Comparison of articular manifestations of mixed connective tissue disease and systemic lupus erythematosus on clinical examination and musculoskeletal ultrasound.

BACKGROUND: Polyarthritis is common to both mixed connective tissue disease (MCTD) and systemic lupus erythematosus (SLE). Apart from being erosive and deforming in the former, we speculated that it was more common and the extent of joints involved would be higher in MCTD. METHODS: This was a cross-sectional study that included patients with MCTD aged 18-75 years fulfilling the Kasukawa criteria. An equal number of patients with SLE matched for disease duration and gender were included. Clinical manifestations were compared between patients with MCTD and with SLE. Examination of joints was done for the presence of tenderness or swelling and deformity. Musculoskeletal ultrasound was done on the non-dominant hand for detection of synovitis and tenosynovitis and radiographs of the hands were obtained. The use of methotrexate and non-steroidal anti-inflammatory drugs (NSAIDs) for arthritis was noted. Statistical tests used were non-parametric. RESULTS: Forty patients with MCTD and forty patients with SLE were included in this study, with patients being slightly older in MCTD than SLE (36 ± 10.2, 31.8 ± 13.3 years, p = 0.01). There were no significant differences in disease duration (4.7 ± 3.1, 3.7 ± 2.3, p = 0.1) or gender (females = 38, 38). Nearly one-half of patients with MCTD had at least one swollen joint compared with only 15% of patients with SLE. Median (95% confidence interval) tender joint count (5 (4.8-10.4), 0 (1.3-7.2), p = 0.01) and swollen joint count (0 (0.9-2.6), 0 (0-1.2), p = 0.002) was significantly higher in patients with MCTD compared with SLE. More patients with MCTD than SLE had tender or swollen proximal interphalangeal joints (12, 4, p = 0.025). More patients with MCTD than SLE had received methotrexate (8,2, p = 0.04) and NSAIDs (39, 32, p = 0.03) for arthritis. There was no difference in the number of patients with MCTD or SLE who had evidence of synovitis or tenosynovitis on ultrasound. There was no difference in erosive disease on hand radiographs, but acro-osteolysis was higher among MCTD patients. CONCLUSIONS: A higher proportion of patients with MCTD had at least one swollen and tender joint as compared with patients with SLE, as well as higher use of methotrexate and NSAIDs. However, there was no difference in ultrasound detected synovitis or tenosynovitis.

Correlation between salivary and serum anti-desmoglein 1 and 3 antibody titres using ELISA and between anti-desmoglein levels and disease severity in pemphigus vulgaris.

ELISA for anti-desmoglein antibodies (Dsg) is commonly used for diagnosis and assessment of treatment response in pemphigus vulgaris (PV). The present study was conducted to assess the relationship between salivary and serum Dsg1 and Dsg3 levels, and whether salivary Dsg1 and Dsg3 levels correlate with clinical disease severity of oral mucosal lesions in PV. In total 43, patients with PV with predominantly mucosal involvement were recruited. Both serum and salivary samples were collected from the cases, and salivary samples were also collected from five controls. There was a statistically significant correlation between serum and salivary Dsg1 levels and between serum and salivary Dsg3 levels. There was no correlation between serum or salivary Dsg1 and Dsg3 levels with the objective component of the oral mucosal Autoimmune Bullous Skin Disorder Intensity Score (ABSIS). Serum Dsg1 levels significantly correlated with cutaneous ABSIS, but there was no correlation between cutaneous ABSIS and either salivary Dsg1, salivary Dsg3 or serum Dsg3. As salivary Dsg titres correlate with serum levels, saliva can serve as a simple and noninvasive alternative to serum for Dsg ELISA.

Deranged regulatory T-cells and transforming growth factor-ß1 levels in type 1 diabetes patients with associated autoimmune diseases.

AIM: This study was designed to enumerate regulatory T-cells (Tregs) and estimate transforming growth factor-ß1 (TGF-ß1) levels in type 1 diabetic (T1D) patients with respect to disease duration and associated autoimmune diseases. METHODS: One hundred and fifty patients and twenty healthy controls were recruited in the study. The patients were subcategorized into eight categories on the basis of disease duration (new onset [NO] and long standing [LS]) and associated diseases, i.e., celiac disease (CD) and autoimmune thyroid disease (AiTD). Treg cells were assessed as CD4+ CD25hi+, FOXP3+ cells and serum TGF-ß1 levels were assessed by ELISA. RESULTS: The frequency of Tregs and levels of TGF-ß1 were significantly increased in the patients compared to the healthy controls. Among the different categories of the patients, no significant differences were seen for TGF- ß1 levels, but for Tregs in patients with T1D and AiTD (P = 0.035). A significant correlation was also found between percentage count of Tregs and TGF-ß1 levels in NO cases in all disease subcategories, but not in LS patients. CONCLUSION: Thus, there was an increased percentage of Tregs and serum levels of TGF-ß1 in T1D patients, irrespective of the disease duration and associated autoimmune diseases. The significant correlation in these two parameters at the onset of the disease, but not in LS disease, indicates that the immunological milieu in LS autoimmune diseases is more complicated with disease-associated conditions such as prolonged hyperglycemia, insulin therapy, and/or continued gluten in diet. Treatment and modulation of these long-term complications for improving immunological parameters require further research.

Optic nerve involvement in childhood onset systemic lupus erythematosus: Three cases and a review of the literature.

The ocular system can be affected in systemic lupus erythematosus (SLE) in one third of patients. However, optic nerve involvement is relatively uncommon, but is more so in pediatric SLE patients, where it can occur in 1% of cases. We report three children with SLE who presented with optic nerve involvement. Two children had optic neuritis, with optic neuritis being the first manifestation in one child. The third child had ischaemic optic neuropathy secondary to antiphospholipid syndrome. A careful work up for SLE should be performed in every child with optic nerve disease. Prompt diagnosis and early treatment results in a better prognosis.

Circulating T-helper 17 cells and associated cytokines in psoriasis.

BACKGROUND: Recently, a new population of IL-17-producing CD4 T helper (Th) cells, named Th17, was identified and shown to be involved in various inflammatory and autoimmune diseases, including psoriasis. AIM: To determine the frequency of Th17 cells and related cytokines in peripheral blood of patients with psoriasis, and to analyse their association with disease severity. METHODS: This was a prospective study comprising 34 patients with psoriasis and 24 healthy controls. Clinicoepidemiological details of patients were recorded, and severity of psoriasis was assessed by means of the Psoriasis Area and Severity Index. Circulating Th1 and Th17 cells in untreated patients with psoriasis and healthy controls were quantified by flow cytometry. In sera collected from patients with psoriasis and healthy controls, concentrations of IL-17A and IL-23 were examined by ELISA. RESULTS: Increased frequencies of CD4+ IL-17A+ T cells were seen in peripheral blood of patients with psoriasis vulgaris (P < 0.001). Although serum IL-17A and IL-23 concentrations were higher in patients with psoriasis than in controls, the results did not reach statistical significance. We could not find any correlation between the studied T cells or related cytokines and the disease severity. CONCLUSION: Increased serum levels of circulating Th17 cells and related cytokines may contribute to the cutaneous pathology of psoriasis, as well as the inflammatory process that is a hallmark of psoriasis.

Childhood lupus nephritis in a developing country-24 years' single-center experience from North India.

OBJECTIVE: Data on outcome of childhood lupus nephritis from developing countries are sparse. This study looks at outcome in children with lupus nephritis from a federal government-funded teaching hospital in North India. METHODS: This study included children less than 14 years of age with lupus nephritis who presented to a single center during a period of 24 years (1991 to 2013). Data on clinical characteristics and outcome were extracted from medical records. The primary outcome was actuarial survival (time-to-death) and secondary outcome was actuarial renal survival using Kaplan-Meier analysis. A worst-case scenario that assumed children who were lost to follow-up as having either died or gone into end-stage renal disease was also calculated. Log-rank test and Cox-regression were used to assess difference in survival by histological class and predictors of poor outcome, respectively. RESULTS: This study included 72 children, with a female:male ratio of 3:1, mean (±SD) age at onset of lupus 9.3 (±2.4) years and mean (±SD) time from onset-to-nephritis being 9.4 (±12.6) months. Renal biopsy was conducted in 53 children. The most common histological class was class IV (35 children). Mortality occurred in 22 children (30%), with half of these occurring at presentation. The two important causes of death were infection and end-stage renal disease. Actuarial survival was 81%, 67% and 59% at one, five and 10 years, respectively. In the worst-case scenario, actuarial survival was 72%, 53% and 38%, respectively. Renal survival was 96%, 89% and 78% (worst-case scenario 86%, 73% and 52%) at one, five and 10 years, respectively. There was no difference in survival by histological class. On univariate analysis, serum creatinine at presentation (hazard ratio = 2.2 (95% CI 1.3-3.9)) and serious infection (hazard ratio 7.9 (95% CI 2.6-23.5)) were statistically significant predictors of time-to-death. CONCLUSION: Outcome of children with lupus nephritis from India is worse than developed countries. Nearly one-third of the children died, half at presentation, with common causes being infection and end-stage renal disease.

Clinical and treatment characteristics determining therapeutic outcome in patients undergoing autologous non-cultured outer root sheath hair follicle cell suspension for treatment of stable vitiligo.

BACKGROUND: Autologous non-cultured outer root sheath hair follicle cell suspension (NCORSHFS) is a recently described novel cellular graft technique for the treatment of stable vitiligo. There is lack of data about various factors determining the repigmentation rate in vitiligo patients undergoing this novel surgical therapy. OBJECTIVE: To study the clinical characteristics and treatment variables determining therapeutic outcome in patients of stable vitiligo undergoing NCORSHFS. METHODS: Non-cultured outer root sheath hair follicle cell suspension was prepared from anagen hairs extracted from the occipital area. The number of melanocytes and hair follicle stem cells (HFSC) in the suspension was quantified by staining with anti-HMB45 and anti-CD200 antibody, respectively. In all patients, a 2 mm punch skin biopsy was taken from one of the vitiligo patch to be treated prior to surgery for assessment of histomorphological features. Post surgery patients were followed up at regular intervals for 24 weeks. RESULTS: Thirty patients (21 females, 9 males) with a clinical diagnosis of stable vitiligo, with a total of 60 target lesions were included in this study. The mean age of the study population was 21.10 ± 5.64 years. The number of melanocytes (P = 0.04) and HFSC (P = 0.01) transplanted were significantly higher among patients achieving optimum repigmentation (>75% repigmentation). There was a strong correlation between repigmentation at 24 week and number of melanocytes and HFSC transplanted. Number of HFSC transplanted and absence of dermal inflammation were significant predictors of achieving optimum repigmentation. CONCLUSION: The number of melanocytes and HFSC transplanted and absence of dermal inflammation were important determents of optimal repigmentation in patients undergoing NCORSHFS for treatment of stable vitiligo. Hence, refining the technique of NCORSHFS on the basis of these factors would help in achieving better surgical outcomes.

Clinical and immunological outcomes of high- and low-dose rituximab treatments in patients with pemphigus: a randomized, comparative, observer-blinded study.

BACKGROUND: Rituximab is a promising therapy in pemphigus. However, there is no consensus on optimum dose. OBJECTIVES: To compare the efficacy, in terms of clinical and immunological outcomes in patients with pemphigus, of a high (2 × 1000 mg) vs. a low dose (2 × 500 mg) of rituximab. METHODS: This was a randomized, observer-blinded trial wherein 22 patients with pemphigus were randomized into two treatment groups. Patients received either two doses (day 0 and day 15) of 1000 mg rituximab or 500 mg rituximab, and were followed up for 48 weeks. Clinical activity was assessed by a blinded investigator. Indices of enzyme-linked immunosorbent assays (ELISAs) for desmoglein (Dsg)1 and Dsg3, and CD19 cell count were examined at regular intervals. RESULTS: There was no statistically significant difference in early and late clinical end points, and total cumulative dose of corticosteroids between the two groups. At week 40, the fall in Ikeda severity score was significantly more in the 2 × 1000 mg group than in 2 × 500 mg group (P = 0·049). Patients in the 2 × 500 mg group received a significantly higher cumulative dose of azathioprine (P = 0·018). The ELISA indices of Dsg1 and Dsg3 showed a statistically significant decline in the 2 × 1000 mg group only. B cell repopulation occurred earlier in the 2 × 500 mg group by 8 weeks. CONCLUSIONS: A few clinical and immunological study parameters have suggested improved outcomes in patients receiving high-dose (2 × 1000 mg) rituximab.

Gene expression of cytokines (TNF-α, IFN-γ), serum profiles of IL-17 and IL-23 in paediatric systemic lupus erythematosus.

OBJECTIVE: Paediatric systemic lupus erythematosus (pSLE) exhibits an aggressive clinical phenotype and severe complications commonly renal involvement. This could be reflective of the ongoing chronic pro-inflammatory cytokine milieu. We examined relative gene expression of tumour necrosis factor-alpha (TNF-α), interferon-γ (IFN-γ) and serum levels of interleukin-17 (IL-17) and IL-23 and their association with SLEDAI (SLE disease activity index) score and organ manifestations in pSLE. METHODS: We enrolled 40 pSLE patients (age 5-16 years, on treatment) and 20 age-matched healthy controls. Relative gene expression levels of IFN-γ and TNF-α in the peripheral blood were determined by quantitative real-time reverse transcriptase polymerase chain reaction (RT-PCR). ß actin gene was used for normalization of gene expression. Serum levels of IL-17 and IL-23 were determined by solid phase sandwich ELISA. Statistical analysis were carried out for comparing (Mann-Whitney U test) and correlating data (Univariate, multivariate analysis and Pearson correlation test) with SLEDAI scores and clinical manifestations. RESULTS: Over-expression of TNF-α and IFN-γ was found in 90% (36/40) and 80% (32/40) of pSLE patients, respectively. The relative gene expression of TNF-α and IFN-γ were significantly correlated with renal manifestations (p < 0.05). Further, relative expression of IFN-γ gene correlated significantly with skin manifestations and SLEDAI (p < 0.05). Serum levels of IL-17 (766.95 ± 357.83 pg/ml) and IL-23 (135.4 ± 54.23 pg/ml) in pSLE were significantly higher than in controls (IL-17, 172.7 ± 39.19 pg/ml and IL-23, 21.15 ± 10.99 pg/ml) (p < 0.05). Patients with cutaneous (p = 0.002) and haematological involvement (p = 0.003) had high serum IL-17 levels. Serum IL-17 levels correlated with SLEDAI (r = 0.447; p < 0.05). CONCLUSIONS: In this preliminary study, we observed a persistent, strong pro-inflammatory cytokine milieu in pSLE patients which reflects ongoing inflammatory damage in different organs. The gene expression profile of these cytokines may be used for assessing organ involvement in pSLE. IL-17 may also serve as a prognostic marker in pSLE. However, longitudinal studies on treatment of naïve patients are required to corroborate these findings.

Transcriptional expression and gelatinolytic activity of matrix metalloproteinases in Henoch-Schonlein purpura.

AIM: Accelerated extracellular matrix breakdown caused by the increased activity of matrix metalloproteinases (MMPs) has been implicated in several rheumatological disorders and systemic vasculitides, especially Takayasu's arteritis and Kawasaki disease. Therefore, the aim of the present study was to investigate the potential role of MMPs in Henoch-Schonlein purpura (HSP), an acute type of systemic vasculitis in children. METHODS: We studied the activity of MMP-2 and MMP-9 in the sera using gelatin zymography and the transcriptional expression in peripheral blood mononuclear cells using semi-quantitative RT-PCR in 20 patients with HSP in acute and convalescent phase and in 20 healthy children, who were siblings of the subjects with same age group. RESULTS: All 20 children with HSP showed increased levels of serum activity of MMP-2 and MMP-9 in acute phase as compared with their convalescent phase [MMP-2 (p > 0.05); MMP-9 (p > 0.05)] and their control counterparts [MMP-2 (p < 0.001); MMP-9 (p < 0.001)]. Similarly, transcriptional expression of MMPs was found to be higher in the acute phase of HSP than in convalescent phase [MMP-2 (p < 0.05); MMP-9 (p < 0.001)] and in their healthy controls [MMP-2 (p < 0.001); MMP-9 (p < 0.01)]. CONCLUSION: The presence of excessive transcriptional expression and gelatinolytic activity of MMPs may be downstream to the actual aetiopathogenetic factors.

Anti-glomerular basement membrane disease: Case series from a tertiary center in North India.

Anti-glomerular basement (anti-GBM) disease is an uncommon disorder with a bimodal age of presentation. Patients presenting with dialysis-dependent renal failure have poor renal outcomes. There is limited data regarding the clinical presentation and outcomes of anti-GBM disease from India. We conducted this prospective study to analyze the clinical presentation and outcomes of anti-GBM disease at a large tertiary care hospital in North India over 1½ years. Subjects with a biopsy proven anti-GBM disease (light microscopic examination showing crescents and immunofluorescence examination showing linear deposition of IgG) with or without positive anti-GBM antibodies in serum were included in the study and followed-up for at least 12 months. All the patients were treated with steroids, cyclophosphamide, and plasma exchange. A total of 17 patients (nine males) were included. The mean age at presentation was 39.11 ± 16.58 (range 11-72) years. Twelve patients (70%) presented with rapidly progressive glomerulonephritis (RPGN), 4 (23.5%) presented with Goodpasture syndrome, while 1 (5.8%) had nephritic syndrome, 7 (41%) were hypertensive, and 14 (82.3%) required dialysis at the time of presentation. Four patients (23.5%) had associated anti-neutrophil cytoplasmic antibody positivity (anti-myeloperoxidase antibodies in all). Fourteen (87.5%) patients had crescentic glomerulonephritis, while 5 (31.25%) showed necrotizing (n = 4) or granulomatous (n = 1) in the vasculitis. Of 16 patients who received treatment, four (23.25%) achieved complete remission. In this single-center study, the majority of anti-GBM disease patients presented with RPGN and had crescentic glomerulonephritis on biopsy with poor treatment outcome.

Prevalence & significance of hepatitis C virus (HCV) seropositivity in blood donors.

BACKGROUND & OBJECTIVES: The clinical significance of anti HCV antibodies in healthy blood donors remains uncertain. These donors are usually asymptomatic and it is difficult to elicit risk factors of acquiring HCV infection during pre-donation questioning. Limited information on donor recall and follow up studies on anti HCV positive blood donors have been reported from India. Paucity of data which is likely to have an impact on safe blood transfusion programme has prompted us to undertake this study to assess the significance of HCV seropositivity in blood donors with respect to their clinical, biochemical and virological profile. METHODS: A total of 16,250 blood units were screened for the mandatory tests using third generation ELISA (anti HIV 1&2, anti HCV, HBsAg), VDRL and peripheral smear for malaria. Donors reactive for anti HCV were informed. Repeat anti HCV reactive donors were subjected to detailed clinical history focusing on risk factors for HCV transmission. The blood tests included liver function tests (LFT), coagulation and autoimmune profile, qualitative serum cryoglobulins and HCV RNA detection. These donors were followed at 2-3 monthly intervals for a minimum period of six months by LFT. RESULTS: An overall seropositivity of 0.44 per cent (72/16,250) was observed in our donors which was significantly lower in first time, young voluntary donors as compared to replacement donors (0.27 vs. 0.60%). In contrast to drug abuse (6.4%) we found minor percutaneous routes like sharing of shaving kits or visit to a road side barber (32%) as the major risk factor for HCV transmission. There was no prior history of blood transfusion in any of these donors; however history of some surgical procedures was present in 25.8 per cent. Raised transaminases and HCV viraemia were observed in 87 and 71 per cent donors respectively. An association was observed between HCV RNA when the ELISA ratio was >5. INTERPRETATION & CONCLUSION: Voluntary donors form a safe source of blood supply and efforts should be made to increase this precious source to 100 per cent. Abbreviated behavioural donor screening questionnaire for repeat donors is not advisable. Awareness and education of donors is required regarding modes of HCV transmission. HCV positive donors should be informed about their disease, counselled and referred to hepatologist, and permanently deferred for future donations.

Bcl2 and ROS1 expression in human meningiomas: an analysis with respect to histological subtype.

Tumors of the central nervous system account for approximately 9% of all primary neoplasm in humans, while tumors of covering elements, the meninges, account for 13-19% and constitute the second largest group of brain tumors. These are known to exhibit a variety of chromosomal abnormalities besides change in the expression level of certain oncogenes. Among oncogenes, bcl2, an anti-apoptotic factor and ROS1 that encodes a protein with a structure similar to the epidermal growth factor (EGF) and insulin receptor and has a tyrosine kinase activity, have been shown to be associated with many malignant tumors. In the present study we have analysed the expression of bcl2 using immuno-histochemistry and ROS1 expression by reverse-transcription coupled with polymerase chain reaction (RT-PCR) of the transcript using primers specific for the intra-cellular domain and then tried to correlate the findings with the subtype of the meningioma defined on the basis of histology. Out of the six bcl2 positive cases in our study, there were three transitional tumors, two fibroblastic and one recurrent meningioma subtype. bcl2 seemed to be more consistently expressed in the cytoplasm of spindle cell component of meningiomas. Thirteen meningiothelial meningiomas did not show any staining for bcl2. ROS1 gene expression could be detected in 4 tumors all of those were Grade-I meningothelial meningiomas. One of the malignant meningioma included in the study was clearly negative for bcl2 as well as ROS1. Thus bcl2 and ROS1 oncogene expression in meningiomas are not concurrent and neither can be ascribed to any histologic subtype or grade of tumor.

Therapeutic implication of quantitative pp65 antigen assay in living renal transplant in a high seroendemic population.

Various methods have been used to diagnose cytomegalovirus (CMV) infection/disease; however, pp65 antigenemia assay has emerged as a good marker for CMV disease in a high seroendemic population. We studied the role of quantitative pp65 antigen assay in live related renal transplant recipients in a high seroendemic population. Between November 1998 and May 2003, a total of 350 blood samples from 250 symptomatic patients were tested by quantitative pp65 antigen assay; 14% of the patients tested positive. There were 5 (14%) low-positive and 30 (86%) high-positive patients. All high-positive patients had CMV disease. The response to antiviral therapy monitored by the assay was dramatic, and one low-positive patient responded to reduction in immunosuppression. In conclusion, pp65 antigen assay is a good test for diagnosing CMV disease and monitoring response to antiviral therapy in a high seroendemic population.

Intestinal cryptosporidiasis in living related renal transplant recipients.

Cryptosporidium infestations are common in immunocompromised AIDS patients. However, the literature in renal transplant recipients is scarce. We conducted a study to know the prevalence, disease manifestations, and management of cryptosporidial infestations in live related renal transplant recipients. Cryptosporidial infestations were observed in 20% of patients, including 16.6% who had symptomatic diarrhea. We conclude that the prevalence is high in the transplant population, but only a few patients are symptomatic. Clinicians should routinely request special stains to demonstrate cryptosporidium in stool specimens.

Clinical and immunological profile of SLE: some unusual features.

OBJECTIVE: To study the clinical and immunological profile of children with systemic lupus erythematosus (SLE). DESIGN: Retrospective hospital based study. SETTING: Tertiary level center of North India. SUBJECTS: Sixteen children in the age group 4-12 years. METHODS: Medical records of children with SLE were analyzed. Clinico pathological features were compared with 2 other series from India. RESULTS: Mean age of children at the time of diagnosis was 10 yr and 8 (50%) children were less than 10 yr of age. The female to male ratio was 7:1. Fever (56.2%), rash (87%) and arthritis (87%) were the common clinical manifestations, Renal involvement was noted in 56.2% of cases. Other clinical features included hemolytic anemia (31.2%), thrombocytopenia (18.6%) and Raynaud's phenomenon (12.5%). Cardiac involvement in the form of severe myocarditis and endocarditis occurred in one patient each. Pulmonary hypertension was the presenting feature in one child with right heart failure. One child had multiple sclerosis along with SLE--a rare combination. ANA positivity was seen in all children. Five children died; two had severe cardiac involvement. Three children had renal involvement and one died of pulmonary hypertension. Two-thirds of subjects with renal involvement improved after therapy according to NIH, Bethesda protocol. CONCLUSIONS: SLE must be considered in any child with multisystem disease, as the disease may have certain unusual presentations.

Clinico-immunological profile of juvenile rheumatoid arthritis at Chandigarh.

OBJECTIVE: To study the clinical and immunological profile of children with juvenile rheumatoid arthritis (JRA). DESIGN: Retrospective hospital based study. SETTING: Tertiary level center of North India. SUBJECTS: 74 patients attending the Pediatric Rheumatology and Immunology Clinic over last 5 years. RESULTS: The patients were aged between 9 months to 12 years with male female ratio of 1.8:1. Eleven (14.9%) patients had systemic onset JRA, 28 (37.8%) had polyarticular onset type and 35(47.3%) had pauciarticular onset type JRA. Uveitis was present only in one patient and rheumatoid nodules were present in 4(5.4%) patients. Rheumatoid factor was positive in 2(2.7%) and antinuclear antibody was present in one patient only. HLA-B27 was positive in 4 children. Two patients developed amyloidosis. CONCLUSION: The clinico-immunological profile of JRA at Chandigarh appears to be some what different from that reported from other centers in India.