Force-velocity-length relations in hypertrophic cardiomyopathy: Evidence of normal or depressed myocardial contractility

To assess myocardial contractility in patients with hypertrophic Cardiomyopathy (HC), force-velocity-length relations were analyzed during left ventricular (LV) ejection. LV pressure, volume and wall stress data in 15 patients with HC were analyzed and compared with values from 32 normal subjects. Patients with HC had a greater LV mass than did normal subjects (272 versus 96 g/m², p < 0.001), elevated LV end-diastolic pressure (17.5 versus 9.8 mm Hg, p < 0.01) and impaired LV relaxation compared with those of normal subjects. Patients with HC also had a greater ejection fraction (84 ± 7 versus 74 ± 8%, p < 0.01) and mean velocity of shortening than did normal subjects. However, in patients with HC, end-systolic stress (60 ± 29 versus 187 ± 61 kdyne/cm², p < 0.001) was significantly lower. End-systolic volume and stress data were linearly related in normal subjects (r = 0.88), and values from patients with HC fell either within the lowest part of the 95% confidence interval of this normal relation or outside it in the zone of depressed contractility (11 patients with HC). In addition, the slopes of the relations between end-systolic wall stress and ejection fraction or mean velocity of shortening were abnormal in patients with HC; the slope of the stress-volume trajectory during late ejection was also depressed in 12 patients with HC (average slope 2.6 versus 5.5 kdyne/cm⁵/m², p < 0.001). Thus, there is no evidence of a hypercontractile state in patients with HC; their high values of ejection phase indexes may be explained by a reduction in myocardial afterload.     

Cardiac transplantation in patients with hypertrophic cardiomyopathy

Cardiac transplantation is a treatment option for patients with hypertrophic cardiomyopathy (HC) who developed refractory heart failure and/or intractable arrhythmia. However, the pretransplant characteristics and post-transplant prognosis for patients with nondilated idiopathic HC has not yet fully elucidated. Therefore, we retrospectively reviewed 813 consecutive transplant recipients undergoing cardiac transplantation at Columbia University Medical Center from 1999 to 2010 and compared the clinical course of 41 patients with idiopathic HC with that of 373 patients with ischemic heart disease and 398 patients with other heart disease. The patients with HC were younger than those with ischemic heart disease (47.8 ± 14.0 vs 57.1 ± 9.4 years; p <0.0001). The proportion of patients undergoing ventricular assist devise surgery for bridge-to-transplant was lower in patients with HC than in those with ischemic heart disease or other heart disease (14.6% vs 31.1% vs 35.7%, all p <0.01). The post-transplant survival of those with HC was better than that for those with ischemic heart disease (90.1% vs 85.8% and 83.9% vs 67.1% at 1 and 5 years, respectively; p = 0.0359), although it was not significantly different from those with other heart disease. Proportional hazards analysis revealed that the subjects with HC had reduced post-transplant mortality (hazard ratio 0.4760, 95% confidential interval 0.1889 to 0.9762; p = 0.042) on univariate, but not multivariate, analysis. Most patients with HC had nondilated left ventricles (left ventricular end-diastolic dimension ≤ 55 mm; n = 27), and post-transplant survival did not differ from that for those with dilated left ventricles (left ventricular end-diastolic dimension >55 mm; n = 14). In conclusion, the post-transplant survival of those with HC did not differ from those of the subjects who underwent transplant for other non-HC indications.     

Selective neuropathy and preserved vascular responses in the diabetic Charcot foot

Summary Charcot arthropathy is a disabling complication of diabetic neuropathy. It is however, unclear why it occurs in only a small number of neuropathic patients. We have studied 12 diabetic patients (10 insulin-dependent) with an acute Charcot arthropathy, and compared their neuropathy and vascular responsiveness with 12 diabetic patients (10 insulin-dependent) with recurrent neuropathic foot ulceration, 12 diabetic control subjects (9 insulin-dependent) and 10 normal non-diabetic subjects. The Charcot arthropathy patients demonstrated a preservation of warm perception, 6 (5.5) °C, but complete loss of peripheral cold perception, 10 (0) °C, p<0.001 (median (interquartile range)). This contrasted with the ulcerated neuropathy patients, who had equally severe impairment of both warm and cold sensory thresholds, 10 (0.5) °C vs 10 (1) °C, respectively, the diabetic control subjects who were able to detect a 2 (1.3) °C warm stimulus and 3 (3.5) °C cold stimulus and the normal subjects, whose warm threshold was 2 (1) °C and cold was 2 (1) °C. Light touch perception at the foot was preserved in the Charcot patients 4 (4) g vs 100 (50) g, p<0.0002, in the ulcerated neuropathy patients. Vibration perception at the great toe and cardiovascular autonomic function tests (heart rate variability, Valsalva ratio and postural systolic blood pressure fall) were abnormal in both the Charcot patients and ulcerated neuropathy group, with no differences seen between the two groups. Peak skin blood flow at the great toe in response to local heating was preserved in the Charcot arthropathy patients, 63.36 (28.72) flow units when compared to the diabetic and normal subjects, 62.72 (47) flow units and 76.3 (33.92) flow units, respectively and much greater than in the ulcerated neuropathy patients 28.94 (37.39) flow units, p<0.0002. The diabetic patients developing Charcot arthropathy thus have a neuropathy and vascular responsiveness which distinguishes them from diabetic subjects developing neuropathic ulceration. This may be important in the pathogenesis of the Charcot foot.     

Patients with systemic lupus erythematosus and Jaccoud's arthropathy: a clinical subset with an increased C reactive protein response?

Jaccoud's arthropathy is a chronic deforming synovitis occurring in a subset of patients with systemic lupus erythematosus (SLE). To evaluate whether patients with SLE and Jaccoud's arthropathy behave differently in their acute phase reaction from patients with SLE without Jaccoud's arthropathy, a prospective study was carried out on 72 consecutive patients with SLE. Patients were assessed for Jaccoud's arthropathy according to a protocol, disease activity was scored, and laboratory tests, including tests for C reactive protein, were performed. Seven patients were classified as having definite Jaccoud's arthropathy. In those patients the serum concentrations of C reactive protein were higher than in those without Jaccoud's arthropathy, whereas disease activity scores and treatment were comparable. Additionally, patients with Jaccoud's arthropathy had a longer disease duration and a longer history of arthritis than those without, whereas IgM rheumatoid factor was observed more often. The increased concentration of C reactive protein in patients with Jaccoud's arthropathy is compatible with a persistent inflammatory reaction. Serial testing of C reactive protein in combination with other laboratory parameters may be a guideline for treatment in patients with SLE and Jaccoud's arthropathy.     

Pyrophosphate arthropathy: a study of metabolic associations and laboratory data.

105 consecutive patients with pyrophosphate arthropathy have been studied. Disease associations and metabolic abnormalities were compared with those of an age and sex matched group of 105 acute medical admissions, and with 48 patients presenting with uncomplicated osteoarthritis. Patients with pyrophosphate arthropathy had a higher incidence of hypothyroidism (10, hyperparathyroidism 92), and chronic steroid therapy (15) than did those in the comparative groups. Laboratory data showed abnormalities suggesting chronic inflammatory disease in many patients, but no other unexpected metabolic findings. Metabolic abnormalities were uncommon in pyrophosphate arthropathy, and extensive metabolic screening of patients was unrewarding.     

Clinico-pathological correlation of non specific inflammation in bowel histology with joint manifestation in a tertiary center in South India

The aim of our study was to determine whether the pattern of arthropathy in patients with suspected enteropathic arthritis bore any relation to their gut histology and specifically to chronic nonspecific gut inflammation. Records of 39 patients with suspected enteropathic arthritis from rheumatology clinic between January 2006 and December 2008 who had undergone ileocolonoscopic biopsy were analyzed retrospectively. Patients were grouped into 3 categories, namely those with normal bowel histology, those with mild nonspecific chronic changes, and those with histology suggestive of inflammatory bowel disease. Patients with nonspecific chronic gut inflammation had higher occurrence of axial involvement with or without peripheral articular involvement as compared to those with normal gut histology (8/9 vs. 10/21, P = 0.049), and this pattern was similar to that in patients with IBD. Wrist joint involvement was more common in patients with normal bowel histology (12/21) than the other two groups (P = 0.003). All groups had fared well on follow up while taking treatment with sulphasalazine and methotrexate.     

Jaccoud's arthropathy of the hands in overlap syndrome

Summary The objective of this work was to evaluate the frequency and features of Jaccoud's syndrome of the hands in patients with overlap syndrome. Twenty-three patients with overlap syndrome were prospectively evaluated by means of a complete physical examination, serological assessment, and hand X-rays. The presence of Jaccoud's syndrome was defined by Kahn's criteria. Patients with or without Jaccoud's arthropathy were compared in their clinical, serological and radiological features.Five out of 23 patients (21.7%) presented Jaccoud's syndrome. Clinical and serological features of patients with or without the syndrome proved similar except for alignment abnormalities, which by definition were more frequent in the group with Jaccoud. A significantly greater number of patients meeting diagnostic criteria for SLE were observed in the Jaccound group (80% versus 21%; p=0.03). There was no other disease or associated condition such as chronic renal failure or high-dose steroid treatment concomitant with the development of Jaccoud's syndrome.It may be concluded that in patients with overlap syndrome, Jaccoud's arthropathy seems to be more frequent in patients who meet criteria for SLE.     

Hemochromatosis and femoral head aseptic osteonecrosis: a nonfortuitous association?

Chondrocalcinosis, chronic pseudo-osteoarthritis arthropathy, and osteoporosis are classic osteoarticular complications of hemochromatosis (HC). Within HC, femoral head aseptic osteonecrosis (FHAO) is not notified in textbooks. We describe 3 cases of FHAO occurring in this setting in 3 patients homozygous for the C282Y mutation on HFE gene who had no other risk factors for FHAO. FHAO was diagnosed 9 years before (Case 1), concomitantly with (Case 3), or 9 years after HC (Case 2). In one case, FHAO occurred although phlebotomies were regularly carried out. There are scarce data available in the literature on HC and FHAO. Our observations suggest FHAO may be an indicator for HC, and iron balance should be determined before considering FHAO as idiopathic. Thus phlebotomy may not be protective against the occurrence of FHAO. Studies are needed to determine the prevalence of HC in consecutive patients with FHAO.     

Echocardiographic analysis of ventricular septal dynamics in hypertrophic cardiomyopathy and other diseases

It has been suggested that the adynamic or hypokinetic appearance of the ventricular septum is a unique echocardiographic feature of hypertrophic cardiomyopathy (HC). To determine how characteristic of HC the adynamic septum is, 70 patients with this disease, and 31 with other cardiac diseases that produce left ventricular (LV) hypertrophy and pressure overload (aortic valvular stenosis or systemic hypertension), and 25 subjects with normal hearts were studied by echocardiography. On M-mode echocardiography, 53 of 70 patients (75%) with HC had an abnormally low value for percent systolic thickening of the septum associated with either reduced or normal septal excursion; however, 17 patients (25%) showed normal septal dynamics. Twenty of 31 patients (64%) with other cardiac diseases that produce pressure overload showed normal septal thickening and excursion, while 11 (36%) had reduced systolic thickening associated with either diminished or normal excursion. Greatly reduced values for percent systolic thickening of the septum were present both in patients with HC (13 +/- 1%) and in patients with other cardiac diseases (21 +/- 2%). However, differences in systolic septal thickening between the 2 groups were largely a manifestation of the greater absolute diastolic septal thickness in patients with HC. When values for percent systolic thickening were normalized for diastolic septal thickness, or when systolic thickening was compared in only patients with similar diastolic septal thicknesses, differences in septal thickening between patients with HC and those patients with other cardiac diseases were not significant.(ABSTRACT TRUNCATED AT 250 WORDS)     

Contribution of anti-cyclic citrullinated peptide antibody and rheumatoid factor to the diagnosis of arthropathy in haemochromatosis

OBJECTIVE: To investigate the prevalence of antibodies to cyclic citrullinated peptide (anti-CCP) and rheumatoid factor in patients with hereditary haemochromatosis (HHC) and to evaluate their diagnostic reliability in distinguishing HHC-associated arthropathy from rheumatoid arthritis. METHODS: Anti-CCP antibodies and rheumatoid factor levels were determined by ELISA in sera from 87 patients with HHC homozygous for the C282Y mutation of the HFE gene, 31 patients with rheumatoid arthritis and 162 healthy controls. RESULTS: Of the 87 patients with HHC, 32 (36.8%) had joint involvement. Anti-CCP antibodies were detected in only 1 patient (1.1%) with HHC, who had no joint disease, and in (1.2%) healthy controls. In total, 18 (58.1%) patients with rheumatoid arthritis displayed anti-CCP reactivity (p<0.001). Rheumatoid factor was detected in 10 (11.5%) patients with HHC compared with 7 (4.3%) healthy control subjects (p = 0.03) and 21 of 31 (65.6%) patients with rheumatoid arthritis. CONCLUSIONS: Testing for anti-CCP antibodies discriminates HHC arthropathy from rheumatoid arthritis, as these patients were consistently anti-CCP negative. Thus, HHC arthropathy should be considered in the differential diagnosis of CCP-negative arthritis.     

Diphosphonate bone scans in patients with polyarthralgias

Early detection of inflammatory arthropathy is notoriously difficult with standard radiographic techniques. We therefore assessed bone turnover with technetium Tc 99m medronate in 16 patients with persistent polyarthralgias who had no clinical synovitis, normal radiographs, and nondiagnostic results from laboratory evaluations. Abnormal scans were found in 11 of 16; five were unremarkable. Scan abnormality corresponded with symptomatic joints (11 of 11 patients). These 11 patients had normal test results for rheumatoid factor, antinuclear antibody, and HLA-B27. Patients with abnormal scans were treated with nonsteroidal antiinflammatory drugs or analgesics (11 of 11), hydroxychloroquine sulfate (four), or gold salts (one), with improvement (nine of 11); patients with normal scans (five of five) were treated successfully with nonsteroidal antiinflammatory drugs or analgesics and reassurance. One patient with a normal scan developed sarcoidosis; one, hypermobility syndrome; and one, a viral syndrome. Two patients had no diagnosis. Abnormal technetium Tc 99m medronate scans in patients with previously undiagnosed polyarthralgias suggested inflammatory arthropathy and influenced management decisions with favorable therapeutic outcomes.     

Decreased Total and Corrected Antioxidant Capacity in Patients with Inflammatory Bowel Disease

Oxidative stress and depletion of antioxidants may play a key role in the pathogenesis of inflammatory bowel disease (IBD)-related intestinal damage. A new automated assay for the determination of blood total antioxidant capacity (TAC), based on the crocin bleaching method, has been used for the measurement of TAC and corrected TAC (cTAC) in patients with ulcerative colitis (UC) and Crohn's disease (CD) in comparison to healthy controls (HC). Ninety-four patients with UC, 97 patients with CD, and 72 HC were included in this study. Serum TAC was measured in all patients and controls on an Olympus AU-600 chemistry analyzer using a TAC kit. cTAC was calculated from TAC after subtraction of the interactions due to endogenous uric acid, bilirubin and albumin. Mean serum TAC as well as cTAC levels were significantly lower in both UC and CD patients compared with HC (P < 0.0001). Patients with active UC had no different TAC and cTAC compared to those with inactive disease. Patients with active CD had significantly lower mean TAC compared to those with inactive disease but cTAC was not different between the two phases of disease activity. Patients with proctitis had significantly higher TAC and cTAC compared to patients with left-sided colitis and total colitis. In CD patients no association between disease localization and these markers was found. TAC and cTAC are significantly reduced in IBD patients compared with controls irrespective of disease activity. The decreased antioxidant defenses may be a primary phenomenon severely compromising the mucosa and therefore increase susceptibility to oxidative tissue damage.     

Doppler echocardiographic study of the frequency and severity of aortic regurgitation in hypertrophic cardiomyopathy

Aortic regurgitation (AR) has been reported sporadically in hypertrophic cardiomyopathy (HC) but neither its frequency nor severity has been determined. Thirty-one consecutive patients with HC were evaluated by Doppler echocardiography over a 2-year period. Twenty-nine had echocardiographically normal aortic cusps and participated in the study; 2 had calcified aortic valves and were excluded. AR of grade I to grade II severity was demonstrated in 9 of 29 (31%) patients. Patients were divided into 2 groups: group 1 (n = 9) with AR and group 2 (n = 20) without AR. Group 1 patients were significantly older than group 2 patients (73 +/- 7 vs 60 +/- 17 years, p less than 0.05) and had larger end-diastolic (4.5 +/- 0.5 vs 4.0 +/- 0.7 cm, p less than 0.01) and end-systolic (2.7 +/- 0.4 vs 2.3 +/- 0.4, p less than 0.02) left ventricular dimensions. Left ventricular wall thickness, degree of asymmetric septal hypertrophy and left ventricular fractional shortening were similar in the 2 groups. Mitral regurgitation was more common in group 1 (100% vs 35%, p less than 0.005), although there were no differences in left atrial size between the 2 groups. The HC patients were compared with a control group of 23 normal subjects of similar age. There was no mitral regurgitation or AR in the normal subjects. Thus, nearly one-third of patients with HC had mild AR by Doppler. The AR most probably results from high-velocity systolic blood flow causing microscopic damage to the valve cusps.     

Serum human cartilage glycoprotein 39 as a marker of arthritis associated with inflammatory bowel disease

BACKGROUND: Common blood markers of arthritis are difficult to interpret in arthritis associated with inflammatory bowel disease (IBD) owing to the coexistence of two inflammatory events. No specific serological disease marker is available for IBD. OBJECTIVE: To determine a value of serum human cartilage glycoprotein 39 (HC gp39) as a marker of arthritis associated with IBD. METHODS: Serum levels of HC gp39 and ultrasensitive C reactive protein (CRP) were determined in 121 patients with IBD: 58 without arthritis (IBD-nonA) and 63 with arthritis (IBD-A), and in 20 healthy controls. IBD was classified as active (aIBD) and non-active (naIBD), and patients with IBD-A were classified as type I, II, and III arthritis by clinical activity indices. RESULTS: HC gp39 was higher in IBD-A than in IBD-nonA (p<0.001) and controls (p<0.01), while no difference was found between IBD-nonA and controls. CRP was increased in both IBD-A and IBD-nonA compared with the controls (p<0.01 and <0.05, respectively) and in aIBD-nonA v naIBD-nonA (p<0.05), but no difference in CRP was found between aIBD-A and naIBD-A. Finally, a correlation was found between the number of affected joints (NAJ) and HC gp39 (r = 0.6, p<0.001). DISCUSSION: Increased serum levels of HC gp39, which were higher in IBD-A than in IBD-nonA, suggest that this substance might be a marker of arthropathy in IBD. HC gp39, because of its relationship with NAJ in IBD-A, may also be proposed as a disease activity marker in arthritis associated with IBD.     

Thromboelastography Parameters in Patients with Acute on Chronic Liver Failure

Introduction and aim. Patients with acute on chronic liver failure (ACLF) have abnormal conventional coagulation tests- platelet count and international normalized ratio (INR). Thromboelastography (TEG) is a rapid, point-of-care assay, more comprehensive than platelet count and INR as it assesses for platelet adequacy(number and function), coagulation factors and clot retraction. The aim of the study was to evaluate the TEG parameters in patients with ACLF, chronic liver disease having acute decompensation (AD) and healthy subjects (HC).Material and methods. TEG parameters were assessed in patients with ACLF and AD within 24 h of admission. Consecutive patients were included in the study over 12 months. Healthy subjects were recruited as controls.Results. 179 patients were included- 68 ACLF, 53 AD and 58 HC. The mean values of INR in ACLF, AD and HC groups were 2.9 ± 1.4, 1.6 ± 0.4 and 1.1 ± 0.2; P < 0.001. Among TEG parameters - maximum amplitude (MA) was low in ACLF and AD patients as compared with HC (53.8 ± 15, 58.3 ± 13.9 mm and 67.2 ± 12.1 mm, respectively; P < 0.001). Lysis at 30 min (LY30) was high in ACLF patients, as compared to AD and HC (8.6 ± 14.1%, 5.0 ± 9.5% and 4.9 ± 9.8%, respectively; P = 0.060). There were no differences in r time, k time, and alpha angle between groups; normal in >90% patients. There was no difference in TEG parameters between different ACLF grades, whereas CCTs were more deranged with increasing grades of ACLF.Conclusion. Despite abnormal conventional coagulation tests, TEG parameters in ACLF patients are essentially normal, except reduced maximum amplitude. Future studies are needed to explore the utility of TEG in clinical management of ACLF patients.     

Decreased coronary vasodilatory capacity in hypertrophic cardiomyopathy determined by split-dose thallium-dipyridamole myocardial scintigraphy

Split-dose thallium-dipyridamole myocardial scintigraphy was performed in patients with nonobstructive hypertrophic cardiomyopathy (HC) who had angiographically normal coronary arteries. The dipyridamole-induced increases in thallium-201 uptake, calculated to evaluate coronary vasodilatory capacity, were significantly lower in 30 patients with HC than in 13 control subjects (177 +/- 58 vs 281 +/- 46%) and the reductions were observed in both the septal and lateral segments. The reductions of the septal segment in HC patients were significantly greater than those in 10 hypertensive patients with comparable degrees of septal hypertrophy. Of patients with HC, 16 had increases in thallium uptake well below the normal range. Compared with those having normal increases, these patients had significantly lower exercise duration (11 vs 15 minutes), with 33% having ST depression develop at a workload less than or equal to 80 watts. These data indicate that approximately one-half of patients with HC have impaired coronary vasodilatory capacity that could be an important pathophysiologic abnormality of HC resulting in the development of myocardial ischemia and the impairment of cardiac performance during exercise.     

Cartilage proteoglycans in synovial fluid and serum in patients with inflammatory joint disease. Relation to systemic treatment

Proteoglycan concentrations in knee joint synovial fluid and in serum from patients with various inflammatory arthritides were studied using an enzyme-linked immunosorbent assay. Patients with reactive arthritis, calcium pyrophosphate arthropathy, and juvenile rheumatoid arthritis (age less than or equal to 20 years) had the highest synovial fluid concentrations. These values differed significantly (P less than 0.001) from those in patients with rheumatoid arthritis, psoriatic arthropathy, and chronic HLA-B27-associated arthropathy. Rheumatoid arthritis patients receiving low-dose prednisolone treatment had higher synovial fluid (P = 0.006) and serum (P less than 0.001) proteoglycan concentrations than did those taking nonsteroidal antiinflammatory drugs or slow-acting antirheumatic drugs. Serum proteoglycan concentrations were near the detection limits, and did not correlate with levels found in paired samples of knee joint synovial fluid. Patients with calcium pyrophosphate arthropathy had the highest mean serum level of proteoglycan. This assay of proteoglycan antigens is a useful tool in the study of proteoglycan metabolism in patients with joint disease. With its use, differences between disease groups and effects of therapy can be distinguished.     

Reticuloendothelial function in coeliac disease and ulcerative colitis.

Patients with ulcerative colitis and coeliac disease who had been shown by impaired clearance of heat damaged red cells to have diminished splenic phagocytosis, were examined for evidence of more generalised reticuloendothelial malfunction by measuring their circulatory clearance of micro-aggregated albumin. Although in animals micro-aggregated albumin is largely removed by Kupffer cells, we found impaired clearance in otherwise normal subjects who had previously had surgical splenectomy. In patients with hyposplenism because of bowel disease there was no additional impairment of micro-aggregated albumin clearance, indicating that their hyposplenism is an isolated phenomenon and not part of a generalised reticuloendothelial atrophy. Patients with coeliac disease and normal splenic function had increased reticuloendothelial catabolic activity; this was not present in patients with coeliac disease and abnormal splenic function.     

Crohn disease arthropathy: antigens in synovial fluid share epitopes with strains of two species of viridans streptococci

BACKGROUND: There is evidence to suggest that Crohn disease is caused by an immunologic response to an unknown intestinal luminal antigen, probably of bacterial origin. The reported demonstration of yersinia antigen in the synovial fluid of patients with yersinosis therefore prompted a search for bacterial antigens in the synovial fluid of patients with Crohn arthropathy. METHODS: Antisera were raised in rabbits to synovial fluids obtained from seven patients with Crohn arthropathy and from seven 'control' subjects with other forms of arthropathy. These antisera were used to probe sonicates of the bacteria cultured from the gastric juice of patients with gastric Crohn disease. RESULTS: The antisera made from the Crohn synovial fluids, but none of those made from the controls, reacted uniquely with antigens in sonicates of strains of two species of viridans streptococci (Streptococcus parasanguis and an atypical S. oralis) isolated from four of the five patients with gastric Crohn disease. CONCLUSIONS: These findings suggest that the arthropathy of Crohn disease and, possibly, the intestinal disease itself may involve an immunologically mediated inflammatory response to these antigens.     

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.