The Relationship Between 99mTc-Pertechnetate Hand Perfusion Scintigraphy and Nailfold Capillaroscopy in Systemic Sclerosis Patients: A Pilot Study.

OBJECTIVES: This study aims to assess the possible relationship between 99mTc-pertechnetate hand perfusion scintigraphy (HPS) and nailfold capillaroscopy (NC) in systemic sclerosis (SSc) patients. PATIENTS AND METHODS: The study group consisted of 25 SSc patients (6 males; 19 females; mean age 54.2±9.7 years; range, 32 to 67 years), 18 female patients with primary Raynaud's phenomenon (PRP) (mean age 47.1±9.5 years; range, 34 to 65 years) and 10 healthy individuals (3 males, 7 females; mean age 52.7±12.6 years; range, 37 to 73 years). NC and 99mTc-pertechnetate HPS were performed in all examinees. The capillaroscopic findings were classified as normal or scleroderma pattern ("early", "active", or "late"). The fingers-to-palm ratios were calculated for both blood flow (BF) and blood pool (BP) phases of the 99mTc-pertechnetate HPS. RESULTS: Systemic sclerosis patients showed a significantly lower BP ratio than PRP patients and healthy subjects (p=0.004). No statistically significant difference was observed between the SSc and PRP patients in respect to BF ratio. A gradual decrease of BF and BP with the severity of NC microangiopathy pattern ("early", "active" or "late") was found in SSc patients, while the differences were not statistically significant. Patients with diffuse SSc showed lower BF and higher BP than those with limited SSc, while these differences were without statistical significance. There was no significant correlation between BF or BP values and type of SSc (limited or diffuse) (p=0.77 versus p=0.54, respectively) as well as three microangiopathy patterns (p=0.22 versus p=0.54, respectively). CONCLUSION: 99mTc-pertechnetate HPS improves the evaluation of vascular damage in SSc patients. There is no direct relationship between NC and 99mTc-pertechnetate HPS; however, the two methods complement each other in the assessment of microcirculation in SSc.

Long-term effects of immunosuppressive therapy on lung function in scleroderma patients.

The study aims to analyze the effects of induction treatment with cyclophosphamide (CYC) pulse therapy followed by maintenance treatment with other mild immunosuppressive agents on lung function in scleroderma (SSc) patients. Thirty patients with SSc (mean age 52 years, mean disease duration < 2 years) with forced vital capacity (FVC) ≤ 80% and/or diffusing capacity of carbon monoxide (DLco) ≤ 70% were included. Monthly CYC pulses were given for 6 months (induction treatment), followed by 3-monthly maintenance pulses for the next 18 months, and during the next 5 years patients received other mild immunosupressive therapy brought by the competent rheumatologist. The efficacy was evaluated by comparing FVC% and DLco% after 6, 24, and 84 months from the baseline. All patients completed induction and maintenance treatment with CYC. Three patients were lost to follow-up. The rest of 27 patients, during the next 5 years, received other immunosupressive agents (14 azathioprine, 9 methotrexate, and 4 mycophenolate mofetil). Three patients died in the 4 years of follow-up. By 6, 24, and 84 months, the mean FVC and DLco changes were + 0.47 and + 2.10, + 3.30 and - 2.49, and + 1.53 and - 3.76%, respectively. These changes were not significantly different from the baseline values. CYC does not appear to result in clinically significant improvement of pulmonary function but fulfilled criteria of stable disease. Maintenance treatment with other mild immunosupressive agents preserves the benefits achieved during CYC treatment.

Matrix Metalloproteinases-3 Baseline Serum Levels in Early Rheumatoid Arthritis Patients without Initial Radiographic Changes: A Two-Year Ultrasonographic Study.

OBJECTIVE: To investigate the association of high baseline serum levels of metalloproteinases-3 (MMP-3) with structural damage to hand and feet joints, assessed by ultrasonography (US), in patients with early, treatment-naïve rheumatoid arthritis (RA), without initial X-ray-visible erosions, during 24 months follow-up. METHODS: Sixty-three early RA (European League Against Rheumatism/American College of Rheumatology 2010), disease-modifying anti-rheumatic drugs/glucocorticoid naïve patients (mean age 53.4 ± 14.1) with symptom duration ≤12 months, had baseline serum levels of MMP-3 tested. OMERACT US group definition was used to detect the presence, as well as longitudinal diameter of erosions by US at study entry and after 24 months, at the level of wrists, metacarpophalangeal (MCP2/MCP5) joints of both hands, and fifth metatarsophalangeal joints. RESULTS: Complete data were collected from 52 out of 63 patients. High baseline serum levels of MMP-3 (MMP-3-positive) were found in 46/63 patients. 122 bone erosions in total (1.9 bone erosions/patients) were detected by US at baseline visit and 213 erosions (4.3/patients) after 24 months. MMP-3 positive patients had significantly higher total number of erosions than MMP-3-negative (p = 0.039) and higher increase in size of bone erosions in the feet but not in the hand joints after follow-up (OR 4.82 [1.23-18.9], p = 0.024; OR 1.17 [0.320-4.26], p = 0.816 respectively). CONCLUSION: After 2 years of follow-up, US assessment showed a higher number of new bone erosions in MMP-3-positive compared to MMP-3-negative patients with early RA and no visible initial radiographic changes. High baseline levels of MMP-3 predict significantly higher structural damage progression at the level of feet, but not at the level of hand joints.

Cross-cultural validation of the Modified Falls Efficacy Scale in Serbian community-dwelling women at risk for osteoporotic fracture.

OBJECTIVE: Cross-cultural validation of the Serbian version of the Modified Falls Efficacy Scale (MFES). METHODS: This cross-sectional study involved 257 women aged 65 years and above who were referred for dual-energy x-ray absorptiometry examination at the Railway Healthcare Institute in Belgrade, Serbia, between January and April 2016. Data collection comprised of a sociodemographic questionnaire and Geriatric Depression Scale-Short Form (GDS-SF) questionnaire, and data related to fractures, level of physical activity, use of medications that can increase the risk of falls, and frequency of falls in the past 12 months. None of the study participants had been previously treated for osteoporosis. The internal consistency of the questionnaire items was assessed via Cronbach's alpha, whereas the interclass correlation coefficient (ICC) was used to calculate test-retest reliability based on the sample of 257 women. We also evaluated concurrent, convergent, and construct validity. RESULTS: Cronbach's alpha for the total assay score was 0.98. Correlations among the items ranged from 0.84 to 0.93. While ICC for the scale as a whole was 0.99 (95% confidence interval 0.98-0.99), ICC pertaining to individual items ranged from 0.82 to 0.99. Concurrent validity analysis revealed a significant positive correlation between MFES scores and the reported level of physical activity (ρ = 0.34; P < 0.01). Convergent validity was tested through the ratio of MFES and sociodemographic variables. The findings indicated presence of a significant negative correlation between the MFES scores and age (ρ = -0.32; P < 0.01), age of menopause onset (ρ = -0.16; P = 0.01), and GDS-SF scores (ρ = -0.12; P = 0.04), and positive correlation between MFES and the level of social activity (ρ = 0.22; P < 0.01). Significant differences were noted between the MFES scores of participants who had no history fractures and those who did (U = 5277.50; P < 0.01), and between scores of women who reported falling in the past 12 months and those who did not (U = 4968.50; P < 0.01). Similarly, significant differences (P < 0.01) in the scores pertaining to each MFES item were observed between women who had experienced falls in the past (n = 101) and those who had not (n = 156). CONCLUSION: The Serbian version of the MFES is a reliable and valid instrument that can be used in both clinical practice and research to describe and measure self-perceived fear of falling in older individuals.

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.

Adaptation and validation of the Rheumatoid Arthritis Quality of Life (RAQoL) questionnaire for use in Serbia.

Rheumatoid arthritis (RA) is one of the most prevalent inflammatory rheumatic diseases. As it is a chronic and a lifelong destructive disease, the aim of the treatment is to reduce disability and improve quality of life. The Rheumatoid Arthritis Quality of Life (RAQoL) questionnaire is a patient-reported outcome measure, specific to RA. To adapt and validate the RAQoL for use in Serbia, two translation panels were involved to produce the Serbian RAQoL. After successful translation, face and content validity was determined via cognitive debriefing interviews. The psychometric properties of the questionnaire were examined, including reliability and construct validity, by using the Nottingham Health Profile (NHP) as a comparator scale. The RAQoL was translated successfully and rated as applicable, relevant and comprehensive by respondents. The questionnaire had high internal consistency (alpha = 0.94 at both time points) and test-retest reliability (r = 0.92). Moderately high correlations were found between the RAQoL and physical mobility, pain and energy level sections of the NHP, providing evidence of convergent validity. The RAQoL was able to distinguish between patients grouped by perceived general health, incidence of flare-up and disease severity. The Serbian language version of the RAQoL showed strong evidence of reliability and validity and is recommended for use in clinical trials and routine general practice in RA.

Diagnosis of Raynaud's phenomenon by 99mTc-pertechnetate hand perfusion scintigraphy: a pilot study.

We assess the usefulness of 99mTc-pertechnetate hand perfusion scintigraphy in patients with Raynaud's phenomenon (RP). The study population consisted of 18 patients with primary RP, 25 patients with secondary RP within systemic sclerosis (SSc), and ten healthy individuals. Gamma camera dynamic first-pass study during the first 60 s 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. 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 79% and a specificity of 70% when using cutoff values of 0.37 for blood pool. 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 separates primary from secondary RP.

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.

Fatal sulfasalazine-induced eosinophilic myocarditis in a patient with periodic fever syndrome.

OBJECTIVE: The aim of this paper is to report the first case of drug-induced eosinophilic myocarditis (EM) in a patient with hereditary periodic fever syndrome (PFS). CASE: A 28-year-old man with hyper-IgD syndrome, one of the PFS, developed a sulfasalazine-induced systemic hypersensitivity reaction complicated by EM. Thirteen days after sulfasalazine introduction, which had been given for arthritis, the patient developed fever, facial/neck edema, rash and cardiogenic shock, and died within 8 h. The autopsy revealed hemophagocytosis, while acute heart failure caused by necrotizing EM was established as the cause of death. CONCLUSION: This was a case of drug-induced EM in a patient with PFS that had an atypical presentation, rapid evolution and poor outcome.

Lower extremity lipedema, upper extremity lipodystrophy and severe calcinosis complicating juvenile dermatomyositis.

Juvenile dermatomyositis (JDM) is a rare but complex and potentially life-threatening autoimmune disease of childhood. Significant proportions of patients have residual weakness, muscle atrophy, joint contractures, and calcinosis. Recently, new clinical findings, such as lipodystrophy accompanied with increased fat deposition in certain areas, have been reported. So far, it is not known whether the redistribution of body fat may be the type of lipedema of lower extremity. We describe a 39-year-old woman who was diagnosed with JDM at the age of 7. Later she developed symmetrical lipodystrophy of upper extremities and symmetrical lipedema of lower extremities (making 2 and 58.3 % of total body fat mass, respectively), with multiple calcified nodules in the subcutaneous tissues. These nodules gradually increased in size despite therapy. Capillaroscopy findings showed scleroderma-like abnormalities. ANA and anti-U1RNP antibodies were positive. Similar cases with simultaneous occurrence of the lipedema of lower extremities, lipodystrophy of upper extremities, and severe calcinosis complicating JDM have not been published so far. We showed that the calcinosis and lipodystrophy were associated with short duration of active disease. Also, we display case that raises the question whether it is possible overlapping autoimmune diseases revealed during follow-up.

The value of pattern capillary changes and antibodies to predict the development of systemic sclerosis in patients with primary Raynaud's phenomenon.

The aim of this study is to assess the prognostic value of major provisional criteria for the development of systemic sclerosis (SSc) in primary Raynaud's phenomenon (RP) patients. We retrospectively studied the chart of 497 patients with primary RP in whom anticentromere (ACA) and antitopoisomerase I (ATA) antibodies tests and a capillary reading were available. Sensitivity, specificity, positive predictive value, negative predictive value, positive likelihood ratios (LHR+), negative likelihood ratios (LHR-), odds ratio (OR), and area under the receiver operating characteristics curve (AUC) of those criteria were assessed to predict the development of SSc. During the average follow-up of 2.3 ± 1.9 years, 159 (32 %) patients evolved to SSc, 245 (49.3 %) evolved to other connective tissue diseases, and 93 (18.7 %) patients did not progress. The SSc pattern predicted SSc satisfactorily (LHR+ 4.12, LHR- 0.07, OR 63, AUC 0.819; P < 0.001). ACA were not significantly associated with the development of SSc (LHR+ 1.19, LHR- 0.9, OR 1.32, AUC 0.538, P = 0.156). ATA were significantly associated with the development of SSc (LHR+ 9.32, LHR- 0.67, OR 15.13, AUC 0.777; P < 0.001). Both SSc pattern and ACA or ATA were significantly associated with the development of SSc (LHR+ 2.98, LHR- 0.70, OR 4.2, AUC 0.674; P < 0.001 vs. LHR+ 16, LHR- 0.68, OR 24, AUC 0.819; P < 0.001, respectively). SSc pattern or ATA as independent risk factors, as well as following two parameters together (SSc pattern and ATA or SSc pattern and ACA) were good predictors for the development of SSc.

Is there a difference in systemic lupus erythematosus with and without Raynaud's phenomenon?

The aim of this study was to assess the association between Raynaud's phenomenon (RP) and specific capillaroscopic findings in patients with SLE and particular clinical manifestations of the disease. A total of 79 patients with SLE were included in the study: 44 of them (43 women) with RP and 35 (32 women) age-, sex-, and disease-duration-matched patients with SLE without RP. Demographic variables, clinical manifestations, laboratory and nailfold capillaroscopy findings were compared between the two groups. Central nervous systemic involvements (P = 0.0038) and peripheral neuropathy (P = 0.0336) were significantly more common in SLE patients with RP, while secondary Sjögren's syndrome (P = 0.0363) was more common in SLE patients without RP. RP occurred in 52 % of patients before SLE onset while 48 % of patients developed RP after they had been diagnosed with SLE. Arthritis/arthralgia (P = 0.0073) was significantly more common in patients who had been diagnosed with RP before the onset of SLE, while mucosal ulcers were more common in patients who contracted RP after the onset of SLE (P = 0.0258). Enlarged capillaries (P = 0.0482), presence of avascular areas (P = 0.0476), capillary hemorrhages (P = 0.0482), and granular blood flow (P = 0.0482) were more common in patients with SLE who also suffered from RP, than in patients with SLE without RP. The frequency of normal (63.6 vs. 82.9 %, P = 0.100) and nonspecific (25 vs. 17.1 %, P = 0.5696) capillaroscopy findings were similar in either groups. Scleroderma-like pattern of capillaroscopy finding was only found in patients with RP [(11.4 %), P = 0.0482]. RP in our patients with SLE was associated with specific clinical manifestations, indicating that prognostic relevance of RP in SLE should be evaluated.

Late appearance and exacerbation of primary Raynaud's phenomenon attacks can predict future development of connective tissue disease: a retrospective chart review of 3,035 patients.

To assess the prognostic value of the age at onset of Raynaud's (RP) and of a history of exacerbation of RP attacks for the development of connective tissue disease (CTD) in patients initially found to have primary Raynaud's. 3,035 patients with primary RP (2,702 women and 333 men) were followed for an average of 4.8 years (range from 1 to 10 years). At baseline and every 6 months, they were screened for signs and symptoms of CTD. At 4.8 years of follow-up, 54.7 % patients remained as primary RP, 8.1 % had developed suspected secondary RP, and 37.2 % had developed a definite CTD. Primary RP patients had an earlier onset of RP (mean age of 32.2 years) than those with suspected (mean age 36.5 years, P = .007) or definite secondary RP associated with CTD (mean age of 39.8 years, P = .004). RP beginning before the age of forty was not significantly associated with the development of CTD. Conversely, the appearance of RP after the age of 40 was significantly associated with the development of CTD (P = .00001). Worsening of RP attacks predicted the development of CTD, especially systemic sclerosis (relative risk [RR] of 1.42), scleroderma overlap syndrome (RR of 1.18), and mixed CTD (RR of 1.18). Patients whose onset of RP occurred past 40 years of age and those with worsening RP attacks were at risk for the future development of CTD.

The relationship among hypertension, antihypertensive medications, and osteoporosis: a narrative review.

Osteoporosis and hypertension are two frequent diseases among the aging population that share a similar etiopathology and often coexist. Moreover, treatment of hypertension affects bone mineral density and, therefore, can worsen osteoporosis. This narrative review considers the influence of the main etiologic factors that contribute to the development of hypertension and osteoporosis and examines the effect of the most often used antihypertensives on bones. A computerized literature search of relevant English publications regarding the etiology of hypertension and osteoporosis as well as the impact of antihypertensives on osteoporosis from 1996 to 2011 was completed in October 2011. The latest update in the search was performed from May to June 2012. The most relevant nongenetic factors in the etiology of osteoporosis and hypertension are low calcium intake, vitamin D and vitamin K deficiency, high consumption of sodium salt, and the effects of different forms of nitric oxide. Thiazide diuretics are the only antihypertensives that have a positive influence on bone mineral density. For other antihypertensive drugs, the data are conflicting, indicating that they may have a potentially negative or positive influence on bone mineral density and fracture risk reduction. Some studies did not find a correlation between the use of antihypertensives and bone mineral density. Due to the frequent coexistence of hypertension and osteoporosis, when selecting long-term antihypertensive therapy the potential effects of antihypertensive drugs on development, worsening, or improvement of osteoporosis should also be considered.

Validation of the osteoporosis quality of life questionnaire QUALEFFO-41 for the Serbian population.

BACKGROUND: Vertebral fractures could lead to reduced physical, social and mental functioning, and loss of personal independence. Therefore, during the treatment of osteoporosis, it has become necessary to examine the changes in everyday functioning, well-being and health related quality of life (HRQOL). To that effect, this study aims to translate, culturally adapt, and validate the Serbian version of Quality of Life Questionnaire of the European Foundation for Osteoporosis (QUALEFFO-41) for patients with vertebral fractures. METHODS: Nine female patients with osteoporosis participated in the pre-validation study. A validation, case-control study included two groups of female patients: one that consisted of 50 female patients with osteoporosis, and with at least one vertebral fracture, and another one that consisted of 50 control patients with osteoporosis but without fractures. They completed the QUALEFFO-41 and the EuroQol group questionnaire with five dimensions (EQ-5D) twice within a month. The validation study examined internal consistency, concurrent validity, test-retest reliability, sensitivity and specificity. RESULTS: During the pre-validation study, three of the items in the QUALEFFO-41 were slightly changed. Afterwards, during the validation study, the statistically significant differences (adjusted for: age, duration of menopause, current employment and marital status) in the mean values of all domains and total scores between the groups were noted. For the case group, the internal consistency of the QUALEFFO-41 domains and of total questionnaire was above 0.70. The test-retest reliability was tested by the intraclass correlation coefficients (ICC) that were in range 0.87 - 0.96 for the case, and 0.15 - 0.83 for the control group. Correlations between the total scores of the QUALEFFO-41 and the EQ-5D health state value, for both groups were negative and statistically significant (r = -0.78, p<0.001 and r = -0.73, p<0.001, respectively). The QUALEFFO-41 had a better prediction of the value of HRQOL of cases compared to the generic questionnaire EQ-5D (the AUC difference was 0.099, p = 0.013). CONCLUSIONS: The Serbian QUALEFFO-41 version is reliable, valid, sensitive and predictive for examinations of HRQOL in patients with prevalent vertebral fractures and can be used in further studies.

Systemic lupus erythematosus progressing to non-Hodgkin's lymphoma complicated by fatal hemophagocytic syndrome: case report.

Hemophagocytic syndrome (HPS) may be provoked by infections, malignancies and autoimmune diseases. We report on a 56-year-old woman with long-lasting systemic lupus erythematosus (SLE) who presented with malar rash, inflammatory livedo reticularis, fever, weight loss, pancytopenia and mild splenomegaly with cervical lymphadenopathy. She had criteria for SLE flare-up (malar rash, high antinuclear antibody titer, complement consumption, pathological urinary sediment, and retinal vasculitis). Despite high-dose glucocorticoid therapy, pancytopenia and fever worsened. Important elevations of triglycerides and ferritin were also found. Bone marrow aspirate demonstrated hemophagocytosis, which confirmed the coexistence of HPS and SLE. The treatment with glucocorticoids, immunoglobulins, cyclophosphamide, filgrastim and antimicrobial therapy was unsuccessful. After one month, the patient developed Pneumocystis jirovecii pneumonia with fatal outcome. Bone marrow biopsy, taken 5 days before death, showed high grade diffuse large B-cell (CD20+, Ki-67+) non-Hodgkin's lymphoma (DLBCL). We are the first to report the association of both SLE and non-Hodgkin's lymphoma complicated by HPS. We showed that, based on clinical and laboratory data, it was difficult to distinguish the early phase of HPS from SLE flare-up and new-onset DLBCL. Therapy of such a complex case of HPS has not been standardized, and opportunistic infections remain a difficult issue.

Development of a shorter version of the osteoporosis knowledge assessment tool.

The aim of the authors of this study was to develop a short version of the Osteoporosis Knowledge Assessment Tool to be used for the target population of young adult Serbian females as an easily implemented add-on questionnaire. The 20-item Osteoporosis Knowledge Assessment Tool was translated and culturally adapted using the Principles of Good Practice for the Translation and Cultural Adaptation Process for Patient-Reported Outcomes Measures. The validation study was conducted on a sample of 250 female students studying at the Faculty of Pharmacy at the University of Belgrade, during a two-month period (November-December 2010). The difficulty index, item-total correlations, and internal consistency were calculated first. Afterward, confirmatory factor analysis was applied to test the structure of the Osteoporosis Knowledge Assessment Tool models and develop a short version. The mean total Osteoporosis Knowledge Assessment Tool score was 8.31 (possible range 0-20). The confirmatory factor analysis fit indices indicated poor fit of the data to the originally hypothesized structure. The confirmatory factor analysis fit indices, difficulty indices, and content validity allowed trimming of the original model and development of a short version with nine items. The average chi-square value for the Osteoporosis Knowledge Assessment Tool short version was 31.79 (p = 0.240, SE = 0.176) with Bollen-Stine bootstrap p = 0.249, Tucker-Lewis Index = 0.925, Comparative Fit Index = 0.944 and Root Mean Square Error of Approximation = 0.027. The Osteoporosis Knowledge Assessment Tool thus had acceptable characteristics and may be used for osteoporosis knowledge assessment.

Scleroderma pattern of nailfold capillary changes as predictive value for the development of a connective tissue disease: a follow-up study of 3,029 patients with primary Raynaud's phenomenon.

To assess the prognostic value of scleroderma pattern of nailfold capillary changes for the development of connective tissue diseases (CTD) in subjects with primary Raynaud's phenomenon (RP). The study included 3,029 consecutive patients with primary RP who had been followed at 6-month intervals during the mean of 4.8 years. The pathological features of nailfold capillaroscopy were recorded in all patients who had neither clinical nor serological signs of a CTD. In patients who developed CTD, capillary changes obtained 6 months prior to diagnosis were analyzed. A possible relationship between capillary changes and the presence of associated CTD was assessed. At the end of follow-up, 1,660 (54,8%) patients have still the primary RP, 246 (8,1%) had suspected secondary RP, and 1,123 (37,1%) patients developed CTD (363 undifferentiated CTD, 263 systemic sclerosis, 143 systemic lupus erythematosus, 106 rheumatoid arthritis, 102 Sjögren's syndrome, 61 overlap syndrome, 30 vasculitides, 24 mixed CTD, 19 polymyositis, 7 dermatomyositis, and 5 primary antiphospholipid syndrome). Scleroderma pattern were significantly associated with the development of systemic sclerosis [P = .00001, sensitivity 94%, specificity 92%, positive predictive value 52%, negative predictive value 99%, and odds ratio 163 (95% CI, 97,9-271,5)], as well as dermatomyositis (P = .0004), overlap syndrome with signs of systemic sclerosis (P = .0001), and mixed connective tissue disease (P = .007). Capillary microscopy is effective method for differentiation between primary and secondary RP and useful tool for the prediction of scleroderma spectrum disorders in RP patients.