Late Onset Combined Immunodeficiency Presenting with Recurrent<i>Pneumocystis jiroveci</i>Pneumonia

Papakonstantinou, Ilias; Baraboutis, Ioannis G.; Karnesis, Lazaros

doi:10.1155/2014/801805

Cited by 6 publications

(3 citation statements)

References 30 publications

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“…Common respiratory complications in CVID patients include pneumonia, sinusitis and bronchiectasis [ 9 ]. With regards to PJP , there only have been several case reports of patients with CVID and concomitant lymphopenia who were found to have Pneumocystis pneumonia [ 10 , 11 ]. However, our patient had a normal serum lymphocyte count, specifically T cell count at time of diagnosis.…”

Section: Discussionmentioning

confidence: 99%

Cavitary lung lesions caused by Pneumocystis jirovecii in setting of common variable immune deficiency

Zhang

Kern

Joshi

et al. 2020

Respiratory Medicine Case Reports

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Section: Discussionmentioning

confidence: 99%

Cavitary lung lesions caused by Pneumocystis jirovecii in setting of common variable immune deficiency

Zhang

Kern

Joshi

et al. 2020

Respiratory Medicine Case Reports

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“…The outcome turned out to be unfavorable in 12 out of 18 patients (66.7%), thus confirming the high mortality rate in these patients reported in literature, in particular compared with immunosuppressed HIVpositive patients. [70][71][72][73][74][75][76][77][78][79][80][81][82][83][84][85][86][87][88]…”

Section: Discussionmentioning

confidence: 99%

Pneumocystis pneumonia in HIV-negative immunocompromised patients in Internal Medicine ward

et al. 2020

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Pneumocystis jirovecii pneumonia (PJP) is an opportunistic infection typically observed in AIDS patients, for whom it represents a leading cause of death. However, its incidence among HIV-negative immunocompromised patients is progressively increasing, with a significantly higher mortality compared to that of AIDS-patients. We performed a retrospective observational study on HIV-negative patients with PJP. We aimed to determine their epidemiological features and their biohumoral and therapeutic variables, searching for a correlation between them and our patients’ outcome. We included all patients admitted to our Internal Medicine ward from January 2010 to June 2015, who were immunocompromised at the time of admission and had microbiologically confirmed PJP (association between compatible clinical-radiological findings and qualitative polymerase chain reaction positivity on bronchoalveolar lavage). Their immune impairment was assessed considering both their medical history and their complete white blood cells (WBC), differential WBC and their CD4 cell count. Transfer to Intensive Care Unit (ICU) or death was considered as an unfavorable clinical outcome, while hospital discharge or transfer to a non-ICU ward was considered as a favorable outcome. We included a total of 18 patients in our statistical analysis. We used Student’s t-test and Fischer’s χ-square test to compare, respectively, normally distributed continuous variables and non-continuous variables. Our patients’ mean age was 65±13.9 years. All of them had cancer, mostly hematological malignancies (13/18), notably non-Hodgkin lymphoma (NHL; 8/13). They were all being or had been recently treated with chemotherapy (10/18) and/or high-dose glucocorticoids, with full dose or during tapering (13/18). Statistical analysis of blood tests results showed a significant difference between mean serum lactate dehydrogenase (LDH) concentration in the group of patients with favorable vs unfavorable outcome. Also, mean serum immunoglobulins G (IgG) concentration and certain arterial blood gas findings (mean arterial paO2/FiO2, mean blood Ph and mean paCO2) at the time of admission were significantly different in the two groups of patients. 12/18 patient’s outcome turned out unfavorable. Trimethoprim + sulfamethoxazole (TMP+SMX) treatment was given to all our patients, with a mean duration of 13.39±9.36 days. Patients with a favorable outcome had received TMP+SMX treatment significantly earlier than those with an unfavorable outcome. Hematological malignancies, according to literature, confer the strongest predisposition to PJP. Both chemotherapy and high-dose Glucocorticoid treatment are well known predisposing factors. A remarkable elevation of serum LDH represents both a typical clinical feature and a well-known negative prognostic factor in PJP. Low IgG levels have never been reported as a negative prognostic factor, but their role in enhancing macrophage killing of pneumocystis may account for the worst observed prognosis in the group of patients with lower mean levels. Therefore, in order to reduce the heavy mortality rate associated with PJP, an early beginning of specific treatment is of utmost importance, and even if this is certainly true for many infectious diseases, the time gap is particularly limited in the setting of this type of pneumonia. Hence, PJP should be ruled out as soon as possible and, in case of a strong clinical- radiological suspicion, therapy should be started immediately, even while waiting for microbiological confirmation (especially in critically-ill patients).

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“…Other immunodeficiencies (e.g., complement deficiency and late-onset combined immunodeficiency) that present in adulthood are usually due to hypomorphic or compound heterozygous mutations in PID genes with an autosomal recessive pattern of inheritance or polygenic diseases [96,97]. In the context of this hypothesis, the male-female ratio of PIDs during childhood is 5:1, but the ratio approaches 1:1 in adults [94].…”

Section: Pid Diagnostic Clues In Adultsmentioning

confidence: 99%

Important differences in the diagnostic spectrum of primary immunodeficiency in adults versus children

Abolhassani

Rezaei

Mohammadinejad

et al. 2015

Expert Review of Clinical Immunology

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Primary immunodeficiency disorders (PIDs) constitute a heterogeneous group of genetic disorders caused by defects in immunity, leading to recurrent infections, autoimmunity, lymphoproliferation and malignancies. Early diagnosis of PIDs is crucial for improving the quality of life in patients with PIDs while a delay in diagnosis, or inadequate treatment, results in an increased mortality and morbidity in affected individuals. Although most cases of PIDs present in children with recurrent and/or severe acute infections, some of the primary immune disorders are diagnosed during adulthood. Some common clues, both in children and adults, help physicians to diagnose PIDs; however, there are some specific clues to the diagnosis of PIDs for each group. This article reviews the important differences in the diagnostic spectrum of PIDs in adults versus children.

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Late Onset Combined Immunodeficiency Presenting with RecurrentPneumocystis jiroveciPneumonia

Cited by 6 publications

References 30 publications

Cavitary lung lesions caused by Pneumocystis jirovecii in setting of common variable immune deficiency

Cavitary lung lesions caused by Pneumocystis jirovecii in setting of common variable immune deficiency

Pneumocystis pneumonia in HIV-negative immunocompromised patients in Internal Medicine ward

Important differences in the diagnostic spectrum of primary immunodeficiency in adults versus children

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