Predictors of Beta-Hexachlorocyclohexane blood levels among people living close to a chemical plant and an illegal dumping site.

BACKGROUND: Hexachlorocyclohexane is a synthetic chemical with several isomers, including ß-Hexachlorocyclohexane (ß-HCH). In 2005, a large contamination of crude milk from some bovine farms along the Sacco River (Central Italy) was detected; it was related to the illegal disposal of large quantities of processing waste by a chemical industry of the area. A biomonitoring study, conducted in 2007 on a sample of the residing population, found high values of ß-HCH in people living close to the river. These results led to the establishment of a clinical and epidemiological surveillance program on all the exposed population. The aim of the study was to evaluate the determinants of ß-HCH blood levels in people living within 1 Km of the Sacco River, focusing on the role of specific foods, body mass index and risk factors not yet identified. METHODS: The program involved all people living within 1 km of the river. A descriptive analysis of ß-HCH blood levels was done in relation to the potential determinants including specific foods. Regression analysis was used to study the association between potential determinants and (natural log) ß-HCH haematic concentration. The results were expressed as geometric mean ratios (GMR). To take into account similarities within the families we adjusted for family clustering. RESULTS: A total of 602 subjects (87.2%) agreed to participate in the surveillance. The ß-HCH geometric mean serum concentration was 72 ng/g lipid. The regression analysis showed that being female (GMR: 1.32, 95%CI: 1.14-1.53), elderly (GMR> 70yy: 10.04, 95%CI: 6.65-15.15), obese (GMR: 1.63, 95%CI: 1.28-2.08), eating food of local/own production (GMR 1.47, 95%CI: 1.15-1.88) and using water from private wells (GMRdrink:1.47, 95%CI: 1.00-2.14 and GMRwash: 1.48, 95%CI: 1.17-1.87) were associated with higher ß-HCH values. There was inverse association with breastfeeding (GMR: 0.64, 95%CI: 0.47-0.86). The focus on specific foods showed that the most important factors were eggs and beef. CONCLUSIONS: The study indicated a greater contamination for older people, and those drinking and washing with water from private wells and consuming locally produced food, especially eggs and beef.

The Hidradenitis suppurativa patient journey in Italy: current status, unmet needs and opportunities.

BACKGROUND: Hidradenitis suppurativa (HS) is a chronic, progressive inflammatory, debilitating, follicular disease of the skin with a reported incidence of less than 1% in the general population. Diagnosis of HS may typically be delayed for years, and even when diagnosed, is challenging to treat. Thus, HS has considerable negative impact on patient quality of life. OBJECTIVES: To describe the HS patient journey in Italy, identify unmet needs and provide a proposal for integrated patient care. METHODS: A multidisciplinary panel of leading Italian experts met to evaluate routine clinical practice and patient experience, and analyse the available scientific evidence on HS. RESULTS: A comprehensive map of the journey of the HS patient in Italy was built based on integrated data obtained from clinical practice and patient experience. The journey is non-linear and can be grouped into four broad stages that are somewhat overlapping and intersecting: (i) onset to diagnosis; (ii) circle of visits; (iii) circle of treatments; (iv) living with HS. Several unmet needs were identified that included greater awareness of disease by both healthcare practitioners and patients. CONCLUSION: Improved disease awareness can be addressed through continuing medical education for physicians and development of educational materials for patients, in addition to greater utilization of social networks. Moreover, the development of integrated treatment centres was considered a worthwhile goal, and would offer patients the possibility to receive multidisciplinary care, ideally with dermatologists coordinating treatment, along with surgeons, psychologists, endocrinologists, gastroenterologists, pain specialists, gynaecologists and paediatricians as needed. Lastly, standardized management protocols for HS are also needed.

Differences in clinicopathological features and distribution of risk factors in Italian melanoma patients.

BACKGROUND: No studies are available in the literature on the distribution of different melanoma features and risk factors in the Italian geographical areas. OBJECTIVE: To identify the differences in clinical-pathological features of melanoma, the distribution of risk factors and sun exposure in various Italian macro-areas. METHODS: Multicentric-observational study involving 1,472 melanoma cases (713 north, 345 centre, 414 south) from 26 referral centres belonging to the Italian Multidisciplinary Group for Melanoma. RESULTS: Melanoma patients in northern regions are younger, with thinner melanoma, multiple primaries, lower-intermediate phototype and higher counts of naevi with respect to southern patients; detection of a primary was mostly connected with a physician examination, while relatives were more involved in the south. Northern patients reported a more frequent use of sunbeds and occurrence of sunburns before melanoma despite sunscreen use and a lower sun exposure during the central hours of the day. CONCLUSIONS: The understanding of differences in risk factors distribution could represent the basis for tailored prevention programmes.

Matter of margins.

BACKGROUND: Basal cell carcinoma (BCC) is the most common cancer, it represents a significant economic burden to health services because of a large volume of affected patients. Surgical excision with histological assessment of the surgical margins is widely considered as the mainstay of BCC treatment. Incomplete removal, in fact, should be considered a poor prognostic indicator, as incomplete removal of lesions is at risk of local recurrence. Actually, dermatological surgeries are carried out by a variety of different types of practitioners, such as plastic surgeons, maxillofacial surgeons, otorhinolaryngologists, ophthalmologists and finally dermatologists. Incomplete removal of the tumour ranges from 6.3% to 25%, depending on the improper intra-operative evaluation of the extent of the tumour. It depends on the clinical knowledge derived from both training and daily experience. In this sense, the majority of the largest studies derive from plastic surgeons, while dermatologists have small case series, albeit with a higher therapeutic efficacy in terms of complete surgical excision. OBJECTIVES: We conducted a retrospective analysis of the surgical activity, more specifically we evaluated both our therapeutic accuracy and analyzed the prognostic factors related to incomplete excisions. METHODS: A retrospective review of all BCC removals was performed. A total of 4523 BCC removals were included; other neoplasm, benign lesions and biopsies were also excluded. Each BCC's size diameter, localization, histology and histological presence of complicating factors was assessed, then the percentage of the incomplete removal was calculated. RESULTS: Incomplete resections occurred in 225 (4.97%) BCCs of the cases. Thirteen areas were categorized into in three different levels that rank the risk of incomplete removals. Sub-analysis indicates that just over a third had no complicating factors with the lateral/deep margins. The most frequent complicating factor is ulceration (22.9%), while vascular invasion or seborrheic keratoses were not found. Actinic keratoses, scabs and scars held the most responsibility for the involvement of the lateral margins, while perineural invasion is the main factor leading to deep margin involvement. Finally, a different trend for the involvement of lateral or deep margins according different histological sub-types was highlighted; lateral involvement is more frequent for the infiltrative/morpheic type, while the deep margin is more involved in the nodular type.

Approach in glucocorticoid-induced osteoporosis prevention: results from the Italian multicenter observational EGEO study.

Glucocorticoid-induced osteoporosis (GIO) is the most frequent cause of secondary osteoporosis. GIO is linked to glucocorticoids (GC) daily assumption with maximum effect within first months of treatment and decreasing to basal levels as the therapy is discontinued. In Italy, primary prevention of GIO is suggested when GC therapy (prednisone >5 mg/day or equivalent) is taken for longer than 3 months. Lazio GISMO (Italian Group for Study and Diagnosis of Bone Metabolism Diseases) group organized the GC and Osteoporosis Epidemiology study (EGEO) to evaluate physician's approach in preventing GIO. The study involved 19 osteoporosis centers. Patients taking long-term GC therapy were recruited and information collected: medical history and anthropometric data, GC therapy, primary disease, physician's specialty, osteopororosis screening, and pharmacological intervention. The study included 1334 patients. Mean age was 63 ± 13 yr; 243 (18%) patients had a history of falls from standing position in the previous 12 months, 78 (35%) vertebral fractures, 91 (41%) fractures other than vertebral, 27 (12%) femoral fractures, and 27 (12%) multiple sites fractures. The molecules of GC more often prescribed were prednisone and 6-metil prednisolone. One thousand and forty patients (78%) were taking GC for more than 6 months. GC therapy was prescribed more frequently by rheumatologists (62%). Antiosteoporotic drugs for GIO prevention were prescribed in 431 patients (32%). Among the patients, only 27% (360) received calcium and vitamin D supplements, and 39% (319) treated by rheumatologists received anti-resorptive drugs. In conclusion, our data show that in Italy, as already described elsewhere, only a small subpopulation of GC-treated patients was supported by an anti-osteoporotic therapy, indicating the need to further stimulate awareness of both patients and specialists, prescribing GC therapy, to an appropriate and prompt GIO prevention.

Photodynamic therapy for basal cell carcinoma: clinical and pathological determinants of response.

BACKGROUND: Photodynamic therapy (PDT) is increasingly used in the treatment of basal cell carcinoma (BCC). However, scant information is available about the impact of both patient- and lesion-related characteristics on the effectiveness of therapy. Therefore, on the basis of the current data, it is difficult to draw clear-cut indications to use PDT for treatment of BCC in clinical practice. OBJECTIVE: To investigate the clinical and pathological determinants of response of BCC to PDT with methylaminolevulinate (MAL) and red light. METHODS: The clinical and pathological characteristics of 194 BCCs in 135 patients, treated with MAL-PDT, were evaluated. Lesions were treated with MAL-PDT according to established methods and the response was assessed by clinical follow-up of the patients. RESULTS: Complete response to PDT was 62%, with a better response for superficial BCC (95/116, 82%) than nodular BCC (26/78, 33%). When determinants of response were analysed, the nodular type and the location on the limbs emerged as significant clinical predictors of failure. Among the pathological characteristics, the nodular and infiltrative histotypes, as well as ulceration and tumour thickness were associated with a lower response to therapy. Patients' age and gender, as well as the size of the lesions, were not found to be significant predictors. CONCLUSIONS: Optimization of PDT procedure for BCC requires a careful selection of the lesions. In particular, superficial BCCs, preferentially located on the trunk, show the best therapeutic response.

Anti-tumor necrosis factor alpha therapy normalizes fibrinolysis impairment in patients with active rheumatoid arthritis.

OBJECTIVES: Rheumatoid arthritis (RA) is associated with increased cardiovascular risk and involvement of inflammation, coagulation and fibrinolysis. Treatment with infliximab, a tumour necrosis factor-alpha (TNF-alpha) blocking chimeric monoclonal antibody, induces a long-term reduction of inflammation and coagulation, but its effect on fibrinolysis is still unknown. We carried out an observational study investigating plasma biomarkers of inflammation and fibrinolysis in RA patients before and after 14 weeks of infliximab treatment given according to the therapeutic guidelines for RA. METHODS: We studied 20 selected patients with active RA and without any other atherosclerosis risk factor as well as 40 healthy controls. Patients, treated with a stable dose of methotrexate, received infliximab (3 mg/kg) at week 0, 2, 6 and 14. At week 0 and 14, we assessed clinical, inflammatory and fibrinolyitic parameters. RESULTS: At baseline, plasminogen activator inhibitor (PAI-1) antigen, PAI-1 activity and tissue-type plasminogen activator (t-PA) antigen were significantly higher in RA patients than in controls (p=0.01, p=0.001 and p=0.0001 respectively). After 14 weeks of infliximab treatment, the levels of PAI-1 antigen, PAI-1 activity and t-PA antigen significantly decreased till normalization (p=0.0001). Plasma levels of C reactive protein (CRP) and interleukin-6 (IL-6) were directly correlated with levels of PAI-1 antigen (p=0.011 and p=0.0001), PAI-1 activity (p=0.013 and p=0.027) and t-PA antigen (p=0.017 and p=0.040). CONCLUSIONS: This study provides evidence that TNF-alpha blockade by infliximab not only decreases inflammation, but also reduces the inhibition of fibrinolysis. Such a combined effect may be pivotal in reducing the whole thrombotic risk in these patients.

Focus on adverse events of tumour necrosis factor alpha blockade in juvenile idiopathic arthritis in an open monocentric long-term prospective study of 163 patients.

OBJECTIVE: To report adverse events (AEs) seen in a large cohort of patients with juvenile idiopathic arthritis (JIA) treated with tumour necrosis factor (TNF)alpha blockers (infliximab and etanercept). METHODS: All patients with JIA treated with infliximab or etanercept at the Paediatric Rheumatologic Centre of the G Pini Institute (Milan, Italy) from November 1999 to February 2006, were enrolled in an open, single-centre, long-term prospective study RESULTS: In all, 163 patients (68 infliximab, 95 etanercept) were enrolled. Mean (SD) age of onset was 6.4 (4.8) years, mean age 17.1 (9.2) years, mean therapy duration 22.9 (17.6) months. A total of 45 patients (32 infliximab, 13 etanercept) failed to respond to or did not tolerate the first therapy and switched to a second one. In all, 208 treatments (81 infliximab, 127 etanercept) were performed. A total of 71 AEs occurred in 51 (62.9%) patients on infliximab and led to discontinuation in 26 (32.1%); 133 AEs occurred in 69 (54.3%) patients on etanercept and led to discontinuation in 18 (14.2%). Some AEs, such as thrombocytopoenia, neuropsychiatric disorders, new onset of Crohn disease and new onset or flare-up of chronic iridocyclitis (CIC), are unusual and have rarely been described before, yet proved to be significant in frequency and/or clinically noteworthy in the large population we followed. CONCLUSIONS: In our 6-year study, anti-TNFalpha agents infliximab and etanercept were well tolerated and safe, and were associated with only few serious, but all reversible, AEs. However, such inhibitors are associated with various and numerous AEs. Children and young adults affected by JIA should be carefully monitored so as to limit the risk of AEs during anti-TNFalpha therapy as much as possible.

[Nailfold capillaroscopic report: qualitative and quantitative methods]. / La refertazione dell'esame capillaroscopico: metodi di valutazione qualitativi e quantitativi.

Nailfold capillaroscopy (NVC) is a simple and non-invasive method used for the assessment of patients with Raynaud's phenomenon (RP) and in the differential diagnosis of various connective tissue diseases. The scleroderma pattern abnormalities (giant capillaries, haemorrages and/or avascular areas) have a positive predictive value for the development of scleroderma spectrum disorders. Thus, an analytical approach to nailfold capillaroscopy can be useful in quantitatively and reproducibly recording various parameters. We developed a new method to assess patients with RP that is capable of predicting the 5-year transition from isolated RP to RP secondary to scleroderma spectrum disorders. This model is a weighted combination of different capillaroscopic parameters (giant capillaries, microhaemorrages, number of capillaries) that allows physicians to stratify RP patients easily using a relatively simple diagram to deduce prognosis.

[Skeletal mineralization in a prepubertal female population affected by juvenile idiopathic arthritis]. / Mineralizzazione scheletrica in una popolazione femminile prepubere affetta da artrite idiopatica giovanile.

BACKGROUND: Puberty is an essential step in bone mass accrual. Growth failure and impairment of sexual maturation are frequent manifestations of chronic illnesses in the paediatric population, and chronic rheumatologic disorders such as juvenile idiopathic arthritis (JIA) are no exception to this. METHODS: The aim of our study was to prospectively evaluate bone density in adolescent females with JIA, and to correlate the results with clinical variables, in particular with age at menarche. Lumbar spine (L2-L4) area bone mineral density (aBMD) (assessed by Dual X-ray Absorbiometry, DXA) was monitored every 6-12 months in a group of 38 girls with JIA. The evaluated bone density accrual during the peripubertal time as well as absolute and relative (Z-score) aBMD in relationship with age at menarche, JIA subset, disease activity (as evaluated by ESR and Hgb), corticosteroid and methotrexate treatment (mean pro kg daily dose, cumulative dose) was assessed. Height, body mass index (BMI), bone mass content (BMC) values were also collected. Volumetric BMD (vBMD) evaluated with a geometric correction formula has been calculated and compared to aBMD. RESULTS: Patients were divided into two groups: - group I included girls with menarche age within normal limits for Italian standards; - group II included girls with delayed menarche. The BMD values and Z scores in group I were not significantly different to normal population. The BMD values and Z scores in group II were significantly decreased when compared to the normal population (p<0.001). With a multivariate analysis only age at menarche seemed independently related to peripubertal mineralization (p=0.025, r between -0.65 and -0.75). With a binary logistic analysis only disease activity (ESR and Hgb values) seems independently related to a menarche delay (1.24+/-0.4 for each mm/h). CONCLUSION: Our data show a critical role for disease activity in determination of a regular pubertal onset and an optimal bone density achievement.

[Biological therapy with TNF-inhibitors in pediatric rheumatology. Review of the literature and personal experience]. / Le terapie biologiche con inibitori del TNF in reumatologia pediatrica. Revisione della letteratura ed esperienza personale.

The therapeutic approach to JIA is sometimes very troublesome and progression to erosive polyarthritis may occur in all JIA categories. Only Methotrexate has shown efficacy and safety in a large controlled trial. Nevertheless, in many cases, drug resistance or intolerance has led to try other therapeutic options, with still debatable results. Therefore, there has been space, in the last few years, for new therapies as the TNF-inhibitors. This therapeutic approach has shown a dramatic clinical benefit in active polyarticular refractory JIA: the rate and rapidity of response have exceeded those of all other studied DMARDs. Preliminary data show that they are efficacious also for other pediatric rheumatic disease (spondyloarthropathies, autoimmune uveitis, dermatomyositis, Kawasaki syndrome and some autoinflammatory diseases). TNF-inhibitors in JIA have demonstrated a favourable benefit-to-risk profile. However, as their use has increased worldwide, some unusual, usually not serious, adverse events have emerged. Severe infections, including TB, and deaths have been reported. Long-lasting active disease, systemic disease, concurrent and previous immunosuppressive therapies, all contribute to risk of infection and other serious AEs. Given the evidence that TNF has a primary role in the pathogenesis of JIA, particularly in joint destruction, neutralizing this cytokine early, within the window of opportunity, could halt or delay progression of joint damage and debilitating consequences of the disease. Thus, for JIA patients whose disease is not quickly controlled with MTX, TNF blockers may be considered as first-line treatment, although long-term safety data still need to be established.

[Simultaneous ultrasonography and arthroscopy for the study of the joint environment: indications and limits]. / Ecografia e artroscopia in contemporanea per lo studio dell'ambiente articolare: indicazioni e limiti.

Arthroscopy is a mini-invasive technique that allows the direct observation of the joint cavity and the execution of diagnostic and therapeutic procedures; arthroscopy needs a very long learning-time curve as well as dedicated spaces and instruments. Ultrasonography is an imaging technique that enables to perform an immediate extension of the standard physical examination. The opportunity to visualize soft tissues, to obtain multiplanar and dynamic images in real time makes this practice easy repeatable at low costs. Ultrasonography allows to detect a variety of changes during inflammatory processes. The wide experience in arthroscopy of rheumatic patients acquired through the years by our team at the G. Pini Institute led us to study in vivo, during arthroscopy, the correspondence between arthroscopic and ultrasonographic images. Up to now three knee arthroscopies have been conducted with the double equipment (ultrasonographic and arthroscopic devices) in operating room. In our experience, the combination of the two methods in operating room may improve the validation of ultrasonography with arthroscopy as gold standard, helps to train the ultrasonographer to give immediate answers in order to clear the doubts aroused by ultrasonographic images; it also allows the arthroscopist to visualize the deeper layers of the synovial membrane making double guided targeted biopsies possible. Limits are the complexity of the procedure (instruments, operators, spaces, training of the doctors), the loose of power-doppler signal with the blood tourniquet and the always difficult evaluation of cartilage.

[Long-term evaluation of infliximab in the treatment of persistently active juvenile idiopathic arthritis refractory to conventional therapy]. / Valutazione a lungo termine di infliximab nel trattamento dell'artrite idiopatica giovanile persistentemente attiva e refrattaria alle terapie convenzionali.

OBJECTIVES: To evaluate, in long-term open label prospective study, infliximab as therapeutic choice for Juvenile Idiopathic Arthritis (JIA) non responsive to conventional therapy. METHODS: We enrolled to treat with infliximab 78 JIA patients (66 females, 12 males): the mean age was 20.7+/-7.1 years (median 20.9, range 5.4-34.9); mean JIA duration was 13.6+/-7.6 years (median 13.5, range 0.4-31.4). Infliximab, at dose of 3-10 mg/kg/infusion added to weekly subcutaneous Methotrexate or other previous DMARDs, was administered by intravenous infusions at weeks 0, 2, 6 and every 8 weeks thereafter. Chest X-ray, Mantoux's test, electrocardiogram were performed at baseline; laboratory tests and clinical evaluation were performed at each infusion. Response was evaluated according to ACR improvement criteria. RESULTS: Mean treatment period was 21.6 months+/-18.8 (median 14.7, range 1.4-72.4). Just after first infusion most of patients reported significant improvement in pain, fatigue, morning stiffness. Infliximab is still successfully administered to 23 patients (29.5%); 55 (70.5%) patients suspended because of: inefficacy (7), infusion reactions (17), adverse events (9), disease flare-up after a period of effectiveness on synovitis, pain, and morning stiffness (19), remission (2), lack of compliance to treatment (1). Infusion reactions, like dyspnea, flushing, chills, headache, hypotension, anxiety, throat oedema, were observed in 29 patients (34.5%). Anti-DNA antibodies were present in 7 patients (none developed Systemic Lupus Erythematous). CONCLUSIONS: Infliximab showed impressive effectiveness treating refractory JIA, although most of patients had to discontinue treatment because of disease flare-up or adverse events. Infliximab may represent a good therapeutic choice in patients non-responders to Methotrexate.

Akinetic versus dyskinetic postinfarction scar: relation to surgical outcome in patients undergoing endoventricular circular patch plasty repair.

OBJECTIVES: This retrospective study attempted to relate surgical outcome with the extent and type of preoperative wall motion asynergy in patients with postinfarction myocardial scar who underwent endoventricular circular patch plasty repair and associated coronary grafting. BACKGROUND: Left ventricular (LV) pump function improvement is difficult to predict after aneurysmectomy, for either akinetic or dyskinetic scar, and previous studies have reported that the absence of paradoxic systolic motion correlates with higher operative mortality and no improvement in pump function. METHODS: Two hundred forty-five patients who underwent endoventricular circular patch plasty repair and associated coronary grafting were retrospectively selected if they had technically adequate right and left anterior LV angiograms before the operation. All had right and left cardiac catheterization. The centerline method was applied to preoperative right anterior oblique LV angiography to assess the absolute motion of the chords and the percent length of the perimeter showing a fractional shortening <2 SD from the normal mean value (extent of asynergy ([A%]). RESULTS: The overall perioperative mortality rate was 6%; 120 patients had akinetic and 125 had dyskinetic scar, and no differences were found among the groups in terms of all the clinical and hemodynamic variables collected in the study. Patients with a large scar (A% >60), either akinetic or dyskinetic, had a higher perioperative mortality rate (12%) than patients with a small scar (2.2%). After the operation, the ejection fraction (EF) increased from 36 +/- 13% to 50 +/- 13% (mean +/- SD), and pulmonary pressures significantly decreased. End-diastolic volume decreased from 199 +/- 75 to 89 +/- 36 ml/m2. Patients with a large akinetic scar had the most severely impaired preoperative function (largest ventricular volumes and highest pulmonary mean pressure); nevertheless, they had an impressive improvement in function (EF from 25 +/- 9% to 41 +/- 12%), not different from that observed with large dyskinetic scarring (EF from 26 +/- 7% to 46 +/- 11%). CONCLUSIONS: Surgical outcome of endoventricular circular patch plasty repair for postinfarction myocardial scar relates to the extent of LV asynergy rather than to the presence or absence of dyskinesia. Patients with a large akinetic scar and severely depressed pump function benefit from a relatively simple surgical procedure previously reserved only for dyskinetic aneurysm. The reduction of wall tension and oxygen demand, owing to the marked decrease of volumes, and the increase in oxygen supply, owing to revascularization, may play a major role in improving pump function.

Capillaroscopic observations in childhood rheumatic diseases and healthy controls.

OBJECTIVE: To describe, by using video nailfold capillaroscopy (NFC), microvascular abnormalities in children with rheumatic diseases and to evaluate the capillary changes over a follow up period. METHODS: 118 children suffering from rheumatic diseases: 55 juvenile idiopathic arthritis (JIA), 7 mixed connective tissue disease (MCTD), 6 primary Raynaud's phenomenon (PRP), 34 systemic lupus erythematosus (SLE), 8 juvenile systemic sclerosis (JSSc) and 8 juvenile dermatomyositis (JDM) were included in the study. Patients with major capillaries abnormalities or scleroderma pattern were followed up for at least 12 months. 70 age- and sex-matched healthy controls (HC) were also examined. RESULTS: In HC there was a significant correlation between age and capillary length (p = 0.001). JIA patients showed capillary number, size, shape and arrangement similar to HC. Minor abnormalities were frequently observed. The percentage of major abnormalities were significantly increased compared to HC in MCTD (p = 0.008), SLE (p = 0.0002) and JDM patients (p < 0.0001). 5/8 of JSSc had a scleroderma pattern from the onset of the disease. The serial observations in connective tissue diseases also showed that the evolution of capillaroscopic pattern was not unidirectional. In fact, in some nailfolds there was an increase in capillary loss and in avascular areas, whereas sometimes it remained stable on repeated examination. CONCLUSION: NFC can be used as a simple, inexpensive, non-invasive method to evaluate the microvascular abnormalities in childhood rheumatic conditions, and it may be useful in early recognition and monitoring scleroderma spectrum disorders.