Diffuse lupus encephalopathy in a case of rhupus syndrome

“Rhupus syndrome” is a rare clinical condition, which is related to anti-CCP antibody, characterized by overlapping clinical and immunologic features of rheumatoid arthritis and systemic lupus erythematosus. Previous reports mentioned that patients have more RA-associated damage, and little organic damage associated with SLE. The severe organ damage, especially central nervous system involvement has been reported to be rare in patients with rhupus syndrome. Here, we report a very rare concurrence of RA, SLE and diffuse lupus encephalopathy in a Chinese woman.     

Anti-citrullinated peptide antibodies in lupus patients with or without deforming arthropathy

The objective was to study the association of antibodies against cyclic citrullinated peptides (anti-CCP) in patients with lupus articular damage. We studied 34 systemic lupus erythematosus patients (30 women) with (n = 14) or without (n = 20) deforming arthropathy. Anti-DNA and arthritis were mandatory inclusion criteria for both groups. As controls, 34 patients with rheumatoid arthritis and nine patients with rheumatoid arthritis and systemic lupus erythematosus (rhupus) were included. Anti-CCP and rheumatoid factor were determined by ELISA and nephelometry respectively. All patients had recent x-ray films of the hands that were evaluated according to Sharp's method. Systemic lupus erythematosus patients had a mean 6.50 +/- 0.86 (SD, range 5-8) American College of Rheumatology (ACR) criteria, rheumatoid arthritis patients met 5.38 +/- 0.60 (range 4-6) ACR criteria for rheumatoid arthritis and rhupus patients had 5.78 +/- 0.44 (range 5-6) criteria for rheumatoid arthritis and 5.11 +/- 0.78 (range 4-6) for systemic lupus erythematosus. Systemic lupus erythematosus patients, with or without deforming arthropathy, had normal serum anti-CCP concentrations. In contrast, rheumatoid arthritis and rhupus patients had 30- and 23-fold higher than normal amounts of anti-CCP (p < 0.001, both comparisons). Rheumatoid arthritis (97%) and rhupus (100%) patients were more frequently positive for anti-CCP than SLE patients with (7%) or without (5%) deforming arthropathy (p < 0.001, both comparisons). Patients with lupus deforming arthropathy were more frequently positive for rheumatoid factor (65%) than patients with non-deforming arthritis (15%) (p = 0.005). Patients with lupus deforming arthropathy had similar frequency of erosions and mean Sharp's score than rhupus patients. Anti-CCP antibodies do not associate with lupus arthropathy, whether deforming, non-deforming or erosive.     

Clinical and immunogenetic characterization of Mexican patients with 'rhupus'

The coexistence of systemic lupus erythematosus and rheumatoid arthritis (rhupus), is a rare clinical condition. To date, 50 cases of rhupus have been described worldwide; however, the lack of clinical criteria for this rheumatic condition has created confusion in the characterization of this disorder. Nevertheless, in this paper we describe a comprehensive clinical and serological characterization of a cohort of 22 Mexican patients with rhupus, supported by generic HLA-DR phenotyping. We found that rhupus patients have a special clinical behavior. In this setting, the signs and symptoms of rheumatoid arthritis prevail, little organic damage associated with systemic lupus erythematosus (SLE) exists and none of the cases present thrombosis or morbidity during pregnancy in spite of presenting a high frequency of anticardiolipin antibodies. We also found an increased frequency of HLA-DR1 and HLA-DR2 alleles compared to healthy ethnically matched controls, systemic lupus erythematosus and rheumatoid arthritis patients.     

Concurrence of rheumatoid arthritis and systemic lupus erythematosus: report of 11 cases.

The concurrence of rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) has been reported infrequently. Eleven patients are described here with both RA and SLE, in whom the diagnoses were separated by one to 24 years. Because of the difficulty in diagnosing RA occurring subsequent to SLE, only patients with classical RA as their initial diagnosis were included. Further difficulties arise because arthritis is common to both diseases and may be deforming in SLE, antinuclear antibodies (ANA) are not uncommon in RA, and rheumatoid factor (RF) may be seen in SLE. Nonetheless, judicious application of the American Rheumatism Association (ARA) criteria allows both diagnoses to be made in the individual patient. In our patients there was erosive arthritis in nine, rheumatoid nodules in five, and urinary abnormalities in 10. Serological evidence of RA and SLE with positive RF and ANA and raised DNA antibodies was universal, all patients had haematological evidence of SLE, and all but one decreased serum complement levels. These cases suggest that the concurrence of RA and SLE is not as rare as previously considered and may occur more often than expected by chance alone.     

Successful treatment using cyclosporine in a patient with rhupus complicated by aplastic anemia: a case report and review of the literature

Systemic lupus erythematosus (SLE) co-morbid with rheumatoid arthritis (RA) is known as 'Rhupus syndrome' and is estimated to be present in between 0.01 and 2% of SLE and RA patients. The occurrence of aplastic anaemia in a patient with rhupus is very rare and a treatment for this condition has not been reported. A 52-year-old woman presented complaining of nausea and dizziness during the preceding month. She had been treated for rheumatoid arthritis for 16 years. At the time of presentation, she had a malar rash, multiple arthritis, pancytopenia, pleural effusion, proteinuria, and positive anti-nuclear and anti-dsDNA antibodies. A kidney biopsy revealed ISN/RPS class IV-G (A) lupus nephritis. Bone marrow aspiration and biopsy showed aplastic anaemia with no evidence of viral infection. The patient was successfully treated using cyclosporine and prednisolone and she remained symptom-free at the one-and-a-half-year follow-up. To our knowledge, this is the first report of a successful treatment using cyclosporine in a patient with rhupus complicated by aplastic anaemia.     

Hemophagocytic syndrome in elderly patients with underlying autoimmune diseases

In patients with autoimmune disease-associated hemophagocytic syndrome (AAHS), the clinical features may differ from hemophagocytic syndrome (HPS) of other etiologies, and new criteria for AAHS have been proposed. Since bone marrow (BM) circumstances are changed according to aging, here we reviewed retrospectively our cases with AAHS in elderly patients, including two systemic lupus erythematosus (SLE), three Evans syndrome, one rheumatoid arthritis (RA), one Hashimoto thyroiditis, and one autoimmune pancreatitis. Although only two SLE patients were diagnosed as HPS by the classical criteria, the remaining patients except one RA met the criteria for AAHS. Seven patients except one SLE patient showed good response to therapy and demonstrated positive autoantibodies to blood cells, lower serum ferritin levels, and increased erythroblastic islands in the BM. We consider the diagnosis of AAHS should be carefully made when macrophages phagocytosing blood cells are observed in BM smear without hyperferritinemia in elderly patients with autoimmune diseases.     

Rhupus综合征的临床特点

目的探讨Rhupus综合征的临床特点。方法收集2000年1月至2013年3月北京协和医院40例Rhupus综合征患者（Rhupus组）资料,并按1：2抽取同期无合并类风湿关节炎（rheumatoid arhritis,RA）的系统性红斑狼疮（systemic lupus erythematosus,SLE）患者作对照组,比较两组临床特点。结果Rhupus综合征患者占同期SLE住院患者的0．93％（40／4301）;病程中位数7．0年;77．5％以RA起病。Rhupus组病程长于对照组,其影像学均有骨侵蚀表现。Rhupus组多发性关节炎、对称性关节炎、手关节肿痛、关节畸形、类风湿结节、红细胞沉降率、C反应蛋白升高的发生率及类风湿因子、抗环瓜氨酸肽抗体的阳性率均高于对照组,差异均有统计学意义（均P〈0．05）。Rhupus组蝶形红斑、肾脏及神经系统受累、补体C3下降的发生率及SLE疾病活动指数评分均低于对照组,差异均有统计学意义（均P〈0．05）。结论Rhupus综合征少见,多以RA起病,其RA相关表现严重但SLE相关表现较轻,并具有独特的临床表现。特异性抗体和影像学检查有助于诊断。     

Thyroid disease in systemic lupus erythematosus and rheumatoid arthritis

SIR, We determined the degree of overlap between autoimmunethyroid disease and two non-organ-specific autoimmune diseases,systemic lupus erythematosus (SLE) and rheumatoid arthritis(RA). Both SLE and RA are commonly encountered in our out-patientpractice and were chosen because of their clinical relevance. Sixty-nine SLE and 64 RA patients fulfilling the American RheumatismAssociation criteria for SLE [1] and RA [2] were selected forthis study. Patients from both groups were found     

Systemic lupus erythematosus evolving into rheumatoid arthritis

We describe 3 patients who presented with clinical and serological evidence of systemic lupus erythematosus (SLE) and 10 or more years later developed for the first time clinical and serological manifestations of rheumatoid arthritis (RA). Each patient now meets the American College of Rheumatology criteria for both SLE and RA.     

Lupus arthropathy: a case series of patients with rhupus

Among the clinical manifestations of systemic lupus erythematosus (SLE) is an arthropathy, which is usually nonerosive. In many cases the joint involvement is mild. A subset of patients have deforming, nonerosive Jaccouds arthropathy, and a minority have an arthropathy with clinical findings similar to rheumatoid arthritis (RA) that has been called rhupus. We report our series of eight patients (seven female, one male) with rhupus arthropathy. Patients were between the ages of 17 and 38 years (average: 30.3 years) at disease onset. All had deforming or Jaccouds arthropathy, and three had erosive disease. The arthritis was typically the first disease manifestation. Other symptoms of lupus including vasculitis and glomerulonephritis appeared after an average of 2.8 years. All had positive antinuclear antibody and rheumatoid factor. Rhupus arthritis is not a combination of RA and SLE, but should be regarded as a variant of the arthropathy of lupus.     

THE RELATIONSHIP OF ANTICARDIOLIPIN ANTIBODIES TO DISEASE ACTIVITY IN SYSTEMIC LUPUS ERYTHEMATOSUS

Sera from 124 blood donors, 60 rheumatoid arthritis (RA) and57 SLE patients were measured for anticardiolipin antibodiesby ELISA. Significantly raised IgG (aCLG) and IgM (aCLM) anticardiolipinantibody levels were found in RA and SLE (p<0.0005). However,in SLE, both aCLG and aCLM levels were significantly higherthan in RA (p<0.0025). We then conducted a transectionalstudy to evaluate aCL levels and disease activity in SLE. Therewas a good positive predictive value (70%) between aCL and overalldisease activity, but not for individual systems. A strong associationbetween aCL and renal involvement irrespective of activity wasalso found (80%). Nine SLE patients fulfilled both the clinicaland serological criteria for the antiphospholipid syndrome (APS)and a further 18 patients fulfilled the serological criteriafor APS. Results indicate that aCL levels are of value in predictingoverall disease activity in SLE and in monitoring those patientswho fulfil or partially fulfil the criteria for APS. KEY WORDS: Anticardiolipin antibodies, Systemic lupus erythematosus, Disease activity     

Anticyclic citrullinated peptide antibodies in patients with mixed connective tissue disease

Abstract

The clinical significance of anticyclic citrullinated peptide (CCP) antibodies in patients with mixed connective tissue disease (MCTD) was assessed. Altogether, 86 sera from MCTD patients, 96 from rheumatoid arthritis (RA) patients, 42 from systemic lupus erythematosus (SLE) patients, 23 from systemic sclerosis (SSc) patients, 21 from poymyositis/dermatomyositis (PM/DM) patients, and 17 from those with Sjögren’s syndrome (SjS) were tested for anti-CCP antibodies using an enzyme-lined immunosorbent assay. Among the 96 RA patients, anti-CCP antibodies were detected in 85%, with the frequency being significantly higher than in MCTD, SLE, SSc, PM/DM, and SjS patients (9%, 14%, 13%, 14%, and 18%, respectively; P < 0.001). Among eight MCTD patients who fulfilled the diagnostic criteria for RA, only 50% had anti-CCP antibodies, and the prevalence was significantly lower than for all RA patients (p < 0.01). All eight patients who fulfilled the criteria for RA had overlap of SLE and SSc, except one patient, whereas the four anti-CCP-positive patients who did not fulfill the criteria for RA had SjS without overlapping features of SLE and SSc; moreover, most of their antibody titers were low. These results suggested that anti-CCP antibodies are associated with RA in MCTD patients, but careful diagnosis of RA is required if patients with low titers of anti-CCP antibodies lack overlapping SLE and SSc.     

The risk of lymphoma development in autoimmune diseases: a meta-analysis

BACKGROUND: The risk of development of non-Hodgkin lymphoma (NHL) in autoimmune patients has been investigated in several cohort studies. These studies revealed inconclusive results. To shed some light on this controversy, we conducted a meta-analysis of all available cohort studies linking systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), and primary Sj?gren syndrome (pSS) to the risk of NHL development. METHODS: We searched the PubMed database (1974 to April 2005) for English-language cohort studies using the key words systemic lupus erythematosus, SLE, rheumatoid arthritis, RA, Sj?gren syndrome, or SS; non-Hodgkin lymphoma; and relative risk, RR, standardized incidence rate, or SIR. All cohort studies that used established diagnostic criteria for SLE, RA, and pSS; had histologic confirmation of NHL; and provided standardized incidence rates (SIRs) were included in the meta-analysis. RESULTS: The 20 studies chosen for the analysis included 6 for SLE, 9 for RA, and 5 for pSS. Overall, the meta-analysis suggested extreme heterogeneity among the studies (P < .01; I2 > 70%), high risk of NHL development for pSS (random effects SIR, 18.8; 95% confidence interval [CI], 9.5-37.3); moderate risk for SLE (random effects SIR, 7.4; 95% CI, 3.3-17.0); and lower risk for RA (random effects SIR, 3.9; 95% CI, 2.5-5.9). In RA, the random effects SIRs of NHL with conventional antirheumatic treatment, cytotoxic treatment, and treatment with a biological agent were 2.5 (95% CI, 0.7-9.0), 5.1 (95% CI, 0.9-28.6), and 11.5 (95% CI, 3.7-26.9), respectively. CONCLUSIONS: Rheumatic disease may present a potential risk factor for development of NHL. In this regard, we focused on the underlying pathophysiologic mechanisms related to lymphomagenesis in pSS, SLE, and RA, to justify the varying potential for and background of NHL development.     

Anti-CCP in systemic lupus erythematosus patients: a cross sectional study in Brazilian patients

Recently, it has been found that some lupus patients may have anti-cyclic citrullinated peptide antibodies (anti-CCP), although the clinical significance of such finding is not well established. Systemic lupus erythematosus (SLE) patients may have joint complaints that are very similar to those observed in rheumatoid arthritis (RA). In early stages of disease, this form of arthritis can be difficult to differentiate from RA, so it is not rare that some SLE patients are initially misdiagnosed to have this disease. This study aims to investigate the prevalence of anti-CCP in SLE patients from Southern Brazil and its association with clinical and serological profiles. One hundred nine SLE patients were studied for anti-CCP and compared with data of 156 RA patients and 100 healthy volunteers. Comparison of clinical and autoantibody profile of anti-CCP-positive and anti-CCP-negative SLE patients was done. All SLE patients positive of anti-CCP were submitted to hand and feet X-rays. Anti-CCP was positive in 15 of 109 SLE patients, and one of them had confirmed the diagnosis of rhupus. This prevalence was significantly higher than in healthy controls (p?=?0.0004) and lower than in RA patients (p?<?0.0001). No relationship could be found with clinical profile, including joint complaints. SLE patients with anti-CCP had higher prevalence of anti-Ro (p?=?0.02) and anti-La (p?=?0.004) autoantibodies, in comparison with those negative to anti-CCP. We found that 13.7 % of Brazilian patients with SLE have positive anti-CCP. Patients with anti-CCP showed higher prevalence of anti-Ro and anti-La autoantibodies than those negative for anti-CCP. Only a careful and prolonged follow-up will reveal the real clinical value of these markers in each patient individually.     

The levels of FDP, FDP-E and D-dimer in patients with rheumatoid arthritis]

We studied the relationship of FDP and D-dimer levels of rheumatoid arthritis (RA) patients with their activities of RA. We evaluated FDP/D-dimer levels of thirty-six RA patients. And we also evaluated FDP/D-dimer levels of 14 patients with systemic lupus erythematosus (SLE) and 12 patients with other rheumatic diseases as control. RA patients were divided into two groups according to their activities. Nineteen patients, who fulfilled at least three of four activities criteria, were classified as active group [RA (A) group], and other 17 patients were classified as not-active group [RA (B) group]. FDP and D-dimer levels of RA patients were higher than those of SLE or other patients group significantly (P < 0.01). Furthermore, in RA patients, high levels of FDP and D-dimer were observed in RA (A) group compared to RA (B) group. In some RA patients, decrease of FDP and D-dimer levels were observed according to their RA activities. FDP and D-dimer levels were not correlated with the level of rheumatoid factor (RF). These results show that the FDP and D-dimer levels were elevated according to RA activity in RA patients.     

Use of anti-citrullinated peptide and anti-RA33 antibodies in distinguishing erosive arthritis in patients with systemic lupus erythematosus and rheumatoid arthritis

OBJECTIVES: Systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) can both present with an erosive arthritis with the small joints of the hands affected. Therefore a serological marker would be useful to distinguish between these two diseases at onset. In this study anti-RA33 antibodies, which are found in patients with SLE and RA, and anti-citrullinated peptide antibodies (anti-CCP), which have recently been described as highly specific for RA, were assessed. METHODS: Two hundred and thirty one patients receiving long term follow up for SLE were evaluated for arthritis and classified as erosive and non-erosive disease. Sixty six patients were tested for anti-RA33 and anti-CCP antibodies. All the patients were tested for rheumatoid factor (RF) and HLA-DR4 status. RESULTS: Ten patients had erosive disease, six of whom were RF positive (60%), and six anti-RA33 positive (60%), whereas only two were anti-CCP positive (20%). Two hundred and twenty one patients had non-erosive disease, 40 of whom were RF positive (18%), 14 were anti-RA33 positive (6%), whereas only one patient was found to be anti-CCP positive (0.5%). CONCLUSION: The presence of anti-CCP antibodies may be useful in distinguishing RA from erosive SLE. Anti-RA33 antibodies and RF are unhelpful.     

Hand ultrasound: Comparative study between “no rhupus” lupus erythematosus and rheumatoid arthritis

Abstract

Objective. To compare hand US between systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) patients.

Methods. Hands (1st–5th metacarpophalangeal [MCP] and 1st–5th proximal interphalangeal [PIP] joints) and wrists (radiocarpal and distal radioulnar joints) of 62 “no rhupus” SLE and 60 RA patients were compared through US (linear probe, 6–18 MHz). The findings were compared to clinical, functional, serological outcomes, and disease activity indices.

Results. 2108 and 2040 joint recesses were evaluated in SLE and AR patients, respectively. Synovitis was found in 46.8% and 75% of wrists, 83.9% and 86.7% of MCPs and 58.1% and 70% of PIPs in the SLE and RA groups, respectively. More significant US findings were found in RA group. Greater values of synovitis (mm) in RA group were only found in the joint recesses of wrist (p < 0.001–0.002). In SLE group, US findings were associated with “puffy hands,” Health Assessment Questionnaire score and dynamometry. Twenty-two SLE patients (35.5%) had erosion in any of joints studied. SLE patient subgroup with US erosion was associated with hematological involvement and Jaccoud's arthropathy.

Conclusions. US of “no rhupus” SLE and RA patients is different, especially in wrists. In SLE patients the clinical variable most associated with US findings was “puffy hands.”     

Anticyclic citrullinated peptide antibodies in patients with mixed connective tissue disease

The clinical significance of anticyclic citrullinated peptide (CCP) antibodies in patients with mixed connective tissue disease (MCTD) was assessed. Altogether, 86 sera from MCTD patients, 96 from rheumatoid arthritis (RA) patients, 42 from systemic lupus erythematosus (SLE) patients, 23 from systemic sclerosis (SSc) patients, 21 from poymyositis/dermatomyositis (PM/DM) patients, and 17 from those with Sjögrens syndrome (SjS) were tested for anti-CCP antibodies using an enzyme-lined immunosorbent assay. Among the 96 RA patients, anti-CCP antibodies were detected in 85%, with the frequency being significantly higher than in MCTD, SLE, SSc, PM/DM, and SjS patients (9%, 14%, 13%, 14%, and 18%, respectively; P < 0.001). Among eight MCTD patients who fulfilled the diagnostic criteria for RA, only 50% had anti-CCP antibodies, and the prevalence was significantly lower than for all RA patients (p < 0.01). All eight patients who fulfilled the criteria for RA had overlap of SLE and SSc, except one patient, whereas the four anti-CCP-positive patients who did not fulfill the criteria for RA had SjS without overlapping features of SLE and SSc; moreover, most of their antibody titers were low. These results suggested that anti-CCP antibodies are associated with RA in MCTD patients, but careful diagnosis of RA is required if patients with low titers of anti-CCP antibodies lack overlapping SLE and SSc.     

The coexistence of rheumatoid arthritis and systemic lupus erythematosus

Summary A 29-year-old white female with longstanding classical rheumatoid arthritis (RA) developed clinical and serological manifestations of systemic lupus erythematosus (SLE) with prominent signs of diffuse proliferative lupus nephritis. She fulfilled the ARA criteria for the classification of SLE as well as the ARA criteria for classical RA. The concomitant presence of these two affections in the same patient is rare and the discriminating features suggest that this coexistence may be coincidental. With respect to treatment, our patient had good relief of symptoms by a combined administration of methylprednisolone pulses and cyclophosphamide.     

Monospecific but not polyreactive human hybridoma rheumatoid factors exhibit preferential binding specificities for IgG3 and IgG4

Summary Among 38 human hybridoma-derived monoclonal rheumatoid factors (RFs) generated from patients with either rheumatoid arthritis (RA) or systemic lupus erythematosus (SLE), two groups of RFs can be identified. Monospecific RFs were derived primarily from patients with RA and are characterized by a binding specificity for IgG3 and/or IgG4. Polyreactive RFs were derived largely from patients with SLE and show a broader pattern of reactivity to all four isotypes of IgG. Neither population of RFs was exclusive to either disease. The binding specificities identified appear to be different from the RFs isolated from patients with mixed cryoglobulinemia and may reflect a different antigen selection mechanism.