Protein-losing enteropathy in systemic lupus erythematosus: analysis of the clinical features of fifteen patients.

OBJECTIVE: Protein-losing enteropathy (PLE) is an unusual manifestation of systemic lupus erythematosus (SLE), so its clinical manifestations and management are not well understood. In this study, we try to characterize the basic clinical features and the management of PLE by retrospectively analyzing the clinical data of 15 PLE patients and hope this study can improve the awareness of PLE in lupus patients with severe hypoalbuminemia that could not be explained by other causes. METHODS: The clinical data of 15 SLE patients with PLE hospitalized during November 2001 and April 2006 in Peking Union Medical College Hospital were retrospectively reviewed. The PLE was diagnosed by Tc-99m albumin scintigraphy (99mTc-HAS). The clinical characteristics, laboratory tests, response to treatment, and the outcome were studied. RESULTS: The mean age of PLE onset was 40.1 +/- 15.4 years (19-71 years). Twelve were female and 3 were male. 53.3% (8 of 15) patients had PLE as the initial presentation of SLE. All patients had different degree of peripheral pitting edema. Eleven had ascites, 9 had pleural effusion, and 7 had pericardial effusion. Only 6 patients presented with abdominal pain and diarrhea. Positive antinuclear antibodies (HEP-2) with a speckled pattern were found in all patients, but the antidsDNA antibody was negative in most cases. All patients had marked hypoalbuminemia, 80% had hypocomplementemia, 66.7% had hyperlipoproteinemia, and 40% had hypocalcemia. The liver function tests and the prothrombin time were in normal ranges. The 24-hours urine protein was less than 0.5 g in 60% (9 of 15) and more than 1.0 g in 20% (3 of 15) patients who were renal biopsied but only found to have very mild pathologic changes. Gastrointestinal endoscopy examination discovered generalized edema in the intestinal wall whereas the biopsy showed chronic inflammation only. Most cases had good response to corticosteroid and immunosuppressive therapies. The serum albumin level improved evidently in all patients after treatment and normal scintigraphic finding was found in 9 patients. CONCLUSION: PLE can be the initial presentation of SLE or can develop a very long time after the diagnosis of SLE. The prominent clinical presentations are caused by hypoalbuminemia. 99mTc-HAS is useful not only for the diagnosis of PLE but is also helpful for monitoring the efficacy of treatment. When a SLE patient presents with evident hypoalbuminemia without evidence of other causes, PLE should be considered. Early diagnosis and treatment may improve the prognosis.     

蛋白丢失性肠病61例临床分析

目的 通过对蛋白丢失性肠病临床资料的总结分析,提高对本病的认识.方法 对北京协和医院1997至2009年诊断的61例蛋白丢失性肠病进行分析总结.结果 男26例,女35例,年龄16～77(40±15)岁.水肿为首发症状51例;腹水为主要症状41例;合并双侧胸腔积液23例;腹痛16例,腹泻33例;所有患者均有显著的低蛋白血症.37例患者经核素99Tcm标记白蛋白显像证实存在肠道蛋白丢失,24例为临床诊断.原发病主要为系统性红斑狼疮(28例),先天性淋巴管扩张(12例).治疗上以原发病治疗为主.结论 蛋白丢失性肠病临床并非罕见,以严重的低蛋白血症和多浆膜腔积液为特征,核素99Tcm标记白蛋白显像是特异性的诊断方法之一,治疗上以原发病治疗为主,预后与原发病控制与否相关.

Abstract：

Objective To increase the understanding in protein-losing enteropathy (PLE).Methods Sixty-one PLE patients were enrolled in the study and the clinical characteristics, complicated disease, diagnosis and treatment were analyzed. Results The age of the patients was 16-77 (40±15)years, and the gender ratio was 35:26 (female: male). The main clinical manifestations were bilateral lower limb edema in 51 cases, ascites in 41 cases, bilateral pleural effusion in 23 cases, pericardial effusion in 13cases, abdominal pain in 16 cases and diarrhea in 33 cases. The prominent abnormality in laboratory examinations was hypoalbuminemia. The underlying diseases include systemic lupus erythematosus (SLE) in 28 cases, intestinal lymphangiectasia in 12 cases, hepatic cirrhosis in 5 cases, heart diseases in 5 cases,Crohn's disease in 3 cases, membranous nephropathy in 2 cases, Budd-Chiari syndrome in 1 case. Four cases happened after abdominal operation and 1 case after radiation therapy of gastric cardia cancer. Thirtyseven cases were diagnosed by 99Tcm-labelled human serum albumin scintigraphy and 24 cases were diagnosed clinically. Treatment was focused on underlying diseases. The clinical manifestations in 21 cases of SLE improved after SLE was controlled. In 2 cases of intestinal lymphangiectasia and one with Crohn's disease, the clinical manifestations improved after surgery. The other patients had no improvement.Conclusions PLE was not uncommon in clinical practice. Its predominant characteristics were severe hypoalbuminemia, edema and dropsy of serous cavity. PLE can complicate other diseases such as SLE,intestinal lymphangiectasia. Treatment should be focused on primary disease.     

Patients with systemic lupus erythematosus at risk for Pneumocystis carinii pneumonia.

We describe 6 cases of patients with systemic lupus erythematosus (SLE) who developed Pneumocystis carinii pneumonia. All were treated with high dose corticosteroids, and all developed the infection within 4 months of beginning new or revised cytotoxic therapy. All patients tested (5 of 6) were negative for human immunodeficiency virus (HIV). Those patients who developed Pneumocystis carinii pneumonia had more severe lymphocytopenia (median 595 vs 833/mm3) and received higher doses of corticosteroids (median prednisone dose = 43 vs 20 mg/day) than other patients with active SLE. A threshold lymphocyte count of 350/mm3 identified 4 of 6 cases but only 1 of 20 controls. Patients with SLE treated with high dose corticosteroids and cytotoxic drugs and with severe lymphocytopenia may be at increased risk for this opportunistic infection.     

Clinical characteristics of lupus myocarditis in Korea

Clinically important myocarditis is an unusual feature in systemic lupus erythematosus (SLE). We describe the clinical characteristics, management and outcomes of five SLE patients who developed severe left ventricular dysfunction. Four patients were female with mean age of 36.4 years. Three patients had both lupus myocarditis and lupus nephritis. Four patients had raised anti-dsDNA antibody titer and low complement level and two patients had positive IgG anticardiolipin antibody. Three patients were treated by high-dose corticosteroids, one patient by intravenous pulse methylprednisolone, and one patient by intravenous immunoglobulin and pulse cyclophosphamide with high dose corticosteroids. Left ventricular function improved markedly in four patients and all of them had no recurrence of lupus myocarditis up to follow-up of 33 months. However, one patient, who showed no improvement of left ventricular function, was expired due to sudden cardiac arrest. Lupus myocarditis should be treated by immunosuppressive therapy with high-dose corticosteroids and mostly the prognosis might be good with early treatment.     

Skin manifestations of systemic lupus erythematosus refractory to multiple treatment modalities: poor results with mycophenolate mofetil

OBJECTIVE: Skin disease can be one of the most refractory clinical manifestations of systemic lupus erythematosus (SLE). The standard therapy consists of sunscreens, topical corticosteroids and antimalarials. However in difficult cases a variety of other drugs have been tried. Here we describe our clinical experience with mycophenolate mofetil (MMF) in patients with cutaneous manifestations of SLE. METHODS: Seven patients with SLE and skin involvement (including acute cutaneous lupus, subacute cutaneous lupus, discoid lupus erythematosus, vasculitis, urticarial rash and chilblain lupus) who had received treatment with MMF were included. The clinical characteristics, serologicalfindings and response to treatment were recalledfrom retrospective review of the files. RESULTS: Our results showed no response in 5 patients, partial response in 1 patient and initial response but skin flare whilst on MMF in 1 patient. The median dose of MMF was 2 g (range 2-3 g). Adverse events on MMF were mild, mainly gastrointestinal and occurred in 5 patients. No patients discontinued MMF due to adverse events. CONCLUSIONS: MMF appears not to be particularly effective in the treatment of skin disease in SLE. It should be noted that our group of patients had previously failed to respond to a median of 4 (range 2-10) different drugs used to treat SLE skin disease. Thus, the patients in the study could be considered at the severe end of skin disease spectrum.     

Analysis of lupus activity in end-stage renal disease treated by hemodialysis

OBJECTIVE: The activity of systemic lupus erythematosus (SLE) has been reported to decrease in patients who have developed end-stage renal disease (ESRD). However, extrarenal symptoms attributable to the disease activity are noted, especially during the first year of dialysis. We studied the clinical course and evaluate the disease activity of SLE in patients with ESRD on hemodialysis for more than 6 months. SUBJECT AND METHODS: Fourteen patients with SLE who had been initiated on maintenance dialysis at our center between 1982 and 1999 were examined retrospectively. Their clinical details, organ system manifestations, serologic profiles and immunosuppressive treatment regimens were reviewed. Patients with and without postdialysis flaras of SLE were compared statistically. RESULTS: Five patients exhibited 6 SLE flares under treatment with corticosteroids. Two flares occurred within the first year of the initiation of dialysis, and in 1 patient, aggravation of the disease activity was noted 98 months after the initiation of dialysis. Polyarthritis was noted in 5 cases and fever in 4 cases. The serum complement levels decreased in all 6 cases with relapse of SLE activity. Compared with the other 9 patients who did not exhibit SLE relapse, no significant differences were found in 5 patients who did with respect to the demographic and serologic features at the initiation of dialysis. CONCLUSION: We conclude that the disease activity does not always burn out in patients of SLE who show progression to ESRD. SLE flares can sometimes occur even after one year of the initiation of dialysis. SLE patients on dialysis should be carefully followed up by clinical and serological monitoring, and treated by appropriate immunosuppressive therapy.     

Entéropathies exsudatives : à propos de cinq observations et revue de la littérature

Objective

Protein-losing enteropathy (PLE) is a rare entity with multiple etiologies. The diagnosis is confirmed by the elevation of faecal alpha-1-antitrypsin clearance. We report five cases of PLE and review the clinical characteristics and prognosis factors.

Methods

We retrospectively reviewed the medical report of 5 patients with PLE seen at the Department of internal Medicine between Hedi Cheker Hospital between 1996 and 2012.

Results

Five women with a mean age of 44.8 years (25–70 years) were studied. The initially suggestive clinical symtomatology was lower edema was in 3 cases, ascites in a patient while EE was discovered incidentally in another case. There were no gastrointestinal symptoms in all cases. Biologically, hypoproteinemia with hypoalbuminemia was found in all patients, hypogammaglobulinemia in 3 patients and lymphopenia in 3 cases. Hypocalcemia was present in one case, moreover, there was no digestive malabsorption in others cases. Renal function was normal without proteinuria in all cases. PLE was confirmed by the elevation of the clearance of alpha -1-antitrypsin in all patients. The investigations revealed systemic lupus erythematosus (SLE) in one case, a duodenal lymphangiectasia associated with non specific ulcerative ileitis in another. Celiac disease was highly likely in a patient, an iatrogenic origin was implicated in another (magnesium hydroxide). However, no cause was found in the fifth patient. All patients received a high-protein diet with specific treatment in three cases. The outcome was good in four patients with resolution of edema and correction of laboratory abnormalities.

Conclusion

PLE is a rare entity with digestive or nondigestive causes. Dietary measurement is generally indicated associated with the treatments of the more common causes.     

The heart in systemic lupus erythematosus and the changes induced in it by corticosteroid therapy: A study of 36 necropsy patients

The natural history of the cardiovascular manifestations of systemic lupus erythematosus (SLE) have been altered by corticosteroids which exert their own cardiovascular effects. This study describes clinical and necropsy observations in 36 corticosteroid-treated patients with SLE and compares them to necropsy observations in patients with SLE reported before the use of corticosteroid therapy. The 36 patients averaged 32 years of age, and 33 were women. Systemic hypertension was present in 25 (69 per cent) and left ventricular hypertrophy in 23 (64 per cent) patients. Hypertension was twice as common in the 19 patients who received this drug for more than 12 months (average 38 months) than in the 17 patients who received this drug for less than 12 months (average 6 months), and was almost five times more common among our patients than in patients with SLE in the presteroid era. Congestive cardiac failure occurred in 15 patients (43 per cent), eight times more frequent than that reported in noncorticosteroid-treated patients with SLE. Subepicardial and myocardial fat was increased in all 36 patients.Lupus carditis was similar in frequency but differed morphologically in our patients compared to those not treated with corticosteroids. Libman-Sacks-type endocardial lesions, present in 18 (50 per cent) of our patients, were smaller, fewer in number, univalvular rather than multivalvular, and mainly left-sided. Most verrucae were either partly or completely healed, and some were calcified. Pericarditis, present in 19 (53 per cent) patients, was predominantly of the fibrous type. Myocarditis was present in three patients, each of whom also had endocarditis and pericarditis. The lumen of at least one of the three major coronary arteries was narrowed more than 50 per cent by atherosclerotic plaques in 42 per cent of the 18 patients who received corticosteroids for more than 1 year, but in none of the 17 patients who received corticosteroids for less than 1 year. Four of the eight patients with narrowed coronary arteries had myocardial infarcts.Although vital to the management of SLE, corticosteroids have an over-all deleterious effect on the heart. Systemic hypertension and left ventricular hypertrophy appear or, when present, worsen; congestive cardiac failure increases; epicardial and myocardial fat increases, and coronary atherosclerosis appears to be accelerated.     

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目的探讨系统性红斑狼疮(SLE)合并急性胰腺炎的临床特点、病因、发病机制和治疗方法。方法回顾性分析1983-01～2003-03北京协和医院住院治疗的SLE合并急性胰腺炎患者15例。结果急性轻型胰腺炎12例,重型胰腺炎3例。所有患者均给予中到大剂量糖皮质激素治疗,病情痊愈5例,好转5例,死亡5例。结论(1)急性胰腺炎是SLE病情活动的表现;(2)胰腺血管病变是导致胰腺炎的主要致病机制;(3)糖皮质激素可能不是导致胰腺炎的病因;(4)中到大剂量糖皮质激素治疗是安全有效的。     

Autoimmune Myelofibrosis and Systemic Lupus Erythematosus in a Middle-Aged Male Presenting Only with Severe Anemia: A case report

Autoimmune myelofibrosis is a distinct clinicopathologic entity that occasionally occurs with autoimmune disorders such as systemic lupus erythematosus (SLE) and rheumatoid arthritis. Most cases of autoimmune myelofibrosis have been reported in female patients with a known history of SLE. We report a case of a middle-aged male patient with an unusual presentation of SLE and autoimmune myelofibrosis who presented only with severe anemia initially and was later diagnosed with SLE and autoimmune myelofibrosis. The patient''s condition improved dramatically after treatment with corticosteroids.SLE and autoimmune myelofibrosis is a rare but potentially devastating condition. Anemia maybe the only presenting symptom in addition to bone marrow fibrosis and careful clinical and laboratory assessment is imperative. Corticosteroids maybe useful and spare patients from bone marrow transplantation.     

Systemic lupus erythematosus with concurrent protein-losing enteropathy and primary sclerosing cholangitis: a unique association

We describe a 24-year old male patient with systemic lupus erythematosus (SLE) with the gastrointestinal manifestations of protein-losing enteropathy (PLE) and primary sclerosing cholangitis (PSC). He presented with periorbital, scrotal and lower limb oedema. PLE was diagnosed because of hypoalbuminaemia together with an elevation of alpha-1-antitrypsin stool clearance and absence of proteinuria. PSC was diagnosed on the basis of an elevated serum alkaline phosphatase and lymphocytic and fibrous cholangitis. His disease was also complicated by neuropsychiatric lupus and hypogonadism. All the manifestations of SLE resolved with systemic corticosteroids and pulsed cyclophosphamide treatment. This case report documents the unusual association of SLE with PLE and PSC, and this relationship suggests that autoimmunity underlie the pathogenesis of these conditions.     

Purtscher-like retinopathy associated with systemic lupus erythematosus treated with rituximab plus low-dose interleukin-2: A case report

Purtscher-like retinopathy, an occlusive microvasculopathy, is a rare and severe ophthalmic complication of systemic lupus erythematosus (SLE) characterized by a sudden loss of vision with retinal whitening, cotton wool spots and minimal intraretinal hemorrhage. Recovery in visual acuity is usually poor even with prompt treatment. This case showed a patient with SLE concurrent Purtscher-like retinopathy treated with rituximab and interleukin-2 (IL-2) with good prognosis. A 16-year-old female with a 2-year history of SLE was admitted because of unrelieved disease activity of SLE when treated with a high dose of corticosteroids and immunosuppressants and she further suffered from reduced visual acuity in both eyes. She was diagnosed with Purtscher-like retinopathy secondary to SLE after ocular examination. Rituximab and low-dose IL-2 for systemic treatment and intravitreal injection of anti-vascular endothelial growth factor antibody to right eye were given. The SLE disease was completely relieved with the sight recovering and no recurrence of Purtscher-like retinopathy was reported during 6-year follow-up. Purtscher-like retinopathy associated with SLE should be treated early and promptly. Rituximab should be considered in SLE patients with Purtscher-like retinopathy who have an incomplete response to initial immunosuppressive therapy and low-dose IL-2 may help induction of clinical remission.     

Inflammatory bowel disease and lupus: A systematic review of the literature

Coexistence of systemic lupus erythematosus (SLE) should be considered in patients with inflammatory bowel disease (IBD) and complex extraintestinal manifestations and the diagnosis of IBD could be established either before or after the diagnosis of SLE. Differential diagnosis of concomitant SLE and IBD is difficult and should always exclude infectious conditions, lupus-like reactions, visceral vasculitis and drug-induced lupus.The underlying mechanism by which 5-ASA/sulphasalazine induces SLE or lupus-like syndromes is not clear and high awareness for possible predictive factors is demanded for early prevention.In most cases the symptoms from drug-induced lupus have been reversible after the discontinuation of the drug and response to steroids is favorable. Treatment of patients co-diagnosed with SLE and IBD may include corticosteroids, immunosupressants and hydroxychloroquine.In severe lupus and IBD patients cyclophosphamide pulse may be of benefit while infliximab may be beneficiary in patients with lupus nephritis. However, the role TNFalpha plays in humans with SLE and IBD is controversial and data on the likely effects of blocking TNFalpha on anti-DNA autoantibody production is always of interest.     

Hyperplastic gastropathy as a presenting manifestation of systemic lupus erythematosus

A patient is described who had severe hyperplastic gastropathy as the presenting manifestation of systemic lupus erythematosus (SLE). Aggressive immunosuppressive therapy with systemic corticosteroids and immunoglobulins resulted in complete remission of lupus, and a prompt clinical and radiological regression of hyperplastic gastropathy. Hyperplastic gastropathy is an uncommon gastric illness, which is usually idiopathic but rarely is associated with Helicobacter pylori infection, cytomegalovirus infection or lymphocytic gastritis. Three previous case reports have noted a response of idiopathic hyperplastic gastropathy to systemic corticosteroid treatment, yet none of the presented patients had a systemic inflammatory disease. The presented case is the first in the medical literature in which hyperplastic gastropathy is directly linked to the development of clinical and laboratory manifestations of SLE. We suggest that hyperplastic gastropathy be added to the list of rare gastrointestinal manifestations of SLE, and that autoimmune disease be considered a possible cause of hyperplastic gastropathy. As such, any patient with symptomatic idiopathic hyperplastic gastropathy accompanied by other evidence of systemic inflammation should be considered for SLE evaluation and immunosuppressive treatment.     

Structural heterogeneity of faecal alpha 1 antitrypsin shown by immunoblot analysis in patients with Crohn's disease.

Faecal alpha 1 antitrypsin was determined in 34 patients with Crohn's disease and in 19 healthy subjects by immune nephelometry. A structural analysis of faecal alpha 1 antitrypsin was carried out using immunoblot analysis under non-reducing conditions. Native serum alpha 1 antitrypsin migrated with an apparent molecular weight of 45 kDa. Proteolytic alpha 1 antitrypsin fragments (5-42 kDa) were specifically immunostained in 13/19 and 22/34 stool samples from control subjects and from patients with Crohn's disease respectively. There was a weak correlation (r = 0.47; p less than 0.02) between the molecular weight of fragmented alpha 1 antitrypsin and the faecal concentration in both groups, indicating that alpha 1 antitrypsin inhibits its own proteolysis by intestinal proteases in a dose dependent way. The incidence of polymeric forms (greater than 45 kDa) was similar in patients (10/34) and control subjects (5/19). In only one case in each group was the native serum form of alpha 1 antitrypsin found in faeces. We conclude that faecal alpha 1 antitrypsin differs structurally from the native serum form. Immunochemical measurements, therefore, reflect rather than represent faecal concentrations of alpha 1 antitrypsin. The controversial results in published reports may be partly explained by these findings. The molecular heterogeneity of faecal alpha 1 antitrypsin is not specifically associated with Crohn's disease.     

Protein-Losing Enteropathy in Congestive Cardiac Failure: An Entity of Minor Clinical Significance

Protein-losing enteropathology (PLE) occurs rarely in congestive cardiac failure (CCF). Using fecal alpha 1-antitrypsin, an endogenous marker of enteric protein loss, we studied 25 patients in severe CCF to determine the frequency, degree, and clinical significance of PLE. Excessive enteric protein loss was found in only two patients, confirming the relative infrequency of this condition. In addition, enteric protein loss did not appear to correlate with the serum albumin level; neither did it influence treatment or prognosis of the CCF. It is concluded that PLE associated with CCF is of minor clinical significance.     

Hepatoid adenocarcinoma of the stomach: A report of three cases

Hepatoid adenocarcinoma of the stomach (HAS) is a rare form of gastric cancer that has unique clinicopathological features and an extremely poor prognosis. Here, we report on three patients with suspected gastric cancer who were referred to our hospital. Gastrointestinal fiberscopy on the three patients revealed two lesions in the antrum and a third lesion in the gastroesophageal junction. The alpha fetoprotein (AFP) serum levels were markedly elevated in all cases. At the time of diagnosis, two cases were advanced stages with lymph nodes and/or liver metastases. Two patients underwent exploratory laparotomy. A total gastrectomy was performed on the operable lesion, and an expanded gastrectomy was completed in the case with hepatic metastases. Histopathological analysis revealed that the tumors displayed two pathological changes:hepatoid-like foci and adenocarcinomatous. Furthermore, the tumor cells were immunohistochemically positive for AFP, alpha-1 antitrypsin, and alpha-1 antichymotrypsin. All three patients received chemotherapy. The follow-up duration ranged from 8-36 mo. Our experience and previous published studies have suggested that HAS is an aggressive type of adenocarcinoma. However, radical surgery and chemotherapy may positively impact clinical outcomes.     

Decline in FEV1 in patients with PiZ alpha-1-antitrypsin deficiency: the Australian experience

OBJECTIVE: Alpha-1-antitrypsin (alpha1antitrypsin) deficiency is a rare hereditary disorder which characteristically presents with emphysema at an early age. The aim of the present study was to determine whether the rate of decline of lung function in alpha1antitrypsin-deficient subjects in Australia was similar to that found elsewhere. METHODOLOGY: Patients registered with the Australian Alpha-1-Antitrypsin Replacement Program were studied. All patients (n = 50) had a serum alpha1antitrypsin concentration of < 0.3 g/L and had had spirometry measured over at least 2 years. They were compared with a group of normal volunteers (hospital staff, n = 107) with normal alpha1antitrypsin levels and phenotypes and with no clinical history of lung disease. All had spirometry measured for periods ranging from 2 to 6 years. The rate of decline of forced expiratory volume in 1 s (FEV1) for each subject was calculated by least squares linear regression using FEV1 against the time from entry into the study. RESULTS: The group mean (+/- SD) rate of decline in FEV1 was significantly greater (P < 0.01) in the alpha1antitrypsin-deficient patients (88 +/- 71 mL/year) than for the normal controls (-15 +/- 48 mL/year). There was no difference in decline in FEV1 when the data was analysed for gender and for index versus non-index cases. CONCLUSION: The results confirm previous reports of an accelerated rate of decline of FEV1 in patients with alpha1antitrypsin deficiency. Our results indicate that the rate of decline of lung function in alpha1antitrypsin deficient subjects in Australia is similar to that found in reported series from elsewhere.     

Treatment of isolated severe immune hemolytic anaemia associated with systemic lupus erythematosus: 26 cases

The aim of this study was to evaluate the response to treatment and the long-term outcome in a cohort of patients in whom severe autoimmune hemolytic anaemia (AHA) was the leading manifestation of systemic lupus erythematosus (SLE). Twenty-six women with severe isolated AHA were included. Corticosteroids were used as the initial treatment for all patients in our study. An initial response was obtained in all but one patient (96%). The overall recurrence rate was three per 100 person-years, with an expected recurrence-free proportion of 73% with a 180 months median follow-up. Seven patients (27%) experienced a relapse of AHA. We found a higher proportion of pleuritis in relapsing patients. Only three patients experienced multiple relapses despite splenectomy and several immunosuppressants. Steroid-sparing effect of hydroxychloroquine and azathioprine could not be assessed because most of the patients received these treatments for other reasons than AHA. Intravenous immunoglobulins induced transient response in three cases. Splenectomy was efficient to definitively control AHA in one patient but two patients quickly experienced relapses while one patient did not benefit. Five patients received immunosuppressants that induced only transient responses. Rituximab was long-term efficient in one case. In conclusion, severe AHA is a serious complication of SLE that warrants appropriate management. On the basis of our experience, the ideal treatment of isolated AHA should be oral corticosteroids in first-line treatment. Our study does not support an important role for splenectomy. Patients refractory to conventional therapy should be treated either with few toxic immunosuppressive drugs, danazol or rituximab.     

Thrombotic thrombocytopenic purpura in systemic lupus erythematosus: disease activity and the use of cytotoxic drugs

Thrombotic thrombocytopenic purpura (TTP) is a rare and occasionally fatal haematologic disorder that can coexist with systemic lupus erythematosus (SLE) and other autoimmune diseases. We identified all cases of TTP seen in our institution over a 3 year period using a computerized database. We found that SLE activity (measured by the SLE Disease Activity Index) and TTP activity ran a parallel course in three patients with coexistent SLE and TTP. TTP in these three patients, although refractory to plasmapheresis, responded to cytotoxic therapy. These observations further support an autoimmune contribution to the pathogenesis of some cases of TTP. A literature review revealed that mortality in SLE patients with more severe, refractory TTP treated with plasmapheresis and cytotoxics, may not be higher than in patients responding to plasmapheresis alone (who are likely to have milder disease). These data suggest that cytotoxics may have a role in treatment of patients with active SLE and TTP refractory to plasmapheresis.