Common variable immune deficiency with cellular immunodeficiency leading to bronchiectasis and liver cirrhosis: case report and literature review_Chinese Journal of Respiratory and Critical Care Medicine

Authors：

CHEN Yusha , YUAN Mengxin , LIANG Binmiao ,  OU Xuemei

Department of Respiratory and Critical Care Medicine, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, P. R. China;

Corresponding?author：

OU Xuemei, Email: ouxuemei@163.com

Keywords：

Common variable immune deficiency; Cellular immunodeficiency; Bronchiectasis; Liver cirrhosis

DOI：

10.7507/1671-6205.201805046

Video：

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Objective To investigate the pathogenesis, clinical manifestations, diagnosis and treatment of common variable immune deficiency (CVID).Methods One case of CVID with cellular immunodeficiency leading to bronchiectasis and liver cirrhosis was analyzed retrospectively. Relevant literatures were also searched through WanFang Database, China National Knowledge Infrastructure and PubMed, Ovid, Embase, Cochrane using the key words " common variable immunodeficiency”, " common variable hypogammaglobulinemia” in Chinese and English.Results A 52-year-old female patient, complained of cough, expectoration for 20 years, edema for 7 years and aggravated for 3 months with a history of recurrent respiratory infections was hospitalized in the West China Hospital of Sichuan University. The chest computed tomography revealed bronchiectasis, liver cirrhosis and portal hypertension. Laboratory tests showed remarkable hypogammaglobulinemia. The CD4⁺ T-cell count was below the normal range. Probable diagnosis of CVID was made based on clinical characteristics and laboratory tests. Immunoglobulin infusion with a dose of 20 g was given and the symptoms were relieved. About 288 case reports including 8 000 patients were searched. Most of them were reported individually. Conclusions CVID has a low morbidity and is rare in China. It is mostly caused by genetic factors. When there are recurrent infections in common areas of body, infections in rare areas or infections of conditioned pathogen, clinicians should be vigilant and give intervention as soon as possible. Family and genetic researches could be done when permitted.

Citation： CHEN Yusha, YUAN Mengxin, LIANG Binmiao, OU Xuemei. Common variable immune deficiency with cellular immunodeficiency leading to bronchiectasis and liver cirrhosis: case report and literature review. Chinese Journal of Respiratory and Critical Care Medicine, 2019, 18(2): 169-173. doi: 10.7507/1671-6205.201805046 Copy

1.	Yong PF, Thaventhiran JE, Grimbacher B. " A rose is a rose is a rose”, but CVID is not CVID: common variable immune deficiency (CVID), what do we know in 2011?. Adv Immunol, 2011, 111: 47-107.
2.	Azizi G, Rezaei N, Kiaee F, et al. T-cell abnormalities in common variable immunodeficiency. J Investig Allergol Clin Immunol, 2017, 26(4): 233-243.
3.	Yong PF, Workman S, Wahid F, et al. Selective deficits in blood dendritic cell subsets in common variable immunodeficiency and X-linked agammaglobulinaemia but not specific polysaccharide antibody deficiency. Clin Immunol, 2008, 127(1): 34-42.
4.	Tak Manesh A, Azizi G, Heydari A, et al. Epidemiology and pathophysiology of malignancy in common variable immunodeficiency?. Allergol Immuno Pathol (Madr), 2017, 45(6): 602-615.
5.	Mooney D, Edgar D, Einarsson G, et al. Chronic lung disease in common variable immune deficiency (CVID): a pathophysiological role for microbial and non-B cell immune factors. Crit Rev Microbiol, 2017, 43(4): 508-519.
6.	Gathman B, Mahlaoui N, Gérard L, et al. Clinical picture and treatment of 2212 patients with CVID. J Allergy Clin Immunol, 2014, 134(1): 116-126.
7.	Resnick ES, Moshier EL, Godbold JH, et al. Morbidity and mortality in common variable immune deficiency over 4 decades. Blood, 2012, 119(7): 1650-1657.
8.	Kumar Y, Bhatia A. Common variable immunodeficiency in adults: current diagnostic protocol and laboratory measures. Expert Rev Clin Immunol, 2013, 9(10): 959-977.
9.	Lucena JM, Burillo SS, Nú?ez RA, et al. Incidence of the C104R TACI mutation in patients with primary antibody deficiency. J Investig Allergol Clin Immunol, 2015, 25(5): 378-379.
10.	Yu JE, Knight AK, Radigan L, et al. Toll-like receptor 7 and 9 defects in common variable immunodeficiency. Allergy Clin Immunol, 2009, 124(2): 349-356.
11.	Azizi G, Abolhassani H, Asgardoon MH, et al. Autoimmunity in common variable immunodeficiency: epidemiology, pathophysiology and management. Expert Rev Clin Immunol, 2017, 13(2): 101-115.
12.	Touw CM, van de Ven AA, de Jong PA, et al. Detection of pulmonary complications in conlnlon variable immunodeficiency. Pediatr Allergy Immunol, 2010, 21(5): 793-805.
13.	Agondi RC, Barros MT, Rizzo LV, et al. Allergic asthma in patients with common variable immunodeficiency. Allergy, 2010, 65(4): 510-515.
14.	Malamut G, Ziol M, Suarez F, et al. Nodular regenerative hyperplasia: the main liver disease in patients with primary hypogammaglobulinemia and hepatic abnormalities. J Hepatol, 2008, 48(1): 74-82.
15.	Ameratunga R, Woon ST, Gillis D, et al. New diagnostic criteria for CVID. Expert Rev Clin Immunol, 2014, 10(2): 183-186.
16.	Jolles S, Chapel H, Litzman J. When to initiate immunoglobulin replacement therapy (IGRT) in antibody deficiency: a practical approach. Clin Exp Immunol, 2017, 188(3): 333-341.
17.	Bonilla FA, Barlan I, Chapel H, et al. International Consensus Document (ICON): Common Variable Immunodeficiency Disorders. J Allergy Clin Immunol Pract, 2016, 4(1): 38-59.
18.	Vettukattil JJ. Pathogenesis of pulmonary arteriovenous malformations: role of hepatopulmonary interactions. Heart, 2002, 88(6): 561-563.
19.	Takahashi N, Matsumoto K, Saito H, et al. Impaired CD4 and CD8 effector function and decreased memory T cell populations in ICOS-deficient patients. J Immunol, 2009, 82(9): 5515-5527.
20.	Malphettes M, Gerard L, Carmagnat M, et al. Late-onset combined immune deficiency: a subset of common variable immunodeficiency with severe T cell defect. Clin Infect Dis, 2009, 49(9): 1329-1338.
21.	Cunningham-Rundles C, Bodian C. Common variable immunodeficiency: clinical and immunological features of 248 patients. Clin Immun, 1999, 92(1): 34-48.
22.	Cooper MA, Pommcring TL, Koranyi K. Primary immunodeficiencies. Am Fam Physician, 2003, 68(10): 2001-2008.

1. Yong PF, Thaventhiran JE, Grimbacher B. " A rose is a rose is a rose”, but CVID is not CVID: common variable immune deficiency (CVID), what do we know in 2011?. Adv Immunol, 2011, 111: 47-107.
2. Azizi G, Rezaei N, Kiaee F, et al. T-cell abnormalities in common variable immunodeficiency. J Investig Allergol Clin Immunol, 2017, 26(4): 233-243.
3. Yong PF, Workman S, Wahid F, et al. Selective deficits in blood dendritic cell subsets in common variable immunodeficiency and X-linked agammaglobulinaemia but not specific polysaccharide antibody deficiency. Clin Immunol, 2008, 127(1): 34-42.
4. Tak Manesh A, Azizi G, Heydari A, et al. Epidemiology and pathophysiology of malignancy in common variable immunodeficiency?. Allergol Immuno Pathol (Madr), 2017, 45(6): 602-615.
5. Mooney D, Edgar D, Einarsson G, et al. Chronic lung disease in common variable immune deficiency (CVID): a pathophysiological role for microbial and non-B cell immune factors. Crit Rev Microbiol, 2017, 43(4): 508-519.
6. Gathman B, Mahlaoui N, Gérard L, et al. Clinical picture and treatment of 2212 patients with CVID. J Allergy Clin Immunol, 2014, 134(1): 116-126.
7. Resnick ES, Moshier EL, Godbold JH, et al. Morbidity and mortality in common variable immune deficiency over 4 decades. Blood, 2012, 119(7): 1650-1657.
8. Kumar Y, Bhatia A. Common variable immunodeficiency in adults: current diagnostic protocol and laboratory measures. Expert Rev Clin Immunol, 2013, 9(10): 959-977.
9. Lucena JM, Burillo SS, Nú?ez RA, et al. Incidence of the C104R TACI mutation in patients with primary antibody deficiency. J Investig Allergol Clin Immunol, 2015, 25(5): 378-379.
10. Yu JE, Knight AK, Radigan L, et al. Toll-like receptor 7 and 9 defects in common variable immunodeficiency. Allergy Clin Immunol, 2009, 124(2): 349-356.
11. Azizi G, Abolhassani H, Asgardoon MH, et al. Autoimmunity in common variable immunodeficiency: epidemiology, pathophysiology and management. Expert Rev Clin Immunol, 2017, 13(2): 101-115.
12. Touw CM, van de Ven AA, de Jong PA, et al. Detection of pulmonary complications in conlnlon variable immunodeficiency. Pediatr Allergy Immunol, 2010, 21(5): 793-805.
13. Agondi RC, Barros MT, Rizzo LV, et al. Allergic asthma in patients with common variable immunodeficiency. Allergy, 2010, 65(4): 510-515.
14. Malamut G, Ziol M, Suarez F, et al. Nodular regenerative hyperplasia: the main liver disease in patients with primary hypogammaglobulinemia and hepatic abnormalities. J Hepatol, 2008, 48(1): 74-82.
15. Ameratunga R, Woon ST, Gillis D, et al. New diagnostic criteria for CVID. Expert Rev Clin Immunol, 2014, 10(2): 183-186.
16. Jolles S, Chapel H, Litzman J. When to initiate immunoglobulin replacement therapy (IGRT) in antibody deficiency: a practical approach. Clin Exp Immunol, 2017, 188(3): 333-341.
17. Bonilla FA, Barlan I, Chapel H, et al. International Consensus Document (ICON): Common Variable Immunodeficiency Disorders. J Allergy Clin Immunol Pract, 2016, 4(1): 38-59.
18. Vettukattil JJ. Pathogenesis of pulmonary arteriovenous malformations: role of hepatopulmonary interactions. Heart, 2002, 88(6): 561-563.
19. Takahashi N, Matsumoto K, Saito H, et al. Impaired CD4 and CD8 effector function and decreased memory T cell populations in ICOS-deficient patients. J Immunol, 2009, 82(9): 5515-5527.
20. Malphettes M, Gerard L, Carmagnat M, et al. Late-onset combined immune deficiency: a subset of common variable immunodeficiency with severe T cell defect. Clin Infect Dis, 2009, 49(9): 1329-1338.
21. Cunningham-Rundles C, Bodian C. Common variable immunodeficiency: clinical and immunological features of 248 patients. Clin Immun, 1999, 92(1): 34-48.
22. Cooper MA, Pommcring TL, Koranyi K. Primary immunodeficiencies. Am Fam Physician, 2003, 68(10): 2001-2008.

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