Pulmonary manifestations of hypogammaglobulinaemia.

Common variable immunodeficiency –

“…In the course of CVID, both immunoglobulin production and response to continuous antigenic stimulation in the gastrointestinal tract do not work properly. Symptoms from the gastrointestinal tract are quite common in CVID and occur in 21 to 50% cases [10,28,38,40]. Most patients suffer from diarrhea, both acute and chronic.…”

“…Recurrent infections, poorly responding to conventional antibiotics, can lead to the formation of bronchiectasis, respiratory failure and cor pulmonale. Such a sequence of events in a number of cohort studies is estimated at 17-76% [24][25][26][27][28][29][30][31][32][33]. Early diagnosis usually protects the patient against this complication, however, development of bronchiectasis occurs even in 30% of patients, despite optimal treatment [34,35].…”

Serial lung function tests in primary immune deficiency

“…Treatment of PID with parenteral immunoglobulin (Ig) replacement therapy reduces the occurrence of severe respiratory tract infections and subsequent pulmonary damage [4,5]. However, there is some evidence that despite Ig replacement therapy to physiological levels, structural lung damage still occurs [2].…”

Alterações tomográficas e funcionais pulmonares em pacientes com hipogamaglobulinemia primária em reposição de imunoglobulina humana

“…Pacientes com SHIGM por deficiência de AID ou UNG apresentam frequentemente hiperplasia linfóide (Revy et al, 2000;Quartier et al, 2004 (Dukes et al, 1978;Roifman et al, 1987;Skull e Kemp, 1996;Aghamohammadi et al, 2004;Quartier et al, 2004;Aghamohammadi et al, 2005;Pourpak et al, 2006). No entanto, o impacto exercido pela reposição de imunoglobulina em relação à prevenção de complicações pulmonares ainda é controverso, visto que a doença pulmonar crônica continua sendo uma das complicações mais frequentes (Hausser et al, 1983;Winkelstein et al, 2006;Aghamohammadi et al, 2007;Aydogan et al, 2008;Basile et al, 2009).…”

“…Estes achados estão de acordo com trabalhos publicados envolvendo pacientes com hipogamaglobulinemia primária (Levy et al, 1997;Plebani et al, 2002;Winkelstein et al, 2003;Quinti et al, 2007;Aydogan et al, 2008;Oksenhendler et al, 2008;Basile et al, 2009;Baris et al, 2011, Maarschalk-Ellerbroek et al, 2012Mohammadinejad et al, 2012). A redução da frequência e gravidade das pneumonias após o início da reposição de imunoglobulina evidenciada no presente estudo também foi demonstrado anteriormente por diversos outros grupos (Dukes et al, 1978;Roifman et al, 1987;Skull e Kemp, 1996;Quartier et al, 1999;Martínez García et al, 2001;Busse et al, 2002;Plebani et al, 2002;Aghamohammadi et al, 2004;Quartier et al, 2004;de Gracia et al, 2004;Aghamohammadi et al, 2005;Pourpak et al, 2006;Quinti et al, 2007;Lucas et al, 2010;Quinti et al, 2011, Maarschalk-Ellerbroek et al, 2012. (Winkelstein et al, 2003;Oksenhendler et al, 2008;Costa-Carvalho et al, 2011 (Plebani et al, 2002;Quinti et al, 2007;Quinti et al, 2011).…”