Simultaneous presentation of hemophagocytic syndrome and mesenteric vasculitis in a patient with systemic lupus erythematosus

We report an 18-year old female patient with systemic lupus erythematosus (SLE), who developed fever, pancytopenia, abdominal pain, and watery diarrhea. Computed tomography (CT) and bone marrow aspirate revealed lupus mesenteric vasculitis (LMV) and hemophagocytic syndrome (HPS). Serologic tests for Epstein–Barr virus (EBV) indicated its reactivation. This case demonstrates that HPS and concomitant LMV associated with viral reactivation can occur as clinical manifestations of SLE flare.     

Systemic lupus erythematosus presenting as hyponatremia-associated rhabdomyolysis: A case report

Rationale:Systemic lupus erythematosus (SLE) is an autoimmune disease that involves multiple organs and causes various clinical manifestations. Cases of rhabdomyolysis as the initial presentation of SLE are rare, and there are no reported cases of SLE presenting hyponatremia-associated rhabdomyolysis as the first manifestation. Herein, we report a case of SLE with lupus nephritis in a patient with acute hyponatremia-associated rhabdomyolysis.Patient concerns:A 44-year-old woman was admitted with complaints of altered consciousness, myalgia, and red-brownish urine that first appeared three days prior. Peripheral blood tests revealed elevated creatine kinase (19,013 IU/L) and myoglobin (5099 U/L) levels and severe hyponatremia (111 mEq/L) with no azotemia. Urinalysis showed nephritic sediments.Diagnosis:Whole-body bone scintigraphy showed increased uptake of radiotracer in the both upper and lower extremities. Serological evaluation revealed the presence of anti-nuclear (speckled pattern, 1:640), anti-double stranded DNA, and anti-Smith antibodies and absence of anti-Jo-1 antibody. A kidney biopsy demonstrated mesangial proliferative (class II) lupus nephritis.Interventions:Fluid therapy, including intravenous administration of 3% NaCl, was initiated. After three consecutive days of intravenous methylprednisolone (1 g/d), oral prednisolone (1 mg/kg/d), mycophenolate mofetil, and hydroxychloroquine were administered.Outcomes:On day 28, the patient was discharged with marked resolution of SLE-associated symptoms and laboratory findings. Lupus reactivation was not present during the subsequent six-month follow-up.Lessons:Hyponatremia-associated rhabdomyolysis can be the first manifestation of SLE. Moreover, prompt fluid therapy and timely administration of immunosuppressive agents in SLE patients presenting with hyponatremia and rhabdomyolysis can significantly help alleviate disease activity and improve clinical outcomes.     

Mesenteric vasculitis in children with systemic lupus erythematosus

Clinical Rheumatology - Lupus mesenteric vasculitis (LMV) is a severe and potentially fatal complication of systemic lupus erythematosus (SLE). Although LMV is always a consideration in adolescents...     

狼疮肠系膜血管炎30例临床分析

目的 通过分析狼疮肠系膜血管炎(LMV)患者的临床资料,提高对LMV的认识.方法 回顾性分析30例LMV住院患者的临床表现、实验室检查及治疗情况等.结果 (1)30例LMV患者中腹痛23例(76.7%),腹泻21例(70%),恶心和(或)呕吐20例(66.7%);(2)28例合并其他系统损害,以狼疮性肾炎(24例)、血液系统(18例)为多见;并发输尿管肾盂积水10例,假性肠梗阻8例;(3)24例患者病情处于活动期(系统性红斑狼疮疾病活动指数≥10);10例患者白细胞和(或)补体C3随病情进展呈进行性下降;(4)抗SSA、U1RNP抗体阳性率分别为55.6%(15/27)、51.9%(14/27);(5)14例腹部CT示肠管壁"靶形征"或肠系膜血管"栅栏征"样改变;(6)27例患者加大糖皮质激素剂量后症状迅速改善.结论 LMV临床表现以腹痛、腹泻、呕吐为主;LMV常与其他系统损害并存,多处于病情活动,为重症系统性红斑狼疮表现之一;白细胞或补体C3动态下降可能与LMV有关;抗SSA抗体、抗U1RNP抗体与LMV关系有待进一步研究;LMV对大剂量糖皮质激素治疗反应良好.     

Reports of three cases with the initial presentation of mesenteric vasculitis in children with system lupus erythematous

Clinical Rheumatology - We reviewed three cases of systemic lupus erythematosus (SLE) in children with mesenteric vasculitis (LMV) as initial presentation and analysed their clinical...     

Serum β2-microglobulin level is a useful indicator of disease activity and hemophagocytic syndrome complication in systemic lupus erythematosus and adult-onset Still’s disease

This study demonstrates whether serum β₂-microglobulin (β₂-MG) level can be an indicator of the status of systemic lupus erythematosus (SLE) and adult-onset Still’s disease (AOSD), and development of hemophagocytic syndrome (HPS) complication. Serum β₂-MG level was compared between the active and inactive statuses of SLE and AOSD in hospitalized patients. Active status was defined as a state for which a therapy was introduced. Serum β₂-MG level was also compared between patients with and without HPS complication. HPS was diagnosed on the basis of clinical and pathological findings. Laboratory markers of HPS including peripheral blood cell counts and levels of serum lactate dehydrogenase (LDH), serum ferritin, plasma fibrin/fibrinogen degradation product (FDP), and plasma D-dimer were examined to determine their correlations with serum β₂-MG level. Sixteen SLE and seven AOSD patients (all females, aged 39.0?±?16.4) were included. The serum β₂-MG level was high in the active status of underlying diseases and decreased significantly after the therapy (3.5?±?1.4 vs. 2.1?±?0.8 mg/L, p?<?0.001). Among patients with active status, the β₂-MG level was higher in patients with HPS (two with SLE and three with AOSD) than in patients without HPS (4.9?±?1.8 vs. 3.3?±?1.4 mg/L, p?<?0.05). Serum β₂-MG level significantly correlated with the levels of serum LDH (r _s?=?0.42, p?<?0.05), plasma FDP (r _s?=?0.58, p?<?0.05), and plasma D-dimer (r _s?=?0.77, p?<?0.01). Serum β₂-MG level would be a useful indicator of disease activity and development of HPS complication in patients with SLE and AOSD.     

Y chromosome microchimerism in patients with systemic lupus erythematosus

Aim of the workTo identify the association of Y chromosome microchimerism with systemic lupus erythematosus (SLE) and investigate its relation to pregnancy history, clinical and laboratory variables.Patients and methodsThe presence of Y chromosome microchimerism was screened in 90 females including 35 women with renal lupus, 25 patients with cutaneous lupus and 30 healthy control females using real time polymerase chain reaction. Demographic parameters, reproductive history, clinical and laboratory data were recorded.ResultsThe percentage of Y chromosome microchimerism was significantly higher in SLE patients compared to healthy women and in females with renal lupus versus those with cutaneous lupus. Among patients with renal lupus, Y chromosome microchimerism was strongly associated with disease activity and damage indices as well as with number of pregnancies, while this association was absent regarding number of pregnancies in cutaneous lupus females. A significant increase in disease activity and severity scores was detected in microchimeric renal lupus females when compared to microchimeric cutaneous lupus patients. Healthy control and cutaneous lupus women with microchimerism experienced significant decrease in number of pregnancies and abortions against microchimeric renal lupus group.ConclusionsThese results may provide a preliminary suggestion that Y chromosome microchimerism could have a pathogenic role in SLE and it may be related to inflammation, the extent of disease damage and reproductive history. Further studies are needed among SLE patients with different clinical presentations, as fetal microchimerism is a promising field of investigation which may lead to novel strategies in treatment.     

Lupus mesenteric vasculitis: Clinical features and associated factors for the recurrence and prognosis of disease

Objective

To evaluate the clinical characteristics of lupus mesenteric vasculitis (LMV) and identify the potential factors and appropriate treatments that are associated with disease relapse and prognosis in LMV.

Methods

A retrospective cohort study was performed among patients admitted to the First Affiliated Hospital of Sun Yet-sen University between 2002 and 2011. Demographic information, clinical symptoms, laboratory findings, imaging characteristics like abdominal CT scan, ultrasonography, medications including corticosteroid, cyclophosphamide, and other immunosuppressive agents, and outcomes were documented. The endpoints of the study were defined as occurrence of severe complications that needed surgical intervention, disease recurrence, or death.

Results

Out of 3823 systemic lupus erythematosus (SLE) patients, 97 were diagnosed with mesenteric vasculitis with the overall prevalence of 2.5%. Among these 97 LMV patients, 13 died because of serious complications (13/97, 13.4%) and 2 presented intestinal perforation during the induction therapy stage. The logistic regression multivariate analysis indicated that leukopenia [peripheral WBC, odds ratio (OR) = 0.640, 95% confidence interval (CI): 0.456–0.896, P = 0.009], hypoalbuminemia (serum albumin, OR = 0.891, 95% CI: 0.798–0.994, P = 0.039) and elevated serum amylase (OR = 7.719, 95% CI: 1.795–33.185, P = 0.006) were positively associated with the occurrence of serious complications, while intravenous cyclophosphamide (CYC) therapy inhibited the occurrence of serious complications (OR = 0.220, 95% CI: 0.053–0.903, P = 0.036). A total of 79 patients who achieved remission were followed-up for 2–96 months and 18 cases experienced disease relapse (18/79, 22.8%). The statistical analysis adjusted by Cox proportional hazards models indicated that high-dose CYC therapy (≥1.0 g/m²/month) was a protective factor for disease relapse and led to better outcomes [hazard ratio (HR) = 0.209, 95% CI: 0.049–0.887, P = 0.034], while the severe thickness of the bowel wall (>8 mm) was a risk factor (HR = 7.308, 95% CI: 1.740–30.696, P = 0.007). LMV and lupus cystitis occurred concurrently in 22 (22/97, 22.7%) patients, and the symptoms of urinary tract resolved after treatment with corticosteroid and immunosupressants.

Conclusion

LMV is one of the serious complications of SLE with high mortality. The current study demonstrated that leukopenia, hypoalbuminemia, and elevated serum amylase were associated with severe adverse events, while CYC therapy led to better outcomes during remission-induction stage. Severe thickness of the bowel was a risk factor while high-dose CYC therapy was a protective factor for disease relapse in intensification therapy stage. It is necessary to evaluate the urinary tract involvement once LMV is diagnosed due to the frequent coexistence of these 2 diseases.     

Lamivudine prophylaxis prevents hepatitis B virus reactivation in anti-HBc positive patients under rituximab for non-Hodgkin lymphoma

Backgound

A significant proportion of hepatitis B surface antigen (HBsAg) negative/anti-hepatitis B core antigen (anti-HBc) positive patients with non-Hodgkin lymphoma (NHL) undergoing rituximab-based chemotherapy (R-CT) may suffer hepatitis B virus (HBV) reactivation.

Intravascular lymphoma associated with systemic lupus erythematosus

We report a case of systemic lupus erythematosus (SLE) associated with intravascular (angiotropic) lymphoma. A 27‐year‐old woman had been suffering from uncontrolled severe eruptions for a long time and was admitted because of high fever due to hemophagocytic syndrome (HPS). As her SLE had not been well‐controlled by moderate doses of steroids and azathioprine, autoimmune associated HPS was first considered. She was initially treated with steroid pulses and γ‐globulin for HPS. However, chromosomal analysis of bone marrow cells revealed severe abnormalities. After malignant lymphoma‐associated HPS was diagnosed, chemotherapy was commenced in the intensive care unit with artificial respiration and continuous hemodiafiltration. The patient died of cerebral infarction at day 45. It is suggested that the SLE itself was associated with the development of the intravascular lymphoma rather than due to the azathioprine.     

Silica-associated systemic lupus erythematosus with lupus nephritis and lupus pneumonitis: A case report and a systematic review of the literature

IntroductionSeveral epidemiological studies have shown that silica exposure triggers the onset of systemic lupus erythematosus (SLE); however, the clinical characteristics of silica-associated SLE have not been well studied.Patient concernsA 67-year-old man with silicosis visited a primary hospital because of a fever and cough. His respiratory condition worsened, regardless of antibiotic medication, and he was referred to our hospital.DiagnosisThe patient showed leukopenia, lymphopenia, serum creatinine elevation with proteinuria and hematuria, decreased serum C3 level, and was positive for anti-double stranded DNA antibody, anti-nuclear antibody, and direct Coombs test. He was diagnosed with SLE. Renal biopsy was performed, and the patient was diagnosed with lupus nephritis (class IV-G(A/C) + V defined by the International Society of Nephrology/Renal Pathology Society classification). Computed tomography revealed acute interstitial pneumonitis, bronchoalveolar lavage fluid showed elevation of the lymphocyte fraction, and he was diagnosed with lupus pneumonitis.InterventionsPrednisolone (50 mg/day) with intravenous cyclophosphamide (500 mg/body) were initiated.OutcomesThe patient showed a favorable response to these therapies. He was discharged from our hospital and received outpatient care with prednisolone slowly tapered off. He had cytomegalovirus and herpes zoster virus infections during treatment, which healed with antiviral therapy.Review:We searched for the literature on sSLE, and selected 11 case reports and 2 population-based studies. The prevalence of SLE manifestations in sSLE patients were comparative to that of general SLE, particularly that of elderly-onset SLE. Our renal biopsy report and previous reports indicate that lupus nephritis of sSLE patients show as various histological patterns as those of general SLE patients. Among the twenty sSLE patients reported in the case articles, three patients developed lupus pneumonitis and two of them died of it. Moreover, two patients died of bacterial pneumonia, one developed aspergillus abscesses, one got pulmonary tuberculosis, and one developed lung cancer.ConclusionClose attention is needed, particularly for respiratory system events and infectious diseases, when treating patients with silica-associated SLE using immunosuppressive therapies.     

Hemophagocytic syndrome as one of main manifestations in untreated systemic lupus erythematosus: two case reports and literature review

Hemophagocytic syndrome (HPS) is an unusual but fatal disorder characterized by pancytopenia and activation of macrophages. We describe two cases of untreated systemic lupus erythematosus (SLE) with HPS that presented as one of the manifestations of SLE. The onset of HPS was after parturition for one patient, and after abortion for the other. Bone marrow examination revealed severe hemophagocytosis in both patients. One patient responded to pulsed methylprednisone alone, and the other responded to pulsed methylprednisone plus intravenous immunoglobulin (IVIG). We believe accurate diagnosis, intensive therapy, and sufficient supportive cares are essential in improving patients’ prognosis.     

Successful treatment of reactive hemophagocytic syndrome by plasmapheresis and high-dose γ-globulin in a patient with systemic lupus erythematosus

A 31-year-old woman who had been administered corticosteroid and immunosuppressive agents for systemic lupus erythematosus (SLE) without flare-up was diagnosed as having reactive hemophagocytic syndrome (HPS) with severe disseminated intravascular coagulation. The causative underlying disease was uncertain, but it was not the SLE itself. Her fulminant HPS with increased serum ferritin and inflammatory cytokines (sIL-2R, TNF-α, IL-6, and IFN-γ) was successfully treated with plasmapheresis and high-dose γ-globulin therapy. Received: December 24, 1999 / Accepted: June 28, 2000     

Mycobacterium avium complex-associated hemophagocytic syndrome in systemic lupus erythematosus patient: report of one case

Hemophagocytic syndrome (HPS) in systemic lupus erythematosus(SLE) patients has not commonly been reported. In this case study, we report the first case of Mycobacterium avium complex (MAC)-associated hemophagocytic syndrome in a patient with systemic lupus erythematosus (SLE). This SLE patient, a 15-year-old girl, had been on a high dose of prednisolone (> 0.5mg/kg/day) for more than 3 years. She presented with a spiking fever, hepatosplenomegaly, pancytopenia, hyperferritinemia and adult respiratory distress syndrome. Bone marrow examination revealed hemophagocytosis as well as non-caseating granulomatosis. There was no indication of SLE fare-up. She responded poorly to initial treatment with methyl-prednisolone, intravenous immumoglobulin, etoposide, and drugs for Mycobacterium tuberculosis including rifampin, ethambutol, isoniazid and pyramide. However, gastric lavage culture revealed MAC. Following treatment with clarithromycin, ciprofloxacin and amikacin, her condition gradually improved and she was discharged 3 months after admission. In SLE patients with pancytopenia and hyperferritinemia, MAC-associated HPS should be considered in the differential diagnosis.     

Neutrophil to lymphocyte and platelet to lymphocyte ratios in systemic lupus erythematosus: Relation with disease activity and lupus nephritis

ObjectivesTo investigate the role of neutrophil to lymphocyte ratio (NLR) and platelet to lymphocyte ratio (PLR) as activity markers in systemic lupus erythematosus (SLE) without nephritis and lupus nephritis (LN) patients.Patients and methodsThis study included 60 SLE patients with LN, 60 SLE patients without renal involvement and 30 healthy controls. We analyzed correlations between NLR and PLR and both disease activity and renal affection.ResultsThe NLR of SLE patients was much higher than those of the controls. Both ratios showed significantly increased values in SLE patients with active disease. NLR and PLR were positively correlated with SLEDAI, ESR, and CRP and negatively correlated with C4. SLE patients with LN had higher levels of NLR than those without nephritis. NLR showed positive correlations with BUN, serum urea, serum creatinine and 24 h urinary protein. We found NLR to be related to anti-ds-DNA level and renal biopsy classes. While PLR was related only to anti ds-DNA. The best NLR to predict SLE active disease was 2.2 and the best PLR cut-off value was 132.9.ConclusionNLR and PLR are useful inflammatory markers to evaluate disease activity in SLE patients. Also, NLR could reflect renal involvement in SLE patients and is associated with the different classes of its histological staging.     

A regulatory role for CD72 expression on B cells in systemic lupus erythematosus

BackgroundB regulatory cells and their regulatory products/markers, such us semaphorin 3A (sema3A) and its receptor NP-1, FcγIIB, IL-10, and others, act at the very base of self-tolerance, maintenance, and prevention of autoimmune disease development.ObjectivesThe aim of the present study was to assess the involvement of CD72, a regulatory receptor on B cells, in systemic lupus erythematosus (SLE). In addition, the potential of soluble sema3A in enhancing the expression of CD72 on B cells of SLE patients was investigated.ResultsCD72 expression on activated B cells of SLE patients was significantly lower than that of normal controls. This lower expression of CD72 in SLE patients correlated inversely with SLE disease activity and was associated with lupus nephritis, the presence of anti-dsDNA antibodies, and low levels of complement. Co-culture of purified B cells from healthy controls with condition-media containing recombinant sema3A resulted in significant enhancement of CD72. Similar enhancement of CD72 on activated B cells from SLE patients, though significant, was still lower than in normal individuals.ConclusionsThe lower expression of CD72 on activated B cells from SLE patients correlates with SLE disease activity, lupus nephritis, the presence of anti-dsDNA antibodies, and low levels of complement. The improvement of CD72 expression following the addition of soluble semaphorin 3A suggests that CD72 may be useful as a biomarker to be followed during the treatment of SLE.     

Successful cessation of lamivudine using interferon in a patient with chronic hepatitis?B who received prophylactic lamivudine treatment during chemotherapy

Lamivudine (LMV) prophylaxis is effective in preventing hepatitis B virus (HBV) reactivation in patients with chronic hepatitis B undergoing chemotherapy. However, the optimal duration of LMV prophylaxis remains unclear. We report herein the case of a woman with localized follicular B-cell lymphoma who received chemotherapy with LMV prophylaxis. She achieved complete response to lymphoma, and LMV treatment was continued for 8 months after completion of chemotherapy. HBV status was still inactive. LMV was then stopped, but reactivation of hepatitis developed 1 month after cessation of LMV. LMV was restarted, resulting in successful treatment of reactivated hepatitis. Interferon (IFN) was used for 6 months before withdrawal of LMV, which was successfully ceased without flare hepatitis. This report describes the utility of sequential therapy with LMV and IFN to treat flare after withdrawal of LMV in hepatitis B carriers who receive LMV prophylaxis during chemotherapy and to prevent flare after withdrawal of LMV.     

High sensitivity C-reactive protein (hsCRP): Association with clinical subsets in systemic lupus erythematosus (SLE) patients from Western India

BackgroundHigh-sensitivity C-reactive protein (hsCRP) is a marker of systemic inflammation. hsCRP have been related to disease presence and clinical activity in systemic lupus erythematosus (SLE).AimTo understand the association between hsCRP and SLE disease manifestations and other associated immune parameters.Material & methodsOne hundred and ten SLE patients were studied and SLE disease activity was evaluated by SLE Disease Activity Index (SLEDAI). Among these 42.7% patients had lupus nephritis (LN). hsCRP and complement levels were detected by nephelometer.ResultsA total of 40/110 (36.4%) had elevated hsCRP levels. High CRP was associated with presence of infection (p < 0.001) Patients having bacterial, parasitic and viral infections had elevated levels of hsCRP. LN patients showed slightly higher hsCRP levels (29.3 ± 29.9 mg/L than non-LN group (27.3 ± 21.3 mg/L) but this difference was not significant. Patients with raised hsCRP levels showed a higher prevalence of low complement levels as compared to patients with normal CRP levels (p = 0.03).ConclusionElevated hsCRP levels were found to be associated with infections in SLE patients and low complement levels. Elevated hsCRP levels can be used as a marker of active infection in SLE patients.     

Neutrophil/lymphocyte and platelet/lymphocyte ratios are useful predictors comparable to serum IL6 for disease activity and damage in naive and relapsing patients with lupus nephritis

Neutrophil-lymphocyte ratio (NLR) and platelet-lymphocyte ratio (PLR) have become recently useful as predictive and prognostic tools in patients with various medical conditions.Aim of the workTo evaluate NLR and PLR in systemic lupus erythematosus (SLE) and their relation to disease clinical characteristics, nephritis, disease activity and damage.Patients and methodThe study involved 110 Egyptian SLE patients; 80 with lupus nephritis (naive and relapsing) and 30 without as well as 50 matched control. Patients were subjected to full clinical examination, SLE disease activity index (SLEDAI) scoring, and damage using Systemic Lupus International Collaborating Clinics Damage Index (SLICC-DI). Laboratory and immunology profiles included the complete blood count (CBC) with differential white blood cell counts and estimation of both NLR and PLR, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), protein/creatinine ratio, anti-nuclear antibody (ANA), anti-double stranded deoxyribonucleic acid (anti-dsDNA), serum complements and interleukin-6 (IL-6) levels.ResultBoth NLR and PLR were significantly higher in SLE patients (4.8 ± 1.4 and 281.7 ± 66.7 respectively) compared to controls (3.8 ± 2 and 138.2 ± 50.4). Both ratios were significantly elevated in patients with active lupus nephritis (at presentation or as a flare) and were comparable between naive and relapsing lupus nephritis patients. In lupus nephritis patients, NLR and PLR significantly correlated with proteinuria, ESR, hypocomplementemia, IL6, SLEDAI and SLICC-DI. The best NLR cut-off value to predict nephritis activity was 5.65, whereas the best PLR cut-off value was 316.5.ConclusionNLR and PLR appear to be potentially useful cheap parameters of activity, relapse and severity in SLE patients with nephritis.     

Emergence of Epstein-Barr virus-associated haemophagocytic syndrome upon treatment of systemic lupus erythematosus

A 32-year-old female patient with systemic lupus erythematosus was admitted to our hospital with fever and cytopenia, and diagnosed as haemophagocytic syndrome (HPS) by bone marrow aspiration study showing haemophagocytosis. Since the serologic activity of lupus was not increased at that time and HPS was refractory to the conventional therapies, an additional aetiological factor was suspected. Real-time PCR analysis identified reactivation of Epstein-Barr virus (EBV). A combination therapy targetting EBV-associated HPS, consisting of intravenous administration of cyclosporine A as well as immunoglobulin with a high titre of anti-EBV antibody, significantly suppressed EBV viraemia and led to the remission of HPS until the time of writing.