Chronic enteropathy-related malabsorption syndrome in an adult with common variable immunodeficiency and symptomatic norovirus infection of the gut

2021 ◽  
Vol 14 (5) ◽  
pp. e241752
Author(s):  
Punit Jain ◽  
Anand Mishra ◽  
Deepakkumar Gupta ◽  
Satish Kulkarni
Keyword(s):  
Common Variable Immunodeficiency ◽  
Villous Atrophy ◽  
Performance Status ◽  
Ivig Therapy ◽  
Malabsorption Syndrome ◽  
High Morbidity ◽  
Diagnostic Dilemma ◽  
Chronic Enteropathy ◽  
And Performance ◽  
Common Variable

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.

The high mortality of patients with common variable immunodeficiency and small bowel villous atrophy

2019 ◽  
Vol 54 (2) ◽  
pp. 164-168 ◽  
Author(s):  
Maria Vittoria Pensieri ◽  
Federica Pulvirenti ◽  
Annalisa Schiepatti ◽  
Stiliano Maimaris ◽  
Salvatore Lattanzio ◽  
...  
Keyword(s):  
Small Bowel ◽  
High Mortality ◽  
Common Variable Immunodeficiency ◽  
Villous Atrophy ◽  
Common Variable

Granulomas in common variable immunodeficiency: A diagnostic dilemma

2004 ◽  
Vol 45 (1) ◽  
pp. 51-54 ◽  
Author(s):  
Karyn R Lun ◽  
Dominic J Wood ◽  
James B Muir ◽  
Rowland Noakes
Keyword(s):  
Common Variable Immunodeficiency ◽  
Diagnostic Dilemma ◽  
Common Variable

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

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.

scholarly journals SAT-071 Autoimmune Polyglandular Syndrome Type 1 with Common Variable Immunodeficiency

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Yasamen Abdulmannan Shikdar ◽  
Abdullah Almazrooa ◽  
Shaza Samargandy
Keyword(s):  
Common Variable Immunodeficiency ◽  
Villous Atrophy ◽  
Tissue Transglutaminase ◽  
Iga Deficiency ◽  
Primary Hypothyroidism ◽  
Intestinal Biopsy ◽  
Autoimmune Polyglandular Syndrome ◽  
Autoimmune Polyglandular Syndrome Type ◽  
Common Variable

Abstract BACKGROUND: Autoimmune polyglandular syndrome (APS) is a rare disorder. It’s co-existence with common variable immunodeficiency (CVID) was reported in 5 cases before but, none of them was APS type 1up to our knowledge. The overlap between the 2 conditions indicates a possible association between Autoimmunity and immunodeficiency. CLINICAL CASE: A 21-year-old lady was diagnosed to have CVID since infancy, as she was presenting to the hospital with recurrent infections and sepsis. At the age of 7 months, she was diagnosed with type 1 diabetes and was started on insulin. Furthermore, at 2 years of age she was found to have primary hypothyroidism and in her teenage she was diagnosed with primary adrenal insufficiency. Her history became more complicated when she also started to develop recurrent oral and esophageal candidiasis that required systemic anti-fungal therapy. Later in her life, she was incidentally found to have hypoparathyroidism when her labs showed hypocalcemia with inappropriately normal parathyroid hormone. She had chronic diarrhea which was thought to be due to celiac disease based on intestinal biopsy showing villous atrophy. With her IgA deficiency, her Tissue transglutaminase IgA antibodies were not reliable. Splenic atrophy was also detected on abdominal imaging. She never reached puberty and elected with her parents to not start combined hormonal therapy. With the constellation of these features, we concluded that she has type 1 APS along with CVID. CONCLUSION: Autoimmunity and immunodeficiency might be interconnected. Early diagnosis will affect the quality of life and early targeted treatment will prevent morbidity and early mortality. KEY WORDS: Autoimmune Polyglandular Syndrome, Common Variable Immunodeficiency.

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

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.

Common Variable Immunodeficiency (CVID) – variables humorales Immundefektsyndrom

Praxis ◽  
2007 ◽  
Vol 96 (1) ◽  
pp. 3-11
Author(s):  
Suter-Meyer ◽  
Nigg ◽  
Kolyvanos Naumann ◽  
Käser ◽  
Vetter
Keyword(s):  
Common Variable Immunodeficiency ◽  
Common Variable

A case of polyglandular autoimmune disease associated with common variable immunodeficiency

2015 ◽  
Author(s):  
Ishrat Khan ◽  
Mirena Noyvirt ◽  
Aicha Bouraoui ◽  
Penny Owen ◽  
Helen Lane
Keyword(s):  
Autoimmune Disease ◽  
Common Variable Immunodeficiency ◽  
Common Variable
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