Abstract
Lymphoid interstitial pneumonia (LIP) is a rare lymphoproliferative disease. LIP in common variable immunodeficiency (CVID) was observed in a patient during immunomodulatory therapy after progression of the disease (i.e., glucocorticoids, immunoglobulin dose escalation, and finally rituximab). Due to humoral immunodeficiency and serious serum sickness rituximab was used initially at a low dose (150 mg/m2 weekly). It resulted in temporary remission with the decrease of serum paraproteinemia, β2-microglobulin (β2M) and SUV decrease as well as increase of FVC. Owing to the relapse after 6-month remission in the second cycle a standard dose of rituximab was used (375 mg/m2). Therapeutic regimen with 375 mg/m2 of Rtx in optimal schedule (i.e., every 3 weeks) resulted in no longer remission but higher incidence of opportunistic infections. Finally, after another cycle of immunotherapy FVC, paraproteinemia and β2M level normalization were observed as well as the decrease of severe splenomegaly. In laboratory and immunological progress the increase of NK and NKT cells was observed after the initial dose but the standard one caused NK cell increase only. Unfortunately, the decrease of CD19+Bcells was comparable between both doses, as was the decline of FoxP3+ regulatory T cell. On the contrary, after the low dose absolute T cell (both CD4 and CD8) number decreased but after the standard one – it normalized. Rtx (especially in low dose) brought further increase of persistent T cell activation (CD38+ T cells made up 79%). Innate immune response and the decrease of Treg are a compensatory pathways for the decrease of B and T cells. Immunodeficiency requires a different investigative approach to a immunotherapy.Clinical Trial Registration: ClinicalTrials.gov, NCT02789397.
Highlights
Lymphoid interstitial pneumonia (LIP) is a rare restrictive disease that usually coexists with immunodeficiency (Hurst et al, 2017; Zdziarski et al, 2017), classified as lymphoproliferative disease (LPD)
Rituximab therapy shows a significant inhibitory effect on CMV-specific CD8+ cytotoxic T lymphocyte (CTL) levels and interferon gamma release, but RTx-induced CD38 expression on T cells was higher after the low dose (Table 1)
Normalization of high percentage of CMV pp65-specific CTL, increase of forced vital capacity (FVC), regression of radiographic and positron emission tomography (PET) abnormalities several years after lymphoid interstitial pneumonia/pneumonitis (LIP) onset indicate that lymphocytic infiltration of the alveolar interstitium and pulmonary defect are reversible, contrary to COPD
Summary
Lymphoid interstitial pneumonia (LIP) is a rare restrictive disease that usually coexists with immunodeficiency (Hurst et al, 2017; Zdziarski et al, 2017), classified as LPD. The restrictive, granulomatous lung disease developed: open lung biopsy and histological examination showed lymphocytic infiltration of interstitial tissue: LIP diagnosis was confirmed by the histologic examination as well as T and B cells repertoire analysis as described previously (Zdziarski et al, 2017). The decrease of spleen size and leading biochemical and serological parameters were observed (i.e., β2M and IgM level, see Figures 1, 2) in line with LIP regression and FVC increase. Absolute number of natural killer T cells (NKT, CD3+56+) increased temporarily after low and standard dose (Figure 2). Rituximab therapy shows a significant inhibitory effect on CMV-specific CD8+ cytotoxic T lymphocyte (CTL) levels and interferon gamma release, but RTx-induced CD38 expression on T cells was higher after the low dose (Table 1). A low dose causes increase of post-mitogenic (PHA) interferon release
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