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Sjogren's Induced CD4+ Lymphocytopenia Causing Pulmonary MAC Infection FREE TO VIEW

Kasey Treger, MD; Sajjad Bhatti, MBBS; Faisal Usman, MD; Hammad Bhatti, MD
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University of Florida, Jacksonville, FL

Chest. 2013;144(4_MeetingAbstracts):185A. doi:10.1378/chest.1689635
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SESSION TITLE: Infectious Disease Case Report Posters III

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM

INTRODUCTION: Sjogren’s Syndrome (SS) is known to cause lymphocytopenia, and there have been cases of opportunistic infections described in patients with Sjogren’s disease without evidence of AIDS(1) .To our knowledge, there has been no report describing MAC infection due to chronic lymphocytopenia induced by Sjogren’s syndrome.

CASE PRESENTATION: A 38F with insignificant medical history presented to ER with complaints of fever, dyspnea, and productive cough for 6 months. She was initially hospitalized at another facility 3 months prior with same complaints and discharged on azithromycin. She was later notified that she had grown mycobacterium avium complex from respiratory cultures. She was offered treatment but was unable to afford. HIV testing done at outside hospital as well as repeat testing at our institution were negative. During hospitalization at our institute, she had sputum that was positive for AFB however, TB PCR returned negative. CT chest showed severe bilateral upper cavitary lung disease (Fig;1). She was started on ethambutol, rifabutin and clarithromycin. Autoimmune testing revealed positive SSA (RO) antibody. Blood flow cytometric analysis did not reveal evidence of myeloproliferative disorder however, absolute CD4 helper T cells was 95/ul.

DISCUSSION: US Centers for Disease Control (CDC) defines idiopathic CD4+ T lymphocytopenia (ICL) as: prolonged (at least 2) low counts of <300/mm3 or <20% CD4+ without HIV infection or other known causes of immunosuppression. The association between SS and ICL has already been observed previously. Pulmonary MAC is an AIDS defining illness and commonly presents in HIV positive patients with low CD4 counts. In our patient, susceptibility was due to a profound and persistent depletion of CD4+ T cells. No infection, therapy, malnutrition or defined immunodeficiency could be implicated. Opportunistic infections including pulmonary MAC infections with idiopathic CD4+ T-cell lymphocytopenia have been well documented in the literature. CD4+ T-lymphocytopenia - grouped has been a frequent finding in anti-SSA Ab patients with primary SS (up to 16%) (2).

CONCLUSIONS: Patients with unexplained CD4+ lymphocytopenia should be carefully evaluated for an occult Sjogren’s syndrome. There are reports suggesting that IL-2 therapy for ICL as an option to increase CD4 counts.

Reference #1: 1.Delaleu N, Immervoll H, Cornelius J, Jonsson R (2008). "Biomarker profiles in serum and saliva of experimental Sjögren's syndrome: associations with specific autoimmune manifestations". Arthritis Res. Ther. 10 (1): R22.

Reference #2: 2.Mandl T, Bredberg A, Jacobsson LT, Manthorpe R, Henriksson G. CD4+ T-lymphocytopenia--a frequent finding in anti-SSA antibody seropositive patients with primary Sjögren's syndrome. J Rheumatol. 2004 Apr;31(4):726-8.

DISCLOSURE: The following authors have nothing to disclose: Kasey Treger, Sajjad Bhatti, Faisal Usman, Hammad Bhatti

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