Gastrointestinal and hepatic manifestations of primary immune deficiency diseases

Al‐Muhsen, Saleh

doi:10.4103/1319-3767.61230

Cited by 31 publications

(8 citation statements)

References 52 publications

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“…Moreover, the increased rate of several PIDs such as chronic granulomatous disease (5.2 cases/100,000 live births), severe combined immunodeficiency syndrome (19 cases/100,000 live births) are the highest in the world among Saudi population [ 31 , 32 ]. The consequences of PIDs on various infectious diseases including tuberculosis development have been reported previously from different regions including Saudi Arabia [ 33 , 34 ]. Thus, we insist on the fact that the large existence of PIDs in the country may have a direct influence on the large scale consistent reporting of EPTB predominance among Saudi Arabian populations.…”

Section: Discussionmentioning

confidence: 94%

Exploring the Sociodemographic and Clinical Features of Extrapulmonary Tuberculosis in Saudi Arabia

et al. 2015

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BackgroundSaudi Arabia annually reports a relatively higher proportion (28–32%) of extrapulmonary tuberculosis (EPTB) cases in comparison to other global regions. However, there were few studies conducted so far to determine the sociodemographic factors and clinical manifestations associated with EPTB at a nationwide level.MethodologyA retrospective analysis on culture positive EPTB isolates collected from all the provinces of the country were conducted for a period of 12 months to determine the spectrum of diversity in EPTB infection sites and the confounding factors. A detailed clinical and demographical data analysis was carried out along with first line drug susceptibility testing.Principal FindingsIntra-thoracic and extra-thoracic lymph nodes (44.6%) were the most common sites of infection followed by gastrointestinal (17.3%) and central nervous systems (11.8%). Male patients were mostly infected (58.8%), in contrary to the global trend. Any drug resistance was observed in 23.1% isolates with a 2.1% of multi-drug resistance. HIV reactivity was found only in 2.2% cases. A higher proportion of Saudi nationals (58.8%) were infected compared to the immigrants, descending mostly from South Asia (34.4%) and South East Asia (31.2%). The Saudi population predominated with all forms of EPTB while immigrants showed no significant variations.ConclusionsSaudi Arabia faces a serious threat from EPTB, particularly to the central nervous system and gastrointestinal systems. More effective diagnostic strategies and control measures must be implemented to reduce the high rate of EPTB in the country. In addition, these findings warrant further detailed research to explore all related comorbid conditions of EPTB development, particularly the host-related factors.

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Section: Discussionmentioning

confidence: 94%

Exploring the Sociodemographic and Clinical Features of Extrapulmonary Tuberculosis in Saudi Arabia

et al. 2015

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“…Autoimmune GI manifestations are thought to be less common in XLA than in other primary immunodeficiencies, presumably because it is T cell dysfunction that drives intestinal disease in other immunodeficiency syndromes [8]. For example, GI complications are reported in 20–50% of patients with CVID [9], with GI infections occurring at a reported incidence of 27% [10]. It is possible that these hypomorphic XLA mutations are leading to increased autoimmunity.…”

Section: Discussionmentioning

confidence: 99%

Gastrointestinal Manifestations in X-linked Agammaglobulinemia

et al. 2017

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Purpose X-linked agammaglobulinemia is a primary humoral immunodeficiency characterized by hypogammaglobulinemia and increased susceptibility to infection. Although there is increased awareness of autoimmune and inflammatory complications in XLA, the spectrum of gastrointestinal manifestations has not previously been fully explored. Methods We present a case report of a family with two affected patients with XLA. Given the gastrointestinal involvement of the grandfather in this family, we performed a retrospective descriptive analysis of XLA patients with reported diagnoses of GI manifestations and inflammatory bowel disease (IBD) or enteritis registered at the USIDNet, a national registry of primary immunodeficiencies. Results In this cohort of patients with XLA, we found that up to 35% had concurrent gastrointestinal manifestations, and 10% had reported diagnoses of IBD or enteritis. The most commonly reported mutations were missense, which have been associated with a less severe XLA phenotype in the literature. The severity of symptoms were wide-ranging, and management strategies were diverse and mainly experimental. Conclusions Patients with XLA may require close monitoring with particular attention for GI manifestations including IBD and infectious enteritis. Further studies are needed to improve diagnosis and management of GI conditions in XLA patients.

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“…For many years, the serologic diagnosis of coeliac disease was based on the detection of IgA anti‐endomysial antibodies (EMA), IgA and IgG antigliadin antibodies (AGA), and antitissue transglutaminase antibodies (AtTGA) as well as IgA antireticulin antibodies . It has been demonstrated that the levels of these autoantibodies in the serum of PID patients are often lower than in patients without PID . For example, in CVID the serological diagnosis of coeliac disease is more difficultly and doubtfully made due to low production of both IgG and IgA antibodies, .…”

Section: Management Of Autoimmunity In Pid Patientsmentioning

confidence: 99%

Approach to the Management of Autoimmunity in Primary Immunodeficiency

Azizi

Ziaee

Tavakol³

et al. 2017

Scand J Immunol

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Primary immunodeficiency diseases (PIDs) consist of a genetically heterogeneous group of immune disorders that affect distinct elements of the immune system. PID patients are more prone to infections and non-infectious complications, particularly autoimmunity. The concomitance of immunodeficiency and autoimmunity appears to be paradoxical and leads to difficulty in the management of autoimmune complications in PID patients. Therefore, management of autoimmunity in patients with PID requires special considerations because dysregulations and dysfunctions of the immune system along with persistent inflammation impair the process of diagnosis and treatment.

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Gastrointestinal and hepatic manifestations of primary immune deficiency diseases

Cited by 31 publications

References 52 publications

Exploring the Sociodemographic and Clinical Features of Extrapulmonary Tuberculosis in Saudi Arabia

Exploring the Sociodemographic and Clinical Features of Extrapulmonary Tuberculosis in Saudi Arabia

Gastrointestinal Manifestations in X-linked Agammaglobulinemia

Approach to the Management of Autoimmunity in Primary Immunodeficiency

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