2018
DOI: 10.3389/fimmu.2018.01308
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Abstract: BackgroundPrimary immunodeficiency disorders (PIDs) are a group of heterogeneous rare disorders, whereby the immune system is missing or not functioning adequately. For patients requiring treatment, the most common option is immunoglobulin replacement therapy (Ig). Treatment of PIDs is simultaneously associated with both improvements in health-related quality of life (HRQoL) and increased treatment burden.ObjectivesThis review sought to review studies investigating the burden of Ig treatment, synthesize eviden… Show more

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Cited by 20 publications
(23 citation statements)
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References 39 publications
(71 reference statements)
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“…The use of disease-specific tools is desirable to provide a more accurate picture of the burden of disease [ 10 , 24 , 25 ]. The CVID_QoL questionnaire is the first specific instrument for CVID [ 10 ], and in addition to the burden of disease due to the complex clinical picture [ 26 ], the questionnaire analyzes also the experience of the so called “burden of treatment” [ 11 , 27 ]. In fact, the burden of treatment has also an impact on the needs of everyday life, in planning and managing one’s self-care, as well as complex treatments and their clinical monitoring, in addition to family/work commitments [ 28 ].…”
Section: Discussionmentioning
confidence: 99%
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“…The use of disease-specific tools is desirable to provide a more accurate picture of the burden of disease [ 10 , 24 , 25 ]. The CVID_QoL questionnaire is the first specific instrument for CVID [ 10 ], and in addition to the burden of disease due to the complex clinical picture [ 26 ], the questionnaire analyzes also the experience of the so called “burden of treatment” [ 11 , 27 ]. In fact, the burden of treatment has also an impact on the needs of everyday life, in planning and managing one’s self-care, as well as complex treatments and their clinical monitoring, in addition to family/work commitments [ 28 ].…”
Section: Discussionmentioning
confidence: 99%
“…A better self-reported HRQoL has been described among patients who switched from IVIG to SCIG self-infusions at home [ 33 35 ], even though this improvement seems to be largely related to a switch to home-therapy rather to the SCIG therapy itself [ 36 38 ]. Moreover, several studies evaluating health economics in SCIG and IVIG therapy reported that SCIG was a more cost-effective modality, mainly through the reduction of productivity loss and days in hospital [ 33 , 39 42 ]. On the other hand, in previous studies analyzing HRQoL after shifting from IVIG to SCIG, it was demonstrated that elderly patients’ approach to SCIG home therapy could be limited by several factors, including the perception of inconvenience of time consumed with self-administration, the aversion to undertake subcutaneous self-injection, or by the anxiety of possible adverse reactions at home [ 43 ].…”
Section: Discussionmentioning
confidence: 99%
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“…The potential utility of therapy with human immunoglobulin for intravenous use (IVIG) has been recently reported by our group [55], based on the similar etiology and inflammatory pathogenesis of SARS-CoV-2 infection to diseases for which the use of IVIG has already been approved by the FDA or EMA (Table 1) [56][57][58][59][60][61][62][63], or for which IVIG are employed off-label (Table 2) [30,, including viral or bacterial septic shock [91] and the autoinflammatory syndrome with MAS [91][92][93]. To dam inflammation, IVIG should be given at a dosage not exceeding 0.4 g/kg/day for three to five consecutive days (maximum total dosage is 2 g/kg) and the patient should be well hydrated.…”
Section: Immunological Rationale For Targeting the Immune System To Fmentioning
confidence: 99%
“…The IVIG preparation consists of highly purified immunoglobulins (Ig), mostly of the IgG class, obtained from between 1,000 and 15,000 healthy donors per batch [17,18]. Therefore, the majority of these molecules are natural antibodies with polyreactive properties, that can recognize and neutralize different pathogenic exogenous antigens (viral or bacterial antigens / toxins and superantigens) [19], as in the case of coronavirus infection, or endogenous antigens (i.e., cytokines, chemokines and metalloproteases), as in the case of CAPs and SCLLS [9,20] or of Kawasaki disease, parvovirus infection or streptococcus-derived superantigens, hypothesized to trigger the vasculitis [21].…”
Section: Human Immunoglobulin For Intravenous Use (Ivig) Can Be Usefumentioning
confidence: 99%