusion, physicians must be aware that gastrointestinal and neurologic disorders may be related to each other and remember to request trace elements laboratory assessment.

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Bronchiectasis caused by common variable immunodeficiency

Cited by 3 publications

References 5 publications

Trained Immunity Based-Vaccines as a Prophylactic Strategy in Common Variable Immunodeficiency. A Proof of Concept Study

Trained Immunity Based-Vaccines as a Prophylactic Strategy in Common Variable Immunodeficiency. A Proof of Concept Study

Manifestaciones clínicas y diagnóstico tardío de la inmunodeficiencia común variable

Posterior Cord Syndrome and Trace Elements Deficiency as an Uncommon Presentation of Common Variable Immunodeficiency

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