Tertiary Rheumatology Centre Research Articles

Lag time between disease onset and first visit to a tertiary rheumatology centre by patients with rheumatoid arthritis

IntroductionRheumatoid arthritis is a chronic disabling disease. Presently there is great emphasis on early diagnosis and treatment to prevent damage and disability. There is however, lack of awareness of urgency to treat amongst lay persons and doctors resulting in treatment delays. We have studied the delay in referral, disease activity and damage at first presentation to our clinic. PatientsThis is a retrospective chart analysis of 357, consecutively seen patients between January 2002 and December 2005. At first visit demographic details, details of prior treatment, disease activity, disability index, clinical and radiological damage were noted in a predesigned form. ResultsThere were 313 females, 44 males. The median age was 50 years and the disease duration was 88 months. The median delay in seeing a rheumatologist was 24 months (1–372). 278 (78%) had received DMARD with 163 (58%) patients taking regular DMARD treatment. Methotrexate monotherapy was the most common DMARD used: 160 (74%) were on <10 mg per week. 143 (40%) had received or were on steroids, 260 (73%) had taken alternate form of treatment. 257 (72%) had evidence of radiological damage. HAQ (325 patients) was <1 in 159 (49%), 1–2 in 134 (41%), and >2 in 32 (10%). DAS-28 (3) (350 patients) was, <2.4 in 14 (4%), 2.4–3.2 in 21 (6%), 3.2–5.1 in 112 (32%), >5.1 in 203 (58%). ConclusionsThere is a long delay in referral to a rheumatologist. Even on treatment, disease is not well controlled. Majority of the patients have high disease activity with significant disability and evidence of joint damage. Special efforts are needed to ensure early institution of therapy with the aim to achieve maximum disease control as early as possible.

FRI0351 Outcome of 267 JIA patients from a tertiary level indian pediatric rheumatology centre

BackgroundWe run a pediatric rheumatology service at a tertiary level hospital. We follow a policy of “treat to target”.We systematically studied the JIA patients who followed up in the unit...

FRI0361 The use of leflunomide in a paediatric rheumatology clinic

BackgroundMethotrexate (MTX) is the anchor drug used in the treatment of juvenile idiopathic arthritis (JIA). Leflunomide (LFD) has comparable efficacy to MTX in adults with rheumatoid arthritis, and there is...

AB0658 Predictors of access to care in JSLE - evidence from the UK JSLE cohort study

BackgroundDelays in access to specialist care for children with paediatric rheumatic disease are likely to be associated with adverse outcomes1,2. Factors influencing access to care for Juvenile-onset Systemic Lupus Erythematosus...

THU0321 110 patients with enthesitis related arthritis: A demographic and clinical study from a tertiary level pediatric rheumatology centre in india

BackgroundThe ERA subcategory forms a major proportion of children with JIA in India (36%) (1) whereas western literature suggests ERA in JIA patients to be 6-19% (2,3). In these patients,...

AB1251 Neurological manifestations associated with anti-tumor necrosis factor alpha treatment. A longitudinal study

BackgroundAnti-tumor necrosis factor-α (TNFα) agents have been successfully used for the treatment of rheumatic diseases including rheumatoid arthritis (RA), psoriatic arthritis (PsA) and ankylosing spondylitis (AS). Yet, several reports have...

SAT0574 Medical Costs of Patients with Rheumatoid Arthritis and Association with Global Disease Activity in Turkey

BackgroundRheumatoid arthritis is a chronic and debilitating disease which decrease quality of life and productivity.ObjectivesRheumatoid arthritis (RA) has significant economic implications for the individual patient as well as society as...

AB0869 Subclinical atherosclerosis in greek patients with spondyloarthropathy

BackgroundChronic arthritis, particularly rheumatoid arthritis (RA), has been associated with accelerated atherosclerosis. Ultrasound-assessed carotid intima-media thickness (IMT) has been suggested as a surrogate marker of atherosclerosis and the risk for...

OP0319 Genetically Determined Mannose-Binding Lectin Deficiency Increases Risk of Nephritis in Danish Patients with Systemic Lupus Erythematosus

BackgroundMannose-binding lectin (MBL) is involved in the processing of apoptotic debris; deficiencies in this mechanism are strongly associated with autoimmunity, including systemic lupus erythematosus (SLE) with nephritis, i.e. lupus nephritis...

THU0538 Derivation and Validation of a Diagnostic Algorithm to Identify Patients with Rheumatoid Arthritis in Administrative Health Database

BackgroundThe use of administrative health databases (AHD) is a promising strategy to study the impact of rheumatoid arthritis (RA) at population level. Previous studies, using different methodologies, have shown moderate...

Remission occurs still only in minority of rheumatoid arthritis while a tight control is achievable in a routine clinical practice – A cross section study

The current recommended target is to achieve remission, if not at least low disease activity (LDA) in management of rheumatoid arthritis (RA). We analysed the incidence of patients achieving LDA or in remission in a real time clinical situation in a tertiary referral rheumatology centre, at a given point of time. Materials and methodsWe reviewed 480 patients who fulfilled classification criteria for RA and who were assessed for 28 Tender Joint Count (TJC), Swollen Joint Count (SJC), ESR and CRP. Their DAS28 (3) CRP and DAS28 (3) ESR score were calculated and were classified into LDA, remission, low, moderate and high disease activity based on the DAS28 (3) score. Results5.9% and 21.9% were in remission and 12% and 10% were in LDA based on DAS28 (3) ESR and DAS28 (3) CRP respectively. There was no significant influence of duration of illness, treatment and age in attaining both LDA and remission in our population. Conclusion5.9% and 21.9% of RA were in remission based on DAS28 (3) ESR and DAS28 (3) CRP respectively and 12% and 10% of RA patients were in LDA based on DAS28 (3) ESR and DAS28 (3) CRP respectively at the point of our study. DAS (3) CRP overestimate remission compared to DAS28 (3) ESR.

A rare association of not so rare symptoms

A 13 year old boy presented to us with history of vomiting, dizziness, red eyes and lethargy. Within the next few days he was found to have unilateral sensori neural deafness. He was also found to have anterior uveitis which as the disease progressed turned out to be interstitial keratitis. He was extensively investigated for infectious, inflammatory, rheumatological and autoimmune diseases, but all came back as negative. He was started on steroids after discussion with a tertiary rheumatology centre, to which he responded well. He was steroid dependant, so was commenced on methotrexate. His deafness completely resolved and his vision was better whilst on methotrexate. He was diagnosed to have Cogan syndrome based on the association of audio vestibular symptoms and interstitial keratitis and after excluding other conditions. Cogan syndrome is a rare disorder characterised by association of audio vestibular and ocular symptoms particularly interstitial keratitis. There is no diagnostic test as a result of which the diagnosis may be delayed. The disease may also be associated with non-specific systemic symptoms. The ocular symptoms typically may present as interstitial keratitis but in some cases it may present as uveitis or iritis and eventually progress to interstitial keratitis. Steroids and methotrexate are used in the treatment of the disease. If the condition is not diagnosed early and treated promptly it can cause permanent damage such as hearing loss and vision loss. Key message: Cogan syndrome is characterised by audio vestibular and ocular symptoms. One has to think of Cogan syndrome as a possibility when there is such an association. As there is no diagnostic test, there may be delay in diagnosing the disease. But early detection and treatment may prevent the permanent damage such as hearing loss or loss of vision.

P75 Clinical profile of Benign joint hypermobility syndrome from a tertiary care rheumatology centre in North India

P75 Clinical profile of Benign joint hypermobility syndrome from a tertiary care rheumatology centre in North India

P17 Profile of rheumatoid arthritis at first visit to a tertiary rheumatology centre

Non-documented palpitations, or phantom tachycardias, are palpitations deemed to be of unknown origin after evaluation with conventional diagnostic tools, such as 12-lead electrocardiogram and Holter recordings. Our aim was to determine the diagnostic value of an electrophysiologic study (EPS) and its role in the management of patients presenting with non-documented palpitations.We performed EPS in 78 consecutive patients with repeatable, poorly tolerated symptoms of paroxysmal, non-documented tachycardia, the absence of structural heart disease and at least one 24-h Holter recording. The duration and frequency of palpitations was registered in each patient.Long-lasting palpitations (>1 hour) were present in 15.4% of patients. Half of patients reported symptoms less often than once per week. Only 13/78 patients (16.6%) had normal EPS findings, while dual pathways at the AV node ± echo beats were identified in another 13 patients without inducible tachycardia. At least one tachycardia event was induced in 52 patients (66.6%). AVNRT was provoked in 32 patients (41.2%). Ablation was performed in 14/52 patients with inducible tachycardia (26.9%). Slow pathway ablation was also performed in three patients with dual AV pathways and atrial echo-beats but without provoked tachycardia. Follow-up data were available in 52 patients, and 84.6% had fewer or no clinical recurrences.EPS is safe and of enhanced diagnostic value in patients with unexplained palpitations because only 1/6 had negative results. EPS also provided an explanation about the mechanism of arrhythmia and successfully guided the management of these patients, as well as enhanced improvement in the quality of life.

Effectiveness of dexamethasone iontophoresis for temporomandibular joint involvement in juvenile idiopathic arthritis

Temporomandibular joint (TMJ) involvement is common in juvenile idiopathic arthritis (JIA). Dexamethasone iontophoresis (DIP) uses low-grade electric currents for transdermal dexamethasone delivery into deeper anatomic structures. The purpose of this study was to assess the safety and effectiveness of DIP for the treatment of TMJ involvement in JIA, and to delineate variables that are associated with improvement after DIP. Medical records of all JIA patients who underwent DIP for TMJ involvement at a larger tertiary pediatric rheumatology center from 1997-2011 were reviewed. DIP was performed using a standard protocol. The effectiveness of DIP was assessed by comparing the maximal interincisor opening (MIO(TMJ) ) and the maximal lateral excursion (MLE(TMJ) ) before and after treatment. Twenty-eight patients (ages 2-21 years) who received an average of 8 DIP treatment sessions per involved TMJ were included in the analysis. Statistically significant improvement in the median MIO(TMJ) (P < 0.0001) was observed in 68%. The median MLE(TMJ) (P = 0.03) improved in 69%, and resolution of TMJ pain occurred in 73% of the patients who had TMJ pain at baseline. Side effects of DIP were transient site erythema (86%), skin blister (4%), and metallic taste (4%). Improvement in TMJ range of motion from DIP is associated with lower MIO(TMJ) , lower MLE(TMJ) , and absence of TMJ crepitus at baseline. In this pilot study, DIP appeared to be an effective and safe initial treatment of TMJ involvement in JIA, especially among patients with decreased TMJ measurements. Prospective controlled studies are needed.

Risk factors for development of uveitis differ between girls and boys with juvenile idiopathic arthritis

Uveitis is the most common extraarticular manifestation of juvenile idiopathic arthritis (JIA) and is associated with considerable morbidity. The aim of this study was to examine the risk factors associated with uveitis in JIA. We conducted a chart review of 1,047 patients with JIA from a single tertiary care pediatric rheumatology center for factors associated with the development of uveitis. Special emphasis was put on the following known risk factors: oligoarthritis, antinuclear antibody (ANA) status, sex, and age at the time of onset of JIA. The risk of uveitis developing was age dependent in girls but not in boys. Among girls, the risk was maximal (47%) in those who were ANA positive and were ages 1-2 years at the time of the onset of JIA; this risk decreased to <10% in those in whom the age at onset was >7 years. Only girls had an age-dependent and ANA-associated increased risk of uveitis. The time interval from the diagnosis of JIA to the diagnosis of uveitis was statistically significantly longer in patients in whom the onset of JIA occurred at a younger age (P = 0.04). This effect was even more pronounced in ANA-positive patients (P = 0.004). The JIA subtype did not influence a patient's risk of the development of uveitis. An age-associated risk of uveitis was observed only in girls who were younger than 7 years of age at the time of the onset of JIA. The duration of time between the diagnosis of JIA and the onset of uveitis was longer in patients in whom JIA was diagnosed at a younger age, especially in those who were ANA positive. We suggest that our findings have implications for uveitis screening in patients with JIA.

Clinical characteristics and long-term outcomes of 35 patients with Wegener’s granulomatosis followed up at two rheumatology centers in Lithuania

The aim of this study was to investigate the survival of Lithuanian patients with Wegener's granulomatosis, who were followed up at two tertiary rheumatology centers, and to find the factors possibly influencing the outcomes of this disease. Thirty-five patients were followed up prospectively from the onset of disease (the first patient was enrolled in 1994) at Vilnius University Hospital and the Center of Rheumatology of Kaunas University of Medicine (17 and 18 patients, respectively). All patients in both the centers were followed up on a routine basis, and their records contained necessary information about laboratory and biopsy data; the censoring date (end of follow-up) was stated in June 2006. Among the patients, the most frequent organs involved were ear, nose, throat (ENT) (82.6%), lungs (74.3%), and kidney (renal involvement was defined by proteinuria/abnormal urine sediment) (45.7%). Renal insufficiency was present in 20.6% of all the patients. At the end of the study, 32.4% of patients had simultaneously all three organ systems involved, namely upper respiratory tract, pulmonary, and renal. ANCA positivity was found for 26 (74.3%) of all the patients. Overall mortality rate was 25.7% (9/35). The mean survival was 99.4 months (95% CI, 73.6; 125.3) limited to 149 months for the longest-surviving patient. Female gender and all three specific organ involvements being present at the same time and higher vasculitis damage index were associated with poor outcome. Overall mortality rate was 25.7% (9/35) during the 12-year follow-up, and it is similar to the data from other European countries.

Clinical manifestations and outcomes of antithrombotic treatment of the Tan Tock Seng Hospital Singapore antiphospholipid syndrome cohort

To examine the clinical manifestations, intensity of oral anticoagulation and outcomes in the prevention of recurrent thromboses in patients with antiphospholipid syndrome (APS) in a tertiary rheumatology centre in Singapore. Retrospective case review of consecutive patients with APS attending a rheumatology clinic from 1st January 2004 to 31st December 2005. There were 59 (44%) patients with definite APS and 75 (56%) with probable APS. Systemic lupus erythematosus (SLE) was the most common cause of secondary APS. Hypertension and hyperlipidaemia were the most common cardiovascular comorbidities. The most common manifestations were haematological (thrombocytopaenia and haemolytic anaemia), neurological (seizure, headache) and pulmonary hypertension. Among those with definite APS, there were similar proportions with arterial and venous thromboses. Recurrent thromboses occurred in 14 (23.7%) patient with definite APS receiving warfarin, comprising 14 (73.7%) episodes of arterial and 5 (26.3%) episodes of venous thromboses. Recurrent arterial thromboses occurred at international normalized ratio (INR) of <2 in 5 (35.7%), INR 2-3 in 6 (42.9%), INR > 3 in 3 (21.4%) episodes, respectively. Recurrent venous thromboses occurred at INR < 2 in 4 (80.0%) and INR > 3 in 1 (20.0%) episode, respectively. Twenty-eight episodes of bleeding occurred in 21 (35.6%) patients, the majority (78.6%) being minor bleeding. Two-thirds of all major bleeds occurred at INR >/= 3. Venous and arterial thromboses were equally common in our patients with definite APS, although recurrent thromboses were more common in the arterial circulation. Target INR > 3 was associated with lower rates of recurrent arterial thromboses but higher rates of major and recurrent bleeding. Target INR >/= 2 appeared to be sufficient to prevent recurrent venous thromboses.

A comparative evaluation of MRI, radionucleide bone scan and plain radiographs in Indian patients with spondyloarthropathy

Background Spondyloarthropaties (SpA) are a diverse group of disorders characterized by inflammatory low backache, genetic predisposition and a variety of articular and extraarticular manifestations. Evidence of sacroiliitis in plain radiographs forms the cornerstone for establishing the diagnosis. However, it may take many years for the sacroiliitis to become visible. With the availability of biologics that have the potential to modify the course of SpAs, there is a need for early diagnosis of these disorders. Magnetic resonance imaging (MRI) and nuclear scintigraphy (radionuclide bone scan) appear promising in this context with their ability to pick up structural damage and inflammation before their presence is detected in plain radiographs. Objectives To assess the role of MRI and bone scan in patients with early SpA. Methods This was a cross sectional study done at a tertiary care rheumatology center of the armed forces. Patients satisfying the European Spondyloarthropathy Study Group (ESSG) criteria for Spodyloarthropathy and disease duration of less than 8 years were included. All patients underwent conventional radiography, MRI imaging and nuclear scintigraphy of the sacroiliac (SI) joints. The primary outcome assessed was the positivity rate for sacroiliitis of each of the three modalities in this group of patients. The sensitivity of each modality in contributing to the diagnosis over and above that of plain radiographs was assessed. Results Forty-four patients (predominantly young men, n = 39) with a median disease duration of 5 years were included in the study. Most patients had ankylosing spondylitis ( n = 21, 47.7%) closely followed by undifferentiated spondyloarthropathy ( n = 14, 31.8%), reactive arthritis ( n = 5, 11.1%) and psoriatic arthropathy ( n = 4, 9.2%). Evidence of sacroiliitis was seen in 59% (26/44) patients in plain radiographs, in 73% (34/44) with bone Scan and in 77% (34/44) with MRI. There was significant discordance among the three imaging modalities, documented in 49 of the 132 observations (37%). Amongst patients with a disease duration < 2 years (17/44, 39%), the plain radiographs showed changes in less than half the patients (8/17, 47%) with the MRI scan being positive in 88% of patients and the bone scan being positive in over 80% of patients. Though MRI and bone scan continued to have a higher pickup rate with increasing disease duration, the difference was most striking at 2 years. Amongst patients with undifferentiated spondyloarthropathies (USpA), ( n = 14), none of the patients had evidence of sacroiliitis on plain radiographs. However 10 (71.5%) patients each had evidence of sacroiliitis on MRI and bone scan, with 8 (57.1%) patients having both MRI and bone scan findings suggestive of sacroiliitis. Plain radiographs, MRI and bone scan, when used in combination, are able to detect sacroiliitis in almost all patients with SpA. Conclusions MRI had the maximum sensitivity (78%) for detecting sacroiliitis closely followed by bone scan (73%). Their utility was most apparent in patients with disease duration lesser than 2 years where plain radiographs have the least sensitivity in detecting sacroiliitis. They were also very useful in the subgroup of patients with uSpA where the radiographs were universally negative. MRI and bone scan individually picked up evidence of sacroiliitis in most of the patients with USpA and in combination picked up all the cases suggesting their usefulness in this group. However, there was a significant discordance rate amongst the three modalities and bone scan seems to lack specificity. MRI may be the preferred modality in patients with USpA and in those with early disease, given the poor specificity of bone scan.

Impaired reproductive fitness in mothers of children with juvenile autoimmune arthropathies

To assess the reproductive fitness of mothers of children with juvenile idiopathic arthritis (JIA). A mail survey assessing pregnancy outcome was carried out among mothers of children with JIA (JIA mothers) treated at a tertiary paediatric rheumatology centre. The best friends of the JIA mothers served as controls. Besides family history, sociodemographics and reproductive outcomes were measured, including the number of pregnancies, pregnancy complications and gestational age at the time of delivery. JIA mothers (n = 227) and controls (n = 235) had similar sociodemographics and racial backgrounds. On average, JIA mothers reported a greater number of conceptions than controls (3.5 vs 3.1; P = 0.01) but had significantly higher rates of pregnancy complications (25% vs 15%; P<0.001). Corrected for differences in the absolute number of pregnancies between groups, the chances of having a miscarriage [mean (s.d.), 0.12 (0.18) vs 0.09 (0.16); P = 0.02] or preterm delivery [0.08 (0.21) vs 0.04 (0.15); P<0.02] were significantly greater among JIA mothers than controls. Mothers of children with JIA have impaired reproductive fitness. This phenomenon is unlikely to be the result of difficulty with conception but rather to be due to higher rates of pregnancy loss and premature delivery.