An Uncommon Familial Presentation of Long Standing Diarrhea and Common Variable Immunodeficiency Improved with IVIG Therapy

Common variable immunodeficiency (CVID) causes a chronic debilitating syndrome in affected patients and often leads to high morbidity and mortality. Among its several presentations, chronic enteropathy leading to malabsorption syndrome continues to offer a major diagnostic dilemma. Lately, higher testing for norovirus infection in patients with CVID enteropathy has correlated its presence to chronic diarrhoeas, severe villous atrophy and malabsorption syndromes. There have been no such reports of its clinical and histopathological manifestations in CVID from India. Here, we demonstrate the significance of testing for norovirus in the gut with multiplex PCRs in an adult patient with a chronic undiagnosed CVID enteropathy and its response to monthly intravenous immunoglobulin (IVIG) therapy. Our patient responded after three cycles of monthly IVIG with a complete clinical recovery of his bowel functions, leading to a significant improvement in his quality of life and performance status.

Download Full-text

An Uncommon Presentation of Chronic Diarrhea and Common Variable Immunodeficiency Improved with IVIG Therapy

Journal of Allergy and Clinical Immunology ◽

10.1016/j.jaci.2005.12.694 ◽

2006 ◽

Vol 117 (2) ◽

pp. S174 ◽

Cited By ~ 3

Author(s):

S.J. McEldowney ◽

M.H. Moss

Keyword(s):

Common Variable Immunodeficiency ◽

Chronic Diarrhea ◽

Ivig Therapy ◽

Uncommon Presentation ◽

Common Variable

Download Full-text

Common Variable Immunodeficiency (CVID) with Granulomatous Interstitial Lung Disease (GLILD) and SARS COVID-19 Infection: Case Report and Review of Literature

10.21203/rs.3.rs-493851/v1 ◽

2021 ◽

Author(s):

Debendra Pattanaik ◽

Shaunah Ritter ◽

Joseph Fahhoum

Keyword(s):

Case Report ◽

Interstitial Lung Disease ◽

Lung Disease ◽

Common Variable Immunodeficiency ◽

Clinical Course ◽

Pneumococcal Vaccination ◽

Severe Infection ◽

Ivig Therapy ◽

Ivig Treatment ◽

Common Variable

Abstract Background We present a case of CVID complicated by granulomatous interstitial lung disease (GLILD). This patient clinical course was further complicated by COVID-19 infection. This is only the 2nd known case report of COVID 19 in CVID with GLILD. The clinical course and outcome of COVID 19 infection with common variable immunodeficiency (CVID) and GLILD is not well known. Case presentation: Our patient met the clinical features of CVID secondary to low IgG/IgA, recurrent infections, and failure to respond to pneumococcal vaccination. He was treated with monthly maintenance IVIG therapy. Our patient also was diagnosed with co-existing GLILD that despite IVIG treatment was progressing. The patient needed to be started on Rituxan and Mycophenolate Mofetil to achieve control but unfortunately became infected with COVID19 delaying his treatment for GLILD. Our patient only suffered from mild COVID 19 infection and was able to make antibodies to this. We believe severe infection was avoided as his CVID was well controlled with IVIG therapy despite progression of his granulomatous interstitial lung disease. Conclusion In conclusion, our patient with CVID with co-existing biopsy proven granulomatous interstitial lung disease despite being very high risk for severe COVID 19 infection only had mild infection. This was believed to be due to well controlled CVID with IVIG therapy.

Download Full-text

Common variable immunodeficiency (CVID) with granulomatous interstitial lung disease (GLILD) and SARS COVID-19 infection: case report and review of literature

Allergy Asthma & Clinical Immunology ◽

10.1186/s13223-021-00600-y ◽

2021 ◽

Vol 17 (1) ◽

Author(s):

Debendra Pattanaik ◽

Shaunah Ritter ◽

Joseph Fahhoum

Keyword(s):

Case Report ◽

Interstitial Lung Disease ◽

Lung Disease ◽

Common Variable Immunodeficiency ◽

Clinical Course ◽

Pneumococcal Vaccination ◽

Severe Infection ◽

Ivig Therapy ◽

Ivig Treatment ◽

Common Variable

Abstract Background We present a case of CVID complicated by granulomatous interstitial lung disease (GLILD). This patient clinical course was further complicated by COVID-19 infection. This is only the 2nd known case report of COVID 19 in CVID with GLILD. The clinical course and outcome of COVID 19 infection with common variable immunodeficiency (CVID) and GLILD is not well known. Case presentation Our patient met the clinical features of CVID secondary to low IgG/IgA, recurrent infections, and failure to respond to pneumococcal vaccination. He was treated with monthly maintenance IVIG therapy. Our patient also was diagnosed with co-existing GLILD that despite IVIG treatment was progressing. The patient needed to be started on Rituxan and Mycophenolate mofetil to achieve control but unfortunately became infected with COVID19 delaying his treatment for GLILD. Our patient only suffered from mild COVID 19 infection and was able to make antibodies to this. We believe severe infection was avoided as his CVID was well controlled with IVIG therapy despite progression of his granulomatous interstitial lung disease. Conclusion In conclusion, our patient with CVID with co-existing biopsy proven granulomatous interstitial lung disease despite being very high risk for severe COVID 19 infections only had mild infection. This was believed to be due to well controlled CVID with IVIG therapy.

Download Full-text