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Objective: To determine the prevalence of the most often tested autoantibodies in synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome., Methods: We identified 90 patients seen in our unit between June 2002 and June 2009, and diagnosed according to the proposed criteria for SAPHO syndrome. Demographic and clinical data were collected as well as immunological results, including antinuclear, antithyroid peroxydase (TPO), antithyroid globulin (Tg), antigastric parietal cell, antismooth muscle, antimitochondria, and anti-liver-kidney microsome (LKM) antibodies. Anticyclic citrullinated peptide (CCP) antibodies were analyzed in 69 patients, antibodies to soluble extractable nuclear antigens in 43, anti-double-stranded DNA (dsDNA) antibodies in 22 [depending on the type of fluorescence of antinuclear antibody (ANA)], and antiendomysium antibodies in 55., Results: Autoantibodies were found in 20 patients (22.2%): 14 patients (15.5%) had positive ANA (titer >/= 1/160); among them, 10 (11%) patients never took a lupus-inducing drug. Antithyroid antibodies (anti-TPO and/or anti-Tg antibodies) were found in only 3 patients (3.3%). Three patients (3.3%) were positive for antigastric parietal cell antibodies and 4 (4.4%) were weakly positive for antismooth muscle antibodies. Antimitochondria and LKM antibodies were negative in all 90 patients. Anti-CCP and anti-dsDNA antibodies were negative in the 69 and 22 patients tested, respectively. One out of 43 patients (2.3%) had anti-SSA antibodies. Antiendomysium antibodies were negative in the 55 patients tested., Conclusion: Our study indicates an increased prevalence of autoantibodies in SAPHO syndrome, with no specific profile. We failed to confirm the reports of an increased prevalence of antithyroid antibodies. These results tend to support a link between autoimmunity and SAPHO syndrome.

Objective: To describe characteristics and outcomes of vasculitides associated with malignancies., Methods: The requirement for inclusion in this retrospective, 10-year study was development of vasculitis in patients with a progressing malignancy. Malignancies secondary to immunosuppressants used to treat vasculitis were excluded. The main characteristics of vasculitides were analyzed and compared according to the type of malignancy., Results: Sixty patients were included (male/female sex ratio 2.53, mean age 62.4 years). Mean followup duration was 45.2 months. Vasculitides were cutaneous leukocytoclastic (45%), polyarteritis nodosa (36.7%), Wegener's granulomatosis (6.7%), microscopic polyangiitis (5%), and Henoch-Schönlein purpura (5%). Malignancies were distributed as follows: hematologic in 63.1%, myelodysplastic syndrome (MDS) in 32.3%, lymphoid in 29.2%, and solid tumor in 36.9%. Vasculitides were diagnosed concurrently with malignancy in 38% of the cases. Manifestations of vasculitides were fever (41.7%), cutaneous involvement (78.3%), arthralgias (46.7%), peripheral neuropathy (31.7%), renal involvement (23.3%; 11.7% glomerulonephritis, 11.7% microaneurysms, 6.7% renal insufficiency), and antineutrophil cytoplasmic antibody (20.4%). Vasculitis treatments were corticosteroids (78.3%) and immunosuppressant(s) (41.7%). Vasculitis was cured in 65% of patients, but 58.3% died, with 1 death secondary to vasculitis. Independent of subtype, patients with vasculitides associated with MDS more frequently had renal manifestations (P = 0.02) and steroid dependence (P = 0.04) and achieved complete remission less often (P = 0.04) than patients with vasculitides associated with other malignancies. Patients with vasculitides associated with a solid tumor more frequently had peripheral neurologic involvement (P = 0.05). Patients with vasculitides associated with lymphoid malignancy had less frequent arthralgias (P = 0.01) and renal involvement (P = 0.02)., Conclusion: Vasculitides occurring during malignancies present distinctive features according to the vasculitis subtype and nature of the malignancy.

This report is devoted to Philippe Gaucher's life and career. His MD thesis, presented while he was just an intern, is a quite unfrequent example of the description of a new disease, based on the anatomoclinical study of a single case. Since more than 100 years, its eponym is still used in the international literature.

The term "rheumatoid nodulosis" was coined by Ginsberg in 1975 to designate a rare and distinctive form of rheumatoid disease. Anecdotal case reports suggest a benign nondestructive course, although prolonged follow-up data are usually unavailable. We describe two cases of typical rheumatoid nodulosis with follow-ups exceeding 25 years. Onset occurred at 14 and 22 years of age, respectively. Both patients presented with palindromic rheumatism, positive tests for rheumatoid factors, negative tests for other biological markers, and normal radiographs. Multiple subcutaneous nodules developed after 4 and 6 years with palindromic flares, respectively. Functional impairments and disfigurement required several surgical procedures to remove nodules. Histology was typical for rheumatoid nodules. Neither patient responded to disease-modifying anti-rheumatic drugs (gold, antimalarials, and D-penicillamine). Treatment consisted of nonsteroidal anti-inflammatory drugs combined with prednisone as needed. After 20 and 22 years of follow-up, respectively, both patients had typical rheumatoid arthritis with deformities and radiological joint destruction. In conclusion, these two cases establish that rheumatoid nodulosis can occur as a presentation of rheumatoid arthritis with a potential for severe joint damage after many years.

Objective: Postoperative cardiac depression is attributed to ischemia and the effects of cardiopulmonary bypass (CPB). To evaluate the effect of CPB alone on postoperative left ventricular (LV) dysfunction, we used a conductance catheter to determine the LV performance by pressure-volume relation before and after CPB., Methods: Twenty-two 3-week-old piglets underwent sternotomy and normothermic CPB for one hour. A conductance catheter was placed in the LV cavity. End-systolic pressure-volume relationships (ESPVR), left ventricular end-diastolic pressure (LVEDP) and systemic vascular resistance (SVR) were measured under steady-state conditions before and 15 min after weaning from CPB in group A (n = 11). Group B included 11 piglets without CPB and served as control., Results: There was no difference between groups before initiating CPB. As an indication of depressed LV function, the ESPVR slope (mmHg/ml) was significantly lower in group A after weaning from CPB than in group B (1.69 +/- 0.5 vs. 1.86 +/- 0.55; p = 0.008). In group A, peak dP/dt (max index) (mmHg/s/m (2)) decreased markedly (1596 +/- 339 vs. 2045 +/- 206; p = 0.03), while LVEDP (mmHg) was significantly increased (11.7 +/- 2.6 vs. 5.4 +/- 0.9; p < 0.0001). In addition, SVR (index) (dyn x s x cm (-5)/m (2)) in group A was significantly lower (1407 +/- 176 vs. 1677 +/- 313; p < 0.0001) than in group B., Conclusion: Using the very sensitive conductance catheter technique in a pig model, we could show that CPB leads to a significant depression of LV contractility and elastance even without ischemic arrest.

Even if associating in single entity polyalgic symptoms remains a matter of discussion, fibromyalgia concept has gained a large acceptance among medical communities and international instances. Its physiopathological background is still in discussion. Its presentation mostly in middle aged women is a chronic, widespread painful syndrome, mostly appendicular of long duration and resistant to any treatment, associated with multiple tender points. Frequently associated are a poor sleep, local and general fatigue and mood disorders. Clinical, biological and radiological search for a definite condition remains negative, at least for primitive presentation. Concomitant forms are accepted by the American College of Rheumatology criteria, position which can be challenged. It seems more accurate to study such cases as differential diagnosis. Physiopathology involves anomalies of perception transmission and integration of painful signals at different levels of nervous system, either peripheral or central. Medical intervention requires first a good deal of empathy and a multidisciplinary approach combining some drugs, insufficient if used alone and different physical cognitive and comportemental measures. Fibromyalgia, which raises difficult social problems, is an evolving concept.

We report three cases of Nicolau's syndrome induced by intraarticular glucocorticoid injections. Nicolau's syndrome is defined as livedo-like dermatitis secondary to acute arterial thrombosis occurring immediately after intravascular injection of an insoluble drug substance. The cases described by Nicolau occurred in association with injections of oily bismuth suspensions. In 1970s and 1980s, cases occurred with delayed-action penicillin suspensions injected intramuscularly. Typically, the injection is followed immediately by excruciating pain in the buttock, sometimes with syncope. Cyanotic patches and a livedoid pattern develop. Rapid resolution of the pain and slower clearing of the skin changes occur in most patients. We report three typical cases with both severe pain and skin changes. In addition, two other patients had incomplete variants without skin abnormalities. Each of these five patients had received an injection in or about a joint of a glucocorticoid in a crystalline suspension. The pathophysiology of this syndrome probably involves acute vascular spasm related to penetration of microcrystals into a blood vessel.

Hip involvement is uncommon in familial Mediterranean fever (FMF) and can result either from a process specific to this disease or from a coexisting chronic inflammatory joint disease, usually suggestive of ankylosing spondylitis (AS). We report ten cases of FMF with radiologically-documented inflammatory hip disease. Five patients had AS and one had juvenile idiopathic arthritis. There were six men and four women, with a mean age of 34.4 years +/- 17.6 (range, 15-70 years). Onset of the inflammatory hip disease occurred after bouts of acute hip symptoms in one of the patients with isolated FMF and after protracted hip arthritis in another; the two other patients had no history of hip symptoms. The HLA-B27 antigen was looked for in two of the five patients with FMF and AS, with negative results in both; another patient in this subgroup had severe ulcerative colitis. Total hip replacement or replacement of the acetabulum was required in six patients, including two with isolated FMF. Chronic joint disease has been estimated to contribute fewer than 5% of the joint manifestations in FMF. In previous studies, the hips and knees were affected in 75% of patients with chronic joint disease related to FMF. The association of FMF and AS (usually without the HLA-B27 antigen) has been well documented, although the pathogenic mechanisms that link these two conditions remain unknown.

Synovial metastases from neoplasms are uncommon. We report two cases of knee monoarthritis due to joint metastasis. Joint fluid cytology established the diagnosis. In one patient, an epidermoid carcinoma of the ureter metastasized to the left knee. The other patient had chronic monoarthritis of the left knee unresponsive to conventional treatment and was found to have distal femoral metastases from a lung adenocarcinoma. Only 28 cases of synovial metastases from solid tumors have been reported in the literature. The knee is the most common target, the lung the most common site of the primary (12 cases), and adenocarcinoma the most common histological type (12 cases). Joint metastasis carries a poor prognosis with a mean survival of less than 5 months.

The objective of this study was to describe clinical, laboratory, and radiological features in systemic lupus erythematosus (SLE) patients with oculomotor palsy (OMP). Among a cohort of 750 SLE patients receiving follow-up at our unit between 1985 and 2000, all patients with OMP were studied. Clinical and laboratory data were recorded, as well as cerebral magnetic resonance imaging (MRI) findings where available. Immunological tests included tests for presence and specificity of antinuclear and antiphospholipid antibodies. Six patients had OMP, which occurred within the first 3 years in two patients and after more than 20 years in two patients. Four patients had focal neuropsychiatric SLE (NPSLE) manifestations and two had diffuse neurological involvement. The four patients with focal NPSLE were either anticardiolipin- or lupus anticoagulant-positive. Cerebral fluid was abnormal in two of four patients who had this test. In four of the six patients, cerebral MRI showed evidence of vasculopathy. The therapeutic regimens varied, although all six patients initially received high-dose corticosteroids. OMP resolved or improved significantly in all six patients. OMP in SLE occurs in a broad spectrum of clinical situations but is an infrequent manifestation of NPSLE. This case series, together with literature review data, suggests various pathophysiological mechanisms in patients with focal or diffuse neurological symptoms. In patients with focal NPSLE, a possible cause of OMP is microthrombosis associated with presence of antiphospholipid antibodies.

Pseudo-xanthoma elasticum (PXE) is an inherited disorder of the connective tissue characterized by cutaneous, ocular and vascular lesions. Coexisting PXE and rheumatoid arthritis (RA) is rare because three cases have only been described. We report three new cases of this association. Analysis of these six cases failed to show any particular biological or clinical features of rheumatoid arthritis associated PXE. The possible association of PXE and RA is discussed.

Objective: Patients with Still's disease show a prominent acute phase reaction. Our hypothesis is that under these circumstances the iron uptake of ferritin will not keep pace with its synthesis, and is therefore not a valid reflection of the iron status in these patients., Methods: Previously we developed a method to measure the iron content of ferritin; we investigated the usefulness of this method to establish the iron status of patients with anemia of inflammation., Results: In 9 patients with adult onset Still's disease (AOSD) we observed high ferritin concentrations and measured the iron saturation of ferritin. The mean value of saturation was 9.1%, while saturation in the healthy control group was 17.8%, a statistically significant difference (p < 0.005). Soluble transferrin receptor concentrations indicated a functional iron deficiency., Conclusion: We conclude that the acute phase ferritin in patients with AOSD contains less iron in comparison to ferritin in healthy controls. We suggest that soluble transferrin receptor is the method of choice in estimating the iron status of patients with an acute phase reaction.

Acquired C1 inhibitor (C1-INH) deficiency with consequent angioedema is a rare condition that may indicate an underlying lymphoproliferative disorder. The defect is caused by increased catabolism, which is often associated with the presence of serum autoantibodies to C1-INH. The present report describes 3 patients with systemic lupus erythematosus who developed typical symptoms of acquired angioedema, characterized by recurrent swelling of subcutaneous and mucous tissues. The 3 patients demonstrated a major classical pathway-mediated complement consumption, with very low levels of C3 antigen and decreased levels of C1-INH antigen. Neither antibodies to C1-INH nor associated lymphoproliferative disease was found. No patient had clinical and biologic signs of lupus activity at the time the angioedema occurred. All patients were treated with steroids and exhibited a good response, without relapse of angioedema and with normalization of plasma levels of C1-INH. In lupus patients who present with an angioedema syndrome, acquired or hereditary angioedema must be sought by examining parameters of the classical pathway and levels of C1-INH. Our observations suggest the existence of a new form of acquired C1-INH deficiency associated with a major classical pathway-mediated complement consumption and systemic autoimmunity.

Objective: To investigate the prevalence of serum anti-beta2-glycoprotein I (anti-beta2-GPI) antibodies and other antiphospholipid antibodies (aPL) in patients with systemic lupus erythematosus (SLE). To study their diagnostic value for the antiphospholipid syndrome (APS)., Methods: Anti-beta2-GPI and IgG anticardiolipin (aCL) were determined in sera from 102 consecutive patients with SLE using ELISA. Serum and plasma tests were also done for lupus anticoagulant (LAC), syphilis, and antibodies to dsDNA. Clinical and laboratory features of APS were observed., Results: Prevalences were 23.5% for aCL and 18.6% for anti-beta2-GPI. Correlations between the presence of aCL and anti-beta2-GPI and between their titers were statistically significant (p<0.0001). No associations were found between anti-beta2-GPI and disease activity criteria (SLEDAI, ECLAM, dsDNA). Anti-beta2-GPI were significantly associated with LAC (p = 0.005), APS (p = 0.005), and a high aCL titer (aCL > 5 SD; p< or =0.001). LAC was the best diagnostic criterion for APS., Conclusion: These data suggest that determination of anti-beta2-GPI in addition to aCL and LAC is unlikely to improve the diagnosis of APS in patients with SLE.

We determined the outcome of all pregnancies in SLE patients in our lupus cohort between 1991 and 1997. The women were advised that pregnancy was acceptable if the disease had been inactive for 6 months (SLEDAI < or = (4 at 2 serial examinations) and daily prednisone dose was below 10 mg. Patients were advised against pregnancy in case of active nephritis or neurolupus. In case of antiphospholipid antibodies, patients were treated with aspirin or heparin if previous fetal losses were documented. In case of anti-SSA ab, patients were monitored with ultrasound and given dexamethasone in case of atrioventricular block. Fifty-nine pregnancies were registered among 31 women: mean age at diagnosis of SLE was 25.3 +/- 3.7 years (range: 17-31); mean disease duration before pregnancy 4.4 +/- 3 years (0-14); mean ACR score 5.4 +/- 1.5 (4-9). Seven patients had ACL ab, 8 had anti-SSA ab. Pregnancies ended in: 13 early spontaneous abortions (9 not related to disease flare up, 4 related to SAPL); 7 elective abortions (patient decision in 5 cases, severe lupus flare up in 2); one in utero death; 19 full term births (> 38 weeks); and 19 preterm births. Cesarean section was performed in 11 cases (6 for fetal distress, dystocia and previous ceasarian; 5 for active lupus). Severe sepsis occurred in one premature infant who died at the age of 1 week. Intrauterine growth retardation was observed in 11 cases, mean APGAR score was 8.9 +/- 1.43. Child development was normal in all cases except one child with mild mental retardation. Severe lupus flare ups occurred in 6 cases, of which 4 were pregnancies in unadvised situations. Six mild flare ups were documented in the post partum. One fatal case of neonatal lupus with AVB was observed. In conclusion, in our experience, the live birth rate is similar to the general population and the risk of lupus flare up is low when the above mentioned criteria are applied. Systematic increase of steroid dose at pregnancy onset does not seem to be necessary. The high rate of prematurity remains a problem to be solved.

Objective: To evaluate familial history for evidence of antiphospholipid syndrome (APS) and autoimmune disease in rheumatology department patients with primary or secondary APS., Methods: We retrospectively studied patients with APS and systemic lupus erythematosus (SLE) managed at the Rheumatology Department of the Bichat University Hospital, Paris, between 1987 and 1996. Data were collected by chart review and by a 1997 standardized telephone interview., Results: We identified 108 patients with APS managed during the ten-year study period. According to classical classification criteria, 39 patients had primary antiphospholipid syndrome (PAPS) and 69 secondary antiphospholipid syndrome (SAPS). Family history data were obtained for 29 (74%) and 55 (80%) PAPS and SAPS patients. respectively (78% of the 108 patients). Twelve PAPS (41% and 19 SAPS (35%) patients had one or more relatives with evidence of at least one clinical feature of APS such as thrombosis or recurrent fetal loss; of these patients, seven in the PAPS (24%) and 11 in the SAPS (20%) group had two or more relatives with evidence of a clinical feature of APS. Three PAPS (10%) and 14 SAPS (25%) patients had one or more family members with an autoimmune disease., Conclusion: A positive family history for autoimmune disease and/or antiphospholipid syndrome is common in patients with PAPS or SAPS. This finding supports a genetic contribution to APS. The percentage of a positive family history for autoimmune disease tend to be higher in patients with SAPS than in those with PAPS.

Objective: To assess the long-term outcome of the synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome., Methods: All patients with the SAPHO syndrome seen at our unit between 1974 and 1997 were identified. Follow-up was prospective from 1992 to 1997. Data before 1992 were analyzed retrospectively. Clinical symptoms, treatments and biological data, including erythrocyte sedimentation rate and C-reactive protein, were recorded at least yearly. When available, radiological data, HLA B27 status, and findings from bone or skin biopsy specimens were recorded. For each drug, an efficacy index (El) was determined as follows: "0" for less than 30% improvement, as judged by the patient, on horizontal visual analog scale, "0.5" for partial efficacy, and "1" for more than 60% improvement., Results: We identified 120 patients with the SAPHO syndrome (50 men, 70 women), of whom 102 patients were followed-up prospectively after 1992; 3 of these 102 patients were lost to follow-up. Six patients also had Crohn's disease, and three had ulcerative colitis. Except for a significant association of palmoplantar pustulosis (PPP) or psoriasis vulgaris (PV) with axial osteitis (P = .007), the dermatologic presentation had no significant influence on rheumatic symptoms (ie, osteitis or arthritis, peripheral or axial). The HLA B27 antigen was not significantly associated with a particular pattern of distribution of arthritis or osteitis. No severe or disabling complications were noted. In the 47 patients followed-up for more than 5 years (mean, 9.5; range, 5 to 23), the mean number of osteitis or arthritis foci increased during follow-up from 1.57 to 1.91 and from 2.68 to 3.11, respectively. Nonsteroidal antiinflammatory drugs (NSAIDs) were prescribed in 113 of 120 (94%) patients, with a mean El of 0.67 (+/-0.39). Corticosteroid (CS) therapy was used in 23 patients, with a mean El of 0.67 (+/-0.42). Colchicine and sulfasalazine had a mean El of 0.36 (+/-0.44) and 0.16 (+/-0.30), in 28 and 18 patients, respectively. Methotrexate was given to 10 patients (6 with peripheral arthritis), with a mean El of 0.64 (+/-0.48). Doxycyclin (100 mg twice daily) was used in 20 patients, usually to treat osteitis, with a mean El of 0.26 (+/-0.42). Intraarticular injections of a CS or osmic acid were used in 27 patients, with a mean El of 0.77 (+/-0.35)., Conclusions: SAPHO syndrome is a relevant and stable entity, with a good long-term prognosis. NSAIDs and intraarticular injections (CS or osmic acid) most often alleviate rheumatic symptoms, but prednisone or methotrexate are sometimes necessary and appear globally helpful.

Objective: (1) To classify an intermediate group of patients (IntAPS) with antiphospholipid syndrome (APS) and lupus-like disease either as primary (PAPS) or secondary APS (SAPS) and to discuss 2 different classifications. (2) To compare patients of a division of rheumatology with either PAPS or SAPS., Methods: Patients with APS and patients with systemic lupus erythematosus (SLE) followed at the Department of Rheumatology, University Hospital Bichat, Paris, from 1987 to 1996 were analyzed. A chart review and a standardized telephone interview in 1997 completed the data of this study., Results: (1) We found a total of 108 patients with APS: 22 with PAPS, 69 with SAPS, and 17 with IntAPS. The group of IntAPS did not differ from PAPS in any clinical or laboratory signs with the exception of antibodies to dsDNA and to extractable nuclear antigen (ENA). Between IntAPS and SAPS, there were several significant differences in clinical signs of SLE (malar rash, discoid rash, arthralgia) and in laboratory values (leukocytopenia). (2) Comparison of PAPS and SAPS showed statistically significant differences for positive Coombs' test, leukocytopenia, lymphocytopenia, antinuclear antibodies, antibodies to dsDNA and to ENA, and hypocomplementemia., Conclusion: The mainstay of the diagnosis of APS is the clinical event of thrombosis or miscarriage in the presence of antiphospholipid antibodies. Less important are laboratory values, which may help to differentiate PAPS from SAPS in order to initiate adequate therapy (e.g., anticoagulation in the first and additional corticosteroids in the second). Patients with IntAPS are more likely to be integrated into the group of PAPS than in the group of SAPS; therefore, special exclusion criteria for PAPS are not appropriate.

Spinal tuberculosis (TB) accounts for about 2% of all cases of TB. New methods of diagnosis such as magnetic resonance imaging (MRI) or percutaneous needle biopsy have emerged. Two distinct patterns of spinal TB can be identified, the classic form, called spondylodiscitis (SPD) in this article, and an increasingly common atypical form characterized by spondylitis without disk involvement (SPwD). We conducted a retrospective study of patients with spinal TB managed in the area of Paris, France, between 1980 and 1994 with the goal of defining the characteristics of spinal TB and comparing SPD to SPwD. The 103 consecutive patients included in our study had TB confirmed by bacteriologic and/or histologic studies of specimens from spinal or paraspinal lesions (93 patients) or from extraspinal skeletal lesions (10 patients). Sixty-eight percent of patients were foreign-born subjects from developing countries. None of our patients was HIV-positive. SPD accounted for 48% of cases and SPwD for 52%. Patients with SPwD were younger and more likely to be foreign-born and to have multiple skeletal TB lesions. Neurologic manifestations were observed in 50% of patients, with no differences between the SPD and SPwD groups. Of the 44 patients investigated by MRI, 6 had normal plain radiographs; MRI was consistently positive and demonstrated epidural involvement in 77% of cases. Bacteriologic and histologic yields were similar for surgical biopsy (n = 16) and for percutaneous needle aspiration and/or biopsy (n = 77). Cultures for Mycobacterium tuberculosis were positive in 83% of patients, and no strains were resistant to rifampin. Median duration of antituberculous chemotherapy was 14 months. Surgical treatment was performed in 24% of patients. There were 2 TB-related deaths. Our data suggest that SPwD may now be the most common pattern of spinal TB in foreign-born subjects in industrialized countries. The reasons for this remain to be elucidated.

Objectives: Stress proteins (HSPs) are highly conserved immunodominant antigens found in various species. The purpose of this study was to assess the prevalence and prognostic significance of antibodies to HSC 70 kDa and HSP 90 kDa in three groups of patients with longstanding rheumatoid arthritis (RA) defined based on the severity of articular erosions., Methods: 73 patients with longstanding (> 6 years) RA whose HLA-DR genotype was known were divided in three groups according to Larsen's score and compared with 47 recent onset (<1 year) RA patients and with control groups composed of patients with other inflammatory diseases (n=137) or of normal controls (n=48). IgGs and IgMs to HSC 70 kDa and HSP 90 kDa were determined using an ELISA with purified bovine HSC 70kDa or HSP 90 kDa., Results: Concentrations of IgGs and IgMs to HSC 70 were significantly increased in 41.1% and 42.5% of longstanding RA patients, respectively. Corresponding figures for IgGs and IgMs to HSP 90 were 39.7% and 56%. IgMs to HSC 70 and HSP 90 were less frequent in recent onset RA (19% and 13% respectively). Among the groups with other inflammatory diseases, only the MCTD group exhibited high frequencies of IgGs to HSC 70 (80%) and HSP 90 (85%). DRB1*0401 positive RA patients (n=23) were not more likely to have increased concentrations of antibodies to HSC 70 kDa or HSP 90 kDa than other RA patients (DR4 positive but DRB1*0401 negative, or DR1 positive, n=31; or negative for both DR4 and DR1, n=14). IgGs to HSP 90 kDa were significantly more frequent (p<0.05) in longstanding RA patients whose Larsen's score was 4 or more (57%) than in those whose Larsen's score was 2 or 3 (39.4%) or less than 2 (16%). No associations were found between Larsen's score and IgGs or IgMs to HSC 70 kDa or IgMs to HSP 90 kDa. A significant correlation was demonstrated between IgGs to HSP 90 kDa and two other serological markers for RA, rheumatoid factor, and anti-Sa antibody; there were no correlations with antikeratin antibody, antiperinuclear factor, or anti-RA 33., Conclusion: IgGs to HSP 90 kDa are most common in longstanding RA patients with articular erosions, suggesting that they may be related to the articular prognosis in RA

We report a case of well-documented typhoid fever in a 30-year-old woman with inactive systemic lupus erythematosus with asymptomatic lupus anticoagulant and high-titer anticardiolipin antibody (aCL). Despite prompt eradication of the Salmonella typhi obtained with appropriate antibiotic therapy, multiple organ system dysfunction occurred. The central nervous system was involved, with ischemic infarcts in the occipital lobes. High-dose corticosteroid therapy failed to improve the neurologic manifestations, which responded to repeated plasmapheresis. A sharp fall in aCL and anti-beta2-glycoprotein I antibody titers was recorded before the start of plasmapheresis. At the same time, IgM and IgG antibodies to Salmonella group O:9 lipopolysaccharide became detectable; the IgM antibodies disappeared within 4 months, whereas the IgG antibodies remained detectable during the next 13 months. Despite treatment with high-dose corticosteroids and cyclophosphamide, rapidly progressive glomerulonephritis developed, leading to chronic renal failure. There is convincing evidence of a link between the S. typhi infection and the ensuing catastrophic syndrome in this patient, probably precipitated by bacterial antigens.

Objective: Adult Still's disease (ASD) is a rare chronic polyarthritis, usually treated with corticosteroid therapy. Because some patients become dependent on high dose prednisone or are refractory to that treatment, and because adverse events are frequent with corticosteroid, we evaluated the efficacy of low dose methotrexate (MTX) as a second-line drug., Methods: We retrospectively studied 26 patients with ASD treated with low dose MTX because their disease was either resistant to or dependent on corticosteroids., Results: The group included 13 women and 13 men, with a mean age of 32.6 years at onset of ASD. Mean disease duration at the beginning of MTX treatment was 59.9 mo (range 7 to 444). Evaluation took place at the maximum followup, which averaged 48.9 mo (range 8 to 136). The mean dose of MTX was 11.5+/-3.6 mg/week (range 7.5 to 17.5). Twenty-three patients responded to MTX; 18 had complete remission. No difference was seen between patients with or without extraarticular manifestations. Leukocyte and neutrophil counts and erythrocyte sedimentation rate were significantly reduced (p = 0.0001). Daily prednisone intake decreased by 69% (21.5 mg) (p = 0.0001). Eleven patients were able to stop taking corticosteroids. One patient with AA amyloidosis renal failure died of neutropenia: this was the only serious adverse event., Conclusion: MTX is an effective second-line treatment of ASD that does not respond to prednisone. It allows significant reduction of corticosteroid doses, which is beneficial to these patients, who have frequent and numerous corticosteroid related adverse events.

Optical neuromyelitis or Devic's syndrome is a very uncommon neurological manifestation of systemic lupus erythematosus. It is also associated with antiphospholipid antibodies, limited responsiveness to glucocorticoid treatment and a poor prognosis. We report the case of a female systemic lupus erythematosus patient who developed recurrent flares of optical neuritis and transverse myelitis. These flares consistently responded to glucocorticoid therapy. Despite the absence of overt anticardiolipin antibodies in the course of the disease, long-term anticoagulant therapy has been introduced with positive results. Treatments are usually of limited efficacy in Devic's syndrome. In our patient, however, aggressive glucocorticoid treatment resulted in prolonged survival.

Objectives: To evaluate in various groups of patients with chronic joint disease the sensitivity and specificity of anti-Sa antibody, recently described in sera from adults with rheumatoid arthritis (RA); and to determine the prognostic significance of anti-Sa in initial sera from patients with long standing RA with or without severe joint destruction., Methods: Serum samples from 489 patients were included. Of these, 154 were collected from patients with RA attending 2 rheumatology units. Controls were 335 patients with a variety of inflammatory joint diseases other than RA. IgG anti-Sa was detected using an immunoblotting method with purified Sa antigen from human placenta extracts. All patients were tested for the following antibodies: rheumatoid factor (RF), anti-keratin antibody (AKA), antiperinuclear factor (APF), and anti-RA 33. HLA class II DRB alleles were also determined., Results: Anti-Sa was detected in 39.8% of RA sera overall, 46.7% of sera from the long standing RA group, and 23.5% of sera from the recent onset RA group (p<0.01). In patients with long standing RA, statistically significant associations were found between the presence of anti-Sa and the following variables: RF (p<0.0001), AKA (p<0.0001), APF (p<0.00001), and HLA DRB1*04 or 01 (p<0.01). In contrast, no association was found with anti-RA33. Anti-Sa was positive in 11 adult controls (7.8%) and in 26 pediatric patients with juvenile chronic arthritis (22%). The specificity of anti-Sa for RA was 92.1% in adults with well characterized rheumatic diseases and 85.9% in adults and children together. Among patients with long standing RA, those with destructive disease were more likely to test positive for anti-Sa (66.6%) than those with nondestructive disease (22.2%) (p<0.0001). Comparisons with other serologic markers for RA demonstrated that anti-Sa was sensitive (68.4%) and was also the test with the highest specificity (79%), positive predictive value (75%), and negative predictive value (71%) for discriminating between patients who do and those that do not develop late severe radiographic damage., Conclusion: Immunoblot-detected IgG anti-Sa is a sensitive serologic marker for RA patients with severe radiographic damage.