Slavica Pavlov-Dolijanovic

The prognostic value of nailfold capillary changes for the development of connective tissue disease in children and adolescents with primary raynaud phenomenon: a follow-up study of 250 patients.

Abstract:  To assess the prognostic value of capillaroscopy findings for the development of connective tissue disease in children and adolescents with Raynaud phenomenon, we followed up a group of 250 (mean age 15 years) for 1 to 6 years after the first capillaroscopy was performed. Every 6 months they were screened for signs and symptoms of connective tissue disease. Analysis was performed on capillary changes registered 6 months before the development of connective tissue disease. Capillary changes were classified into three types: normal, nonspecific, and sclerodermatous. At the end of the follow‐up period, 191 (76%) subjects had primary Raynaud phenomenon, 27 (10.8%) were diagnosed as having undifferentiated connective tissue disease, and 32 (12.8%) fulfilled the criteria for a diagnosis of a specific connective tissue disease. Systemic lupus erythematosus was found in nine (3.6%) patients, rheumatoid arthritis in 10 (4%) patients (six of them with juvenile onset rheumatoid arthritis), and scleroderma spectrum disorders in 13 (5.2%). The mean time for the evolution of Raynaud phenomenon into undifferentiated connective tissue disease or a form of the disease was 2 years. Most of the subjects with primary Raynaud phenomenon (173/191, 91%), undifferentiated connective tissue disease (22/27, 81%), juvenile onset rheumatoid arthritis/rheumatoid arthritis (7/10, 70%), and systemic lupus erythematosus (6/9, 67%) had normal capillary findings. Nonspecific capillary changes occurred in 3 of 10 (30%) patients with rheumatoid arthritis, 2 of 9 (22%) with systemic lupus erythematosus, 4 of 27 (15%) with undifferentiated connective tissue disease, and 18 of 191 (9%) with primary Raynaud phenomenon. Of all the subjects, only 10 (4%) showed sclerodermatous disease type capillary changes 6 months before the expression of a particular disease: eight (62%) of these developed scleroderma spectrum disorders, one expressed systemic lupus erythematosus, and one had undifferentiated connective tissue disease. We concluded that there were no specific capillary changes predictive for future development of systemic lupus erythematosus, juvenile onset rheumatoid arthritis/rheumatoid arthritis, and undifferentiated connective tissue disease in children and adolescents with Raynaud phenomenon. Most of our study subjects with Raynaud phenomenon who developed these diseases had normal capillary findings or nonspecific changes. Children and adolescents who developed scleroderma spectrum disorders showed a sclerodermatous type of capillary changes 6 months before the expression of the disease, indicating that this type of capillary changes in children and adolescents with Raynaud phenomenon highly correlated with further development of scleroderma spectrum disorders.

Women with silicone breast implants and autoimmune inflammatory syndrome induced by adjuvants: description of three patients and a critical review of the literature

Silicone has been widely used in the manufacture of medical implants. It is well tolerated in most cases. However, in this paper we report the cases of three women who developed autoimmune/inflammatory syndrome induced by adjuvants (ASIA syndrome), namely with silicone breast implants. The symptoms in these cases include arthralgia, arthritis, myalgia, sleep disturbances, the appearance of autoantibodies, miscarriage, Raynaud’s phenomenon, and involvement of autoimmune diseases (scleroderma and undifferentiated connective tissue diseases). In one patient, breast implants were removed, but no improvement was seen after the removal. The remaining two patients received the updated information about their condition, and they decided not to remove the implants. In conclusion, earlier reports that silicone is biologically relatively inert have recently been challenged with the description of ASIA syndrome.

Profile of rheumatology patients willing to report adverse drug reactions: bias from selective reporting

Background Adverse drug reactions (ADRs) have a significant impact on human health and health care costs. The aims of our study were to determine the profile of rheumatology patients willing to report ADRs and to identify bias in such a reporting system. Methods Semi-intensive ADRs reporting system was used in our study. Patients willing to participate (N=261) completed the questionnaire designed for the purpose of the study at the hospital admission. They were subsequently classified into two groups according to their ability to identify whether they had experienced ADRs during the previous month. Group 1 included 214 out of 261 patients who were able to identify ADRs, and group 2 consisted of 43 out of 261 patients who were not able to identify ADRs in their recent medical history. Results Group 1 patients were more significantly aware of their diagnosis than the patients from group 2. Marginal significance was found between rheumatology patients with and without neurological comorbidities regarding their awareness of ADRs. The majority of patients reported ADRs of cytotoxic drugs. The most reported ADRs were moderate gastrointestinal discomforts. Conclusion We may draw a profile of rheumatological patients willing to report ADRs: 1) The majority of them suffer from systemic inflammatory diseases and are slightly more prone to neurological comorbidities. 2) They are predominantly aware of their diagnosis but less able to identify the drugs that may cause their ADRs. 3) They tend to report mainly moderate gastrointestinal ADRs; that is, other cohorts of patients and other types of ADRs remain mainly undetected in such a reporting, which could represent a bias. Counseling and education of patients as well as developing a network for online communication might improve patients’ reporting of potential ADRs.

Multiple Major and Minor Anomalies Associated With Klippel-Feil Syndrome: A Case Report

Klippel-Feil syndrome is defined as congenital fusion of two or more cervical vertebrae. In this article, we report a 55-year-old male patient with one-year history of neck pain, headaches, and one episode of syncope after a severe trauma. X-rays and magnetic resonance imaging of cervical spine revealed fused vertebral bodies of C2-C5. The major anomalies associated with Klippel-Feil syndrome (small stature, thoracic kyphoscoliosis, lumbar scoliosis, restricted opening mouth, and bilateral sensorineural hearing loss) as well as multiple minor anomalies (mild face asymmetry, high arched palate, rhinoscoliosis, high nasal bridge, inclined septi nasi, and thin upper lip) were detected. This is a rare case describing the anomalies of the nose in Klippel-Feil syndrome patients. Our patient had no central cord impairment following a severe trauma.

SAT0630 Sensitivity and Specificity 99mTc-Pertechnetate Hand Perfusion Scintigraphy in Patients with Raynaud's Phenomenon

Objectives The aim of this study was to assess sensitivity and specificity of 99mTc-pertechnetate hand perfusion scintigraphy in patients with Raynauds phenomenon (RP). Methods The study population consisted of 10 healthy individuals (mean age 57 years), 18 patients with primary RP (mean age 48 years) and 25 patients with secondary RP within systemic sclerosis (SSc) (mean age 54 years). Gamma-camera dynamic first-pass study during the first 60 sec and a static scintigraphy after 5 min were recorded following a bolus injection of 99mTc-pertechnetate via a cubital vein. Regions of interest were drawn on the summed images around the fingers and the palmar region. The fingers-to-palm ratios were then calculated from the total counts inside these regions of interest separately for each hand. Results The mean fingers-to-palm ratio for dynamic study (blood flow) was 0,58±0,19 for the healthy group, 0,45±0,18 for the primary RP and 0,43±0,21 for the SSc patients. The mean fingers-to-palm ratio for static study (blood pool) was 0,44±0.06 for the healthy group, 0,42±0,06 for the primary RP and 0,36±0,07 for the SSc patients. Analysis of variance showed these differences to be significant (p=0.039 from blood flow and p=0,004 from blood pool). The receiver operating characteristic curve showed sensitivity of 80% and a specificity of 60% when using cutoff values of 0.40 for blood flow and sensitivity of 72% and a specificity of 73% when using cutoff values of 0.40 for blood pool. Conclusions Our method is able to differentiate between patients with normal and those with abnormal microcirculation of the hands. Dynamic study separates the healthy subjects from patients with RP, while static study separating primary from secondary RP. References Csiki Z, Galuska L, Garai I, Szabό N, Varga J, András C, Zeher M. Raynauds syndrome: comparison of late and early onset forms using hand perfusion scintigraphy. Rheumatol Int. 2006 Sep;26(11):1014-8. Csiki Z, Garai I, Varga J, Szücs G, Galajda Z, András C, Zeher M, Galuska L. Microcirculation of the fingers in Raynauds syndrome: (99m)Tc-DTPA imaging. Nuklearmedizin. 2005 Feb;44(1):29-32. Disclosure of Interest None declared

A case of myositis with immunological background associated with statin use

Statins might cause and/or aggravate the immune-mediated myositis in patients on long-term, stable treatment. We provide a case of polymyositis with an immunological background and gastrointestinal and urinary manifestations in patient on long-term, stable atorvastatin treatment for the past six years. The diagnose of polymyositis was established based on clinical symptoms and signs, electromyography and laboratory test results (elevated aspartate aminotransferase 279 U/L, reference range 0–40 U/L; alanine aminotransferase 198 U/L, 0–33 U/L; lactate dehydrogenase 2200 U/L, 103-227 U/L; creatine kinase 7820 U/L, 15–84 U/L; and positive antinuclear antibodies test, titer of 1:160, with suspect antisynthetase antibodies). Polymyositis was probably related to atorvastatin treatment (Naranjo score, 5). Other probable causes of the myositis were rejected. Coricosteroid therapy, methotrexate and supplementation with vitamin D did not improve the condition. The patient remained bedridden and died two months after the hospital discharge due to the acute myocardial infarction.

OP0248 The prognostic value of scleroderma pattern capillary changes, aticentromere antibodies and anti-topo I antibodies for the development of very early systemic sclerosis - a follow-up study of 497 patients with raynaud’s phenomenon

Background Provisional major criteria for the diagnosis of very early systemic sclerosis (VESSc) proposed of EULAR Scleroderma Trial and Research Group (EUSTAR) are Raynaud’s phenomenon (RP), positive autoantibodies (antinuclear, anticentromere-ACA, antitopoisomerase I- ATA) and scleroderma pattern of nailfold capillary changes (SPNCC)1. However, reports about prognostic value of this antibodies in diagnosis of VESSc are inconclusive. Objectives To assess the prognostic value of SPNCC, ACA and ATA for the development of VESSc in patients (pts) with primary RP. Methods From the group of 3029 pts with primary RP who were prospectively followed from 1996 to 20061, separated a group of 497 pts in whom were identified ACA and ATA by indirect imunofluorescence. Results ACA i ATA were categorized as positive or negative. Nailfold capillary changes were classified as normal, nonspecific and scleroderma pattern. Sensitivity (Sn), specificity (Sp), positive predictive value (PPV), negative predictive value (NPV),Odds ratio (OR) and area under the ROC curve (AU-ROC) of SPNCC, ACA i ATA testing for the diagnosis of VESSc was assesed. Results At the end of follow up period 20.72% pts have still the primary RP, 13.3% had suspected secondary RP, 31,9% developed SSc, 15.3% undifferentiated CTD (connective tissue disease), 5.8% Sjögren’s syndrome, 4.6% overlap syndrome, 3.0% systemic lupus erythematosus, 1.8% rheumatoid arthritis, 1.8% mixed CTD, less of 1% pts developed vasculitides and polymyositis. The SPNCC had 95% pts with SSc, and 23% pts with other CTDs. SPNCC were significantly associated with future development of SSc (p=0.0000) with Sn 95%, Sp 77%, PPV 66%, NPV 97%, OR 63, AU-ROC 0.819. ACA were detected in 41% pts with SSc, and 34% pts with other CTDs. ACA were not significantly associated with future development of SSc (p=0.187) with Sn 41%, Sp 66%, PPV 36%, NPV 70%, OR 1.32, AU-ROC 0.538. ATA were detected in 36% pts with SSc, and 4% pts with other CTDs. ATA significantly associated with future development of SSc (p=0.0000) with Sn 36%, Sp 96%, PPV 81%, NPV 76%, OR 13.9, AU-ROC 0.783. Both parameters together SPNCC-ACA and SPNCC-ATA were significantly associated with future development of SSc (p=0,0001, Sn 40%, Sp 86%, PPV 58%, NPV 75%, OR 4.2, AU-ROC 0.674 v.s. P=0,00001, Sn 33%, Sp 97%, PPV 88%, NPV 76%, OR 24, AU-ROC 0.826 respectively). Conclusions Scleroderma pattern of capillary abnormalities or/and ATA were good predictor of future development of SSc, and important test in pts with RP in order to identify those at higher risk of developing SSc. ACA in patients with RP are not consistently associated with SSc. However, both parameters together (SPNCC and ACA) sugest future development of SSc. References Pavlov-Dolijanovic S, Damjanov N, Stojanovic R, Vujasinovic Stupar N, Stanisavljevic D.Scleroderma pattern of nailfold capillary changes as predictivevalue for the development of a connective tissue disease:a follow-up study of 3,029 patients with primary Raynaud’sphenomenon. Rheumatol Int 2011 DOI 10.1007/s00296-011-2109-2. Disclosure of Interest None Declared