Lupus in a patient with cystinosis: is it drug induced?

A 9-year-old girl with a diagnosis of cystinosis since 2 years of age, on cysteamine therapy, presented with complaints of serositis and arthritis, and laboratory tests revealed high antinuclear antibody titers with hypocomplementemia. Kidney biopsy was not consistent with lupus nephritis. With prednisolone treatment her complaints resolved and creatinine level decreased, but on follow-up, serological features of systemic lupus erythematosus (SLE) continued. Six years after cessation of prednisolone, lupus features were reactivated, with positive antihistone antibodies and ANCA. Coincidence of cystinosis and SLE is very rare, and to the best of our knowledge this is the fourth case reported in the literature. Physicians should be aware that cystinosis patients may have some autoimmune manifestations with features of true or drug-induced lupus. In the light of this case, pathophysiology and treatment are discussed.

Acute lymphoblastic leukaemia in a child with systemic lupus erythematosus.

Haematological involvement of systemic lupus erythematosus (SLE) - which ranges from the well-described haemolytic anaemia to macrophage activation syndrome - has a large impact on both morbidity and mortality. On the other hand, association between haematological malignities and SLE - in terms of pathophysiology and molecular genetics - is an obscure entity which has not been clarified evidently to date. Herein, we present a six-year-old female with the diagnosis of SLE who developed acute lymphoblastic leukaemia following a period of myelodysplasia. It could possibly be coincidental; however, persistent cytopenia, prominent dysplasia on bone marrow smears and azathioprine treatment may be considered as possible triggers for the development of leukaemia in the present case.

Hematological features of pediatric systemic lupus erythematosus: suggesting management strategies in children.

AIM: The aim of this study was to analyze the hematological features in children with systemic lupus erythematosus (SLE) and to review our current treatment protocols. METHODS: We evaluated hematological findings of 43 children with SLE diagnosed and followed at the Pediatric Rheumatology Division of Hacettepe University, Turkey. Thirty-seven patients with hematological abnormalities were analyzed in detail. RESULTS: Median age at presentation was 13 years. Hematological involvement was seen in 86% of patients. The most common hematological finding was anemia (n = 30). Anemia was either a Coombs (+) hemolytic one, or was due to other causes. Hemolytic anemia was treated with steroids and intravenous gamma globulin (IVIG). Leucopenia and thrombocytopenia were detected in 35.1 % and 37.8 %, respectively. Bone marrow aspiration was performed in 15, mainly for cytopenia. Secondary dysplastic changes were common. Acute lymphoblastic leukemia (ALL) was diagnosed in one patient. Six patients were diagnosed as having macrophage activation syndrome (MAS). One patient died due to secondary infections and multiorgan failure despite aggressive treatment. In patients diagnosed early, treatment with steroids and cyclosporine resulted in an excellent response. Thrombotic microangiopathy was detected in two patients. Both were treated successfully with steroids and plasma exchange. Antiphospholipid and anticardiolipin antibodies were positive in 12 and 15 of the patients, respectively. Five developed deep vein thrombosis (DVT), one cerebral sinus thrombosis and one presented with purpura fulminans. They were effectively treated with anticoagulation protocol. CONCLUSION: Hematological findings should be carefully assessed and treated vigorously to prevent the morbidity and possible mortality.

MEFV mutations in systemic onset juvenile idiopathic arthritis.

OBJECTIVES: Autoinflammatory diseases constitute a large spectrum of monogenic diseases like FMF or cryopyrin-associated periodic syndromes (CAPS) and complex genetic trait diseases such as systemic onset juvenile idiopathic arthritis (SoJIA). An increased rate of MEFV mutations has been shown among patients with PAN and HSP, in populations where FMF is frequent. The aim of the study is to search for MEFV mutations in our patients with SoJIA and see whether these mutations had an effect on disease course or complications. METHODS: Thirty-five children with the diagnosis of SoJIA were screened for 12 MEFV mutations. The control data were obtained from a previous study of our centre determining the carrier frequency in Turkish population. RESULTS: Two patients were homozygous and three patients were heterozygous for the M694V mutation. One patient was a compound heterozygote for the M680I/V726A mutations. Heterozygous V726A mutation was found in one patient. The overall mutation frequency of patients was 14.28%. This figure had been compared with the previously published rate of disease-causing mutations in this country, which is 5%. Disease-causing mutations were found to be significantly more frequent in the SoJIA patients than the population (P < 0.01). Among these, M694V was the leading mutation with a frequency of 10% in SoJIA. Six patients carrying MEFV mutations were among the most resistant cases requiring biological therapy. CONCLUSION: SoJIA patients had a significantly higher frequency of MEFV mutations but clinical studies with large number of patients are needed to confirm the association of MEFV mutations with SoJIA and its course.

Renal transplantation in children with lower urinary tract dysfunction of different origin: a single-center experience.

INTRODUCTION: Renal transplantation in patients with lower urinary tract dysfunction (LUTD) of various origins is a challenging issue in the field of pediatric transplantation. We report our single-center experience to evaluate patient and graft survivals as well as the risks of the surgery and immunosuppressive therapy. PATIENTS AND METHODS: Among 70 pediatric transplant patients, 11 displayed severe LUTD. Videourodynamic tests were performed on all patients preoperatively as well as postoperatively if required. The cause of urologic disorders were neurogenic bladder (n = 5) and urethral valves (n = 6). Clean intermittent catheterization (CIC) was needed in six patients to empty the bladder. To achieve a low-pressure reservoir with adequate capacity pretransplantation augmentation ileocystoplasty was created in four patients and gastrocystoplasty in one patient. Three of the patients received kidneys from cadaveric and eight from living donors. All patients were treated with calcineurin-based immunosuppressive therapy. RESULTS: The mean age at transplantation was 15 +/- 4.7 years. The median follow-up after transplantation was 36 months (6 to 62 months). At their last visit the median creatinine level was 0.95 mg/dL (0.8 to 2.4 mg/dL). Three patients had recurrent symptomatic urinary tract infections who had augmented bladder on CIC. One patient with ileocystoplasty who developed urinary leak and ureteral stricture in the early postoperative period was treated by an antegrade J stent. CONCLUSION: Severe LUTD carried high risks for the grafted kidney. However, our data suggested that renal transplantation is a safe and effective treatment modality, if the underlying urologic diseases properly managed during the transplantation course. Since surgery and follow-up is more complicated, patient compliance and experience of transplantation team have significant impacts on outcomes.

Outcome of primary glomerular disease in pediatric renal transplantation: a single-center experience.

INTRODUCTION: The recurrence of primary disease in transplantation is a well-known problem. We report our single-center experience to assess the frequency of the recurrence of primary glomerulonephritis in children after renal transplantation. PATIENTS AND METHODS: Medical reports of 14 children with primary glomerular disease were evaluated. Among the 14 grafts were 10 from living related and four from cadaveric donors. Ten were diagnosed as focal segmental glomerulosclerosis (FSGS), two membranoproliferative glomerulonephritis (MPGN), and two polyarteritis nodosa (PAN). The original diagnosis was biopsy-proven in every case. All patients were treated with calcineurin-based immunosuppressive therapy. RESULTS: The mean age was 15.5 +/- 5.4 years. The median transplantation duration was 47 months; however, one of the FSGS patient had hyperacute rejection. Five years later she received a second graft with a serum creatinine of 0.7 mg/dL at 7 years after transplantation. Posttransplant recurrence of FSGS was confirmed in two patients (20%), who were treated with plasmapheresis with no improvement of proteinuria, two FSGS patients had thromboses after transplantation. One had a cardiac thrombosis with heterozygote MTHFR mutation and one, a renal artery thrombosis and loss of graft with prothrombin 20210A mutation. They all have functioning grafts except these two. We did not observe recurrence of PAN or MPGN in patients. CONCLUSION: Although the number of patients is quite small, our recurrence rate was compatible with the previous reports. Additionally, we strongly recommend evaluation of all risk factors for thrombosis and give appropriate anticoagulation.

Triple immunosuppression with tacrolimus in pediatric renal transplantation: single-center experience.

AIM: In this single-center cohort, we retrospectively analyzed the efficacy and safety of tacrolimus in pediatric renal transplantation. METHODS: We examined the medical records of 22 consecutive renal transplantation recipients (12 boys, 10 girls) receiving tacrolimus, to evaluate occurrence of acute rejection (AR) episodes, glomerular filtration rates (GFR), and side effects. RESULTS: The mean recipient age was 15.07 +/- 3.96 years. Seven grafts came from cadaveric, and 15 from living related donors. The patients were placed on immunosuppression with prednisolone and tacrolimus plus azathioprine (n = 8) or mycophenolate mofetil (MMF) (n = 12) or enteric-coated mycophenolate sodium (n = 2). Eighteen patients received basiliximab on days 0 and 4. There were three AR episodes at 5, 9, and 12 months. Mean GFR at the end of 1 and 2 years were 97.1 +/- 24.0 mL/min/1.73 m(2) and 116.9 +/- 42.2 mL/min/1.73 m(2), respectively. There was no graft loss. Hypertension, hyperlipidemia, and hyperglycemia were present in 14 (63.6%), 3 (13.6%), and 3 (13.6%) patients, respectively, without gingival hyperplasia, tremor, or hypertrichosis. Supraventricular tachycardia was noticed in five patients (22.7%), three of whom needed antiarrhythmic drugs (13.6%). CONCLUSION: Our single-center experience with tacrolimus, steroid plus azathioprine or MMF or enteric-coated mycophenolate sodium regimen in pediatric kidney recipients showed a low rate of AR with excellent graft survival and function at 1 and 2 year posttransplantation. The increased rate of supraventricular tachycardia in this regimen had not been previously reported; this association merits further studies.

Interferon-gamma assays for the diagnosis of tuberculosis infection before using tumour necrosis factor-alpha blockers.

OBJECTIVES: Patients who receive tumour necrosis factor-alpha (TNF-alpha) blockers are mostly immunosuppressed. A study was performed to investigate whether an interferon-gamma (IFN-gamma) assay could represent an alternative approach to the tuberculin skin test (TST) for the diagnosis of latent tuberculosis infection (LTBI) in these patients. DESIGN: We prospectively enrolled 106 individuals into the study in two groups. Group 1 consisted of 38 healthy individuals and Group 2 included 68 patients with chronic inflammatory diseases evaluated for LTBI before the use of TNF-alpha blockers. RESULTS: Of all participants, nine had indeterminate IFN-gamma test results. Agreement between the two tests was poor in both groups (kappa values respectively -0.54 and 0.18). In a total of 97 subjects, 10 (10.3%) were positive by the IFN-gamma test and 49 (50.5%) by TST. CONCLUSION: We found poor agreement between TST and the IFN-gamma test in our study. Our limited preliminary data should be accepted as a basis for designing future studies that will be helpful for physicians to decide whether the IFN-gamma test is more sensitive than the TST test in detecting LTBI before the use of TNF-alpha blockers.

Renal transplantation in children with augmentation enterocystoplasty.

INTRODUCTION: We report our experience with renal transplantation in patients with severe bladder dysfunction who underwent prior augmentation cystoplasty. PATIENTS AND METHODS: Among 58 pediatric patients, three underwent bladder augmentation prior to renal transplantation. The patients' ages at transplantation were 10, 13, and 17. The etiologies of bladder dysfunction were posterior urethral valves in two patients and contracted bladder in one patient. Vesicoureteral reflux was concomitantly present in three patients. Pretransplant ileocystoplasty was created in two patients and gastrocystoplasty in one patient. All patients received kidneys from cadaveric donors and were treated with calcineurin-based immunosuppressive therapy. RESULTS: The patients had normal renal function without hydronephrosis of the transplanted kidney at 13, 22, 49 months follow-up. No patients had morbidity due to technical complications. All the patients were continent. Two of three patients required clean intermittent catheterization from a Mitrofanoff conduit, while one patient spontaneously voids without significant residual urine. Urinary tract infections observed in two patients were successfully treated without any permanent deterioration in graft kidney function. CONCLUSIONS: Our data suggest that augmentation cystoplasty is a safe and effective option to treat patients with end-stage renal disease undergoing kidney transplantation. Experience of the transplantation team with a qualified pediatric urologist is essential due to the potentially high risk of surgical complications during the long term management of these patients.

Mutation frequency of Familial Mediterranean Fever and evidence for a high carrier rate in the Turkish population.

Familial Mediterranean Fever (FMF) is a recessive disorder characterised by episodes of fever and neutrophil-mediated serozal inflammation. The FMF gene (MEFV) was recently identified and four common mutations characterised. The aim of this study was to determine the carrier rate in the Turkish population and the mutation frequency in the clinically diagnosed FMF patients. We found a high frequency of carriers in the healthy Turkish population (20%). The distribution of the five most common MEFV mutations among healthy individuals (M694V 3%, M680I 5%, V726A 2%, M694I 0% and E148Q 12%) was significantly different (P<0.005) from that found in patients (M694V 51.55%, M680I 9.22%, V726A 2.88%, M694I 0.44% and E148Q 3.55%).

Implications of certain genetic polymorphisms in scarring in vesicoureteric reflux: importance of ACE polymorphism.

Polymorphisms of the renin-angiotensin system (RAS) have been shown to affect renal prognosis in a number of diseases. We examined the influence of deletion (D) and insertion (I) polymorphism in the angiotensin I-converting enzyme (ACE) gene and the other polymorphic markers of RAS, and that of plasminogen-activator inhibitor-1 (PAI-1) on renal scarring in reflux nephropathy. Ninety-four children with third- or fourth-degree reflux were the subject of the study. They were stratified into two groups according to the technetium-99m-dimercaptosuccinic acid (DMSA) findings: the first group consisted of 41 patients with no scar formation. In the second group (n = 53), there was significant scar formation in the refluxing units. ACE levels, ACE gene, angiotensin-1 receptor (AT1) A1166C, angiotensinogen (ATG) M235T, and PAI-1 4G/5G polymorphisms were studied. In the second group with scarred kidneys, 18 patients had decreased renal function. The frequency of patients homozygous for the D allele was significantly greater in the second group with scar formation in the refluxing units compared with the first group of patients (P < 0.005). On multivariate analysis, the DD genotype was the only factor that had a significant impact on renal scar formation, introducing a 4.9-fold risk (P < 0.05, 95% confidence interval). We were unable to find any correlation with the presence ofDD genotype and hypertension, decreased renal function, proteinuria, or sex of the patient. DDgenotype correlated with the serum ACE levels (P < 0.005). AT1and ATGpolymorphisms and PAI-1 polymorphism did not correlate with scar formation or any of the parameters. This study provides evidence that the DDgenotype of ACE may be a genetic susceptibility factor contributing to adverse renal prognosis in reflux nephropathy; namely, scar formation. The role of the synergism between the aforementioned genetic polymorphisms can be enlightened with larger patient groups, possibly through multicenter studies.

Polyarteritis nodosa in patients with Familial Mediterranean Fever (FMF): a concomitant disease or a feature of FMF?

BACKGROUND: Familial Mediterranean Fever (FMF) is caused by mutations in the gene encoding pyrin and is characterized by self-limited, recurrent attacks of fever and serositis. Vasculitis has been increasingly reported in FMF. A study evaluating the prognosis in FMF and polyarteritis nodosa (PAN) patients has not been reported previously. OBJECTIVES: To determine the special characteristics and the prognosis of PAN in FMF patients. METHODS: A questionnaire was used for the present survey. The setting was 7 referral centers from Turkey and Israel. Seventeen patients who were diagnosed with FMF and who developed PAN were included. PAN was diagnosed in those who met the Chapel Hill consensus criteria for microscopic polyarteritis or classic PAN. The clinical features of these 17 patients and the outcomes of their vasculitis were analyzed. RESULTS: The age at diagnosis of PAN in these FMF patients ranged from 3.5 to 37 years. All patients had constitutional symptoms, elevated acute phase reactants, and myalgia at the time PAN was diagnosed. The diagnosis of PAN was confirmed by renal angiography in 8 patients, by renal biopsy in 6 patients, and by muscle and/or nodule biopsies in 6 patients. A number of patients had definite features of both classic PAN and microscopic polyarteritis. CONCLUSIONS: When compared with other PAN patients, those with FMF tended to have a younger age at PAN onset, more frequent perirenal hematomas, and an overall better prognosis. The cases with overlapping features of microscopic and classic PAN pose a problem for the current classification of vasculitis. We suggest that the clinical representation of PAN in FMF patients has certain characteristics and may be a feature of FMF per se.

Role of A-SAA in monitoring subclinical inflammation and in colchicine dosage in familial Mediterranean fever.

OBJECTIVES: 1) To compare the sensitivity of serum amyloid A protein (A-SAA) and other acute phase proteins (APPs) in determining subclinical inflammation in patients with familial Mediterranean fever (FMF) during an attack-free period; 2) to define those clinical, laboratory features that may modify the A-SAA level; and 3) to evaluate the effect of an increase in the colchicine dose on the A-SAA level. METHODS: A-SAA, CRP, ESR, fibrinogen and ferritin levels were measured in 183 patients [88 F, 95 M; median age 11.0 years (1.0-20.0)] with FMF during an attack-free period. Mutational analysis was available in 157 patients. The colchicine dose was increased in 26 randomly chosen patients with no attacks within the last year; laboratory studies were repeated at the end of the second month. RESULTS: During an attack-free period, the median A-SAA level was 74 (6-1,500) mg/L; other APPs were within normal ranges in 49-93% of the patients. Age, gender, age at onset, age at diagnosis, the duration of treatment and the frequency of attacks had no significant effect on the A-SAA level. Homozygous and compound heterozygous patients had higher A-SAA levels than heterozygous patients [129 mg/L (8-1,500) versus 29 mg/L (6-216); p < 0.005]. There was a dramatic decrease in the A-SAA level [from 244 mg/L (16-1,400) to 35.5 mg/L (8-1,120); p < 0.001] and an increase in the hemoglobin (1.89 +/- 0.10 mmol/L to 1.98 +/- 0.19 mmol/L; p < 0.005) after the increase in colchicine dose in 26 patients. CONCLUSION: Subclinical inflammation continues during an attack-free period in FMF patients. A-SAA was the best marker of subclinical inflammation. Patients who are homozygous or compound heterozygotes of MEFV mutations had higher A-SAA levels. An increase in the colchicine dose resulted in a dramatic decrease in A-SAA and an increase in hemoglobin level. These findings favor the use of A-SAA in drug monitoring.

Increased neutrophil apoptosis during attacks of familial Mediterranean fever.

AIM: Apoptosis is a programmed form of cell death. Recently much attention has been devoted to the role of apoptosis in rheumatological diseases. We have aimed to analyze apoptosis in the inflammatory pathway of familial Mediterranean fever (FMF). METHODS: 26 FMF patients and 12 age and sex matched controls were the subject of the study. Twelve of the patients were analyzed during an FMF attack whereas samples were obtained at least a week after an attack in 14. Four of the patients had renal amyloidosis. Whole blood was treated with ammonium chloride for RBC lysis. Subsequently the cells were stained with propidium iodide and annexin. Neutrophils and lymphocytes were gated separately for analysis by flow cytometry. We have also analyzed cellular Fas and Fas-ligand expression in these cells. RESULTS: The mean age of the patients was 12.00 +/- 3.17, and was not different than the control subjects. Erythrocyte sedimentation rate and CRP levels were significantly elevated in the attack group as compared to the attack-free group. The mean levels of neutrophil apoptosis in the FMF patients with an attack, attack-free and controls were 12.94 +/- 11.78, 6.60 +/- 7.83 and 3.98 +/- 4.27, respectively. Lymphocyte apoptosis in the same groups were 7.84 +/- 8.63, 2.75 +/- 2.33, and 1.22 +/- 0.93, respectively. Neutrophil and monocyte apoptosis was significantly increased during the attack as compared to the controls (p < 0.05). However lymphocyte apoptosis was not different between the aforementioned groups. On the other hand, lymphocyte apoptosis was significantly increased in the SLE patients (p < 0.05), whereas neutrophil apoptosis was not. Fas staining of neutrophils were not different between the groups (p > 0.05). On the other hand the difference between the groups for FasL was significant (p < 0.05). CONCLUSION: Neutrophil and monocyte but not lymphocyte apoptosis was significantly increased during FMF attacks reminding us that FMF is an autoinflammation of certain peripheral cells. The increased apoptosis in these patients maybe regarded as a response to clear the unwanted inflammatory cells. On the other hand the increased apoptosis maybe the explanation of the self-limited nature of the FMF attacks. Future studies will enlighten us on the significance of this increased apoptosis in the process of inflammation.

Thrombomodulin, tissue plasminogen activator and plasminogen activator inhibitor-1 in Henoch-Schönlein purpura.

OBJECTIVE: To evaluate the changes in the plasma levels of thrombomodulin (TM), tissue-type plasminogen activator (t-PA) and plasminogen activator inhibitor-1 (PA1-1) as indices of endothelial injury/activation and fibrinolysis in childhood Henoch-Schönlein purpura (HSP). METHODS: Twenty-six children with HSP aged between 4-15 years and 10 healthy controls were included in the study. Blood samples were taken from these patients at admission and 6-12 weeks after healing of skin rash and arthritis. Plasma levels of TM, t-PA and PAI-1 activities and t-PA and PAI-1 antigen (Ag) levels were measured. RESULTS: The plasma levels of TM, t-PA Ag and PAI-1 Ag in patients during the acute phase were significantly different from the controls. The difference in TM between the acute phase and recovery in patients was also significant. The decrease in plasma levels of t-PA Ag and PAI-1 Ag in patients between the acute and recovery phases was not significant. t-PA activity was significantly higher in the acute phase than in the recovery phase. CONCLUSION: We suggest that increased levels of TM, t-PA, and PAI-1 activity may reflect the presence of endothelial injury and/or activation and fibrinolytic activation in patients with HSP.

Survey of Turkish systemic lupus erythematosus patients for a particular mutation of C1Q deficiency.

OBJECTIVE: Hereditary C1q deficiency is a rare disease and up to now only 41 cases have been reported. Since all but 3 cases developed SLE or SLE-like disease, C1q deficiency represents the most powerful disease susceptibility gene identified for the development of SLE in humans. A molecular defect in homozygous C1q deficiency has been identified in 13 families. Four of these families are Turkish in origin and they all share the same mutation which is a CAG to TAG change at codon 186 in the A chain. This led us to investigate whether this mutation might be found in Turkish SLE patients and whether it could cause increased disease susceptibility when expressed in the heterozygous form. METHODS: We screened 65 Turkish lupus patients and 49 healthy Turkish individuals by carrying out an amplification of exon 2 of the A chain and restriction enzyme analysis for the C1qA mutation. RESULTS: We found no other example of this mutation in either the homozygous or heterozygous forms. CONCLUSION: C1q deficiency is one of the very strong disease susceptibility genes in lupus and may cause SLE via a critical role in the physiological clearance of apoptotic cells. However, C1q deficiency caused by a particular mutation in the A chain in a heterozygous form is not found in the Turkish SLE population.

Optic neuropathy in primary antiphospholipid syndrome in childhood.

Neurologic symptoms associated with antiphospholipid antibodies in children include thrombotic events, unilateral movement disorders, or migraine. We present a 7-year-old girl with bilateral optic neuropathy, cerebral white-matter lesions, and antiphospholipid IgM that responded to prednisone and tended to relapse when it was stopped. Remission was obtained under maintenance corticosteroid therapy, and the antiphospholipid antibodies disappeared. This case suggests a role for antiphospholipid antibodies in the pathogenesis of optic neuropathy in childhood.

Carbamazepine induced systemic lupus erythematosus. Another warning.

Carbamazepine induced systemic lupus erythematosus is a very rare phenomenon. Seven cases have been reported so far. We report another case documented with both clinical and serologic data and discuss some possible variations in related serology.

Cranial nerve involvement in childhood polyarteritis nodosa.

Amongst a variety of neurological manifestations of childhood polyarteritis nodosa, cranial nerve involvement is unusual. We report 4 cases with cranial nerve palsies in a series of 36 biopsy-proven patients. Two cases presented with IIIrd nerve palsy alone, one with right IIIrd and left IVth nerve palsy, and one with peripheral VIIth nerve paresis. All 4 patients showed good response to prednisolone and cyclophosphamide treatment. Cranial nerve involvement in childhood polyarteritis nodosa seems not so rare when patients are followed on long term basis.

Plasma lipids and lipoproteins in juvenile chronic arthritis.

Altered levels of high density lipoprotein (HDL), low density lipoprotein (LDL), and very-low density lipoprotein (VLDL), as well as apolipoproteins have been previously described in rheumatoid arthritis patients. We have attempted to evaluate the serum triglyceride, total cholesterol, cholesterol in DHL, LDL, apolipoprotein A1 (apo-A1) and apolipoprotein B (apo-B) levels in juvenile chronic arthritis (JCA) and to correlate them with CRP and ESR in the active and non-active stages of JCA. A total of 37 children no fulfilled ARA criteria for the diagnosis of JCA were studied. There were 18 girls and 19 boys. Age range was 2.5-16 years with a mean of 9.5. The mean duration of disease was 1.8 years. Nineteen patients were accepted to have active disease. Eighteen age and sex matched healthy children served as controls. Apo-A1 was significantly lower in the active JCA group when compared to inactive patients and healthy controls (both p < 0.05). There were significant inverse correlations between apo-A1 and both ESR and CRP levels in these patients (r = 0.67, p < 0.05 and r = -0.61, p < 0.-05, respectively). Although mean LDL levels were numerically lower in the JCA patients (67.2 mg/dl in the active and 68.6 mg/dl in the inactive patients) the difference with healthy controls (91.7 mg/dl) was not statistically significant. There was no significant differences in regard to triglyceride, total cholesterol, cholesterol in HDL, and apo-B levels between neither of the groups. We conclude that JCA patients have a dyslipoproteinaemic state with already altered metabolism of lipids at different stages of the chronic inflammation from active to inactive disease.