Background Systemic lupus erythematosus (SLE) is a rare complex autoimmune disease with a multisystem involvement [1]. Although pulmonary involvement is relatively frequent in adult patients; it has been rarely reported in children with SLE. It may be an initial and/or life-threatening complication [2]. There is a scarcity of published literature on pulmonary function tests (PFTs) in patients with JSLE manifestations of pulmonary disease. Objectives To verify a subclinical pulmonary disease in juvenile onset SLE using laboratory parameters, pulmonary functions tests and multislice CT Methods Twenty five patients with JSLE were studied. All fulfilled updated American College of Rheumatology criteria for SLE [3]. All patients had the disease at or before the age of 16 years. Assessment of disease activity was done through SLE Disease Activity Index (SLEDAI) [4]. PFTs were done using (ZAN 100 Flow Handy II) pulmonary function apparatus. PFTs were done according to Grippi and Tino [5]. They included forced expiratory volume in 1 s as FEV1 (liter/min) and forced expiratory flow rate FEV1% of predicted FEV1, forced vital capacity FVC, residual volume RV, total lung capacity TLC, FEF 25-75%, FEF50% and FEF75%, and diffusion capacity of carbon monoxide DLCO. Abnormal PFTs were categorized into restrictive, obstructive, mixed restrictive, and obstructive, and small airway disease. Laboratory and immunological parameters were done for ANA, anti-dsDNA, ENA including Sm, and La, U1-snRNP, C3, C4, RF, Acl and LA. Plain radiography for chest and multislice CT were done. Results Twelve patients (48%) had abnormal results of PFTs, 5 (20%) had an isolated (DLCO) impairment and 6 (24%) had a restrictive pattern. Neurological lupus was significantly associated with decreased FEF25-75% (P<0.04) and FEF75% (P<0.001) respectively. Radiography was normal. Abnormal PFTs were not significantly associated with other parameters. Multislice CT examination of JSLE patients demonstrated a pleural effusion and thickening in 16%, also ground glass opacities found in 16% suggesting early interstitial lung disease. Conclusions A mandatory performance of pulmonary function tests have to be done early in JSLE, without findings of pulmonary involvement. References Torre O, Harari S. Pleural and pulmonary involvement in SLE Presse Med 2011;40 1 Pt 2):e19–29. Diane L, Kamen MD, Strange C. Pulmonary Manifestations of SLE Clin Chest Med 2010;31:479–88.2004; 43:587–91. Hochberg MC. Diagnostic and therapeutic criteria committee of the American college of rheumatology updating the American college of rheumatology revised criteria for the classification of SLE. Arthritis Rheum 1997; 40:1725. Bombardier C, Gladman DD, Urowitz MB, Caron D, Chang CH. Derivation of the SLEDAIA disease activity index for lupus patients. The committee on prognosis studies in SLE. ArthritisRheum 1992;35:630–40. Grippi MA, Tino G. Pulmonary function testing. In: Fishman AP, Elias JA, Fishman JA, Grippi MA, Senior RM, Pack AI, editors. Fishman’s pulmonary diseases and disorders...
Background Systemic lupus erythematosus (SLE) persists as a chronic inflammatory autoimmune disease. It is characterized by the production of autoantibodies and immune complexes that affect multiple organs (Seitz, 2010). The etiology of SLE is not known, but aberrant apoptosis and/or insufficient clearance of apoptotic material have been assigned a pivotal role (Fransen, 2010). Dendritic cells (DCs) represent an important component of the immune system connecting the innate and adaptive immune responses (Gerl, 2010). SLE patients have increase in number of the plasmacytoid (pDCs) per peripheral blood mononuclear cells but have low number of myeloid myeloid (mDCs) (Lande and Gilliet, 2010). Objectives To investigate dendrtic cells (DCs) types, plasmacytoid (pDCs) and myeloid (mDCs) in the peripheral blood of SLE patients and prove a role of a ratio of apoptotic cell/DCs in pathogenesis of SLE Methods This study included 29 SLE patients, 28 females and 1 male with a mean age of 27.13±7.46, who were classified into two groups, SLE patients in activity (16 patients) and SLE patients in remission (13 patients). They were compared with 20 normal controls. All patients were submitted to full history taking, clinical examination using SLEDAI, routine laboratory investigations by complete blood and urine analysis. Blood samples analysis was done using FACS Caliber. Flow cytometric analysis of DCs types, lineage cells and apoptosis (early and late), calculation of (apoptosis/mDCs) ratios were performed in a unit of South Egypt Cancer Institute. Results mDCs and activated mDCs percentage are decreased in SLE patients and SLE in activity. Late apoptosis percentage is increased. pDCs and activated pDCs percentage are increased in SLE patients but they are decreased during SLE activity. Mature APCs (including DCs) had increased expression of CD86 but low expression of HLA DR. Percentage of early apoptosis is increased in SLE patients; this may be due to impaired clearance of apoptotic cells due to a decrease in mDCs percentage. Increase of the early apoptosis percentage in SLE in remission may be considered impending factor to activity. Percentage of late apoptosis is increased in SLE patients in activity may be due to decrease in activated mDCs percentage in SLE in activity and impaired phagocytic function of activated mDCs. Early apoptosis/whole mDCs ratio is increased in SLE patients than normal controls. This explains the increase of apoptosis percentage in SLE patients and that may be an important factor in SLE pathogenesis Conclusions The increase of apoptosis percentage in SLE patients may be an important factor in SLE pathogenesis. A ratio of apoptotic cell/DCs has an important role in pathogenesis of SLE. References Dendritic cells in SLE. Seitz HM, Int Rev Immunol 01-APR-2010; 29(2): 184-209. The role of dendritic cells in the pathogenesis of SLE. Fransen JH, Arthritis Res Ther 01-JAN-2010; 12(2): 207. Blood dendritic cells in SLE exhibit altered activation state and chemokine receptor function. Gerl V, Ann Rheu...
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