SESSION TITLE: Pulmonary Manifestations of Systemic Disease Student/Resident Case Report Posters I
SESSION TYPE: Student/Resident Case Report Poster
PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM
INTRODUCTION: Pneumocystis Pneumonia (PCP) remains an important cause of mortality in non-HIV patients receiving immunosuppresive drugs. The risk of PCP in patients receiving Rituximab has been underestimated considering its primary effects on CD20+ B-cells. We present a case of fatal PCP in a non HIV patient who received rituximab for a flare of cryoglobulinemic vasculitis.
CASE PRESENTATION: A 59-yr-old female with a history of untreated Hepatitis C, depression and heroin use was admitted to the hospital with worsening renal function and palpable purpura on both her extremities. Her laboratory findings of elevated inflammatory markers, low C4, positive RA factor, cryoglobulinemia and leukocytoclastic vasculitis on skin biopsy confirmed the diagnosis of mixed cryoglobulinemic vasculitis(Table 1). She signed out AMA prior to initiation of therapy. Few months later, she returns with a flare of vasculitis. Renal biopsy confirmed MPGN type 1 for which she received two doses of rituximab 1000mg IV, two weeks apart with tapering steroids. Upon discharge, her overall condition was improved remarkably. Two months later, she was readmitted to MICU with severe respiratory failure found to be having Pneumocystis on GMS stain of bronchoalveolar lavage. Despite maximal medical therapy, she died on hospital day nine.
DISCUSSION: Rituximab is a treatment option for a growing number of malignant, inflammatory and autoimmune conditions. It has shown efficay in Hepatitis C associated mixed cryoglobulinemic vasulitis without any reported cases of PCP in the study population. In vitro studies demonstrated that rituximab interferes with T-cell activation both by depriving B-cell APCs and through its direct effect on T cells. This results in a transient, but significant decrease in T-cell inflammatory and proliferation capacity in response to ‘foreign’ stimuli leading to T-cell dependent opportunistic infections . Few reported cases of rituximab related progressive multifocal leukoencephalopathy signifies a role of T-cell suppression. However, recent animal studies have also indicated a role for B lymphocytes and antibody defenses in these opportunistic infections .
CONCLUSIONS: PCP has been reported in patients treated with rituximab for hematologic malignancies. Our case highlights the potential risk of PCP in patients being treated with rituximab for rheumatological indications and that further studies are necessary to consider if routine PCP prophylaxis should be warranted in such individuals.
Reference #1: Petrarca A, Rigacci L, et al. Safety and efficacy of rituximab in patients with hepatitis C virus-related mixed cryoglobulinemia and severe liver disease. Blood. 2010;116(3):335-342.
Reference #2: Stroopinsky D, Katz T, et al. Rituximab-induced direct inhibition of T-cell activation. Cancer Immunol Immunother 2012; 61: 1233.
Reference #3: Lund FE, et al. B cells are required for generation of protective effector and memory CD4 cells in response to Pneumocystis lung infection. J Immunol. 2006;176:6147-6154.
DISCLOSURE: The following authors have nothing to disclose: Imam Shaik, Elvis Patel, Rumana Khan, Valentin Marian, Jyoti Matta
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