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Objectives: To investigate pregnancy outcomes in women with autoimmune rheumatic diseases (ARD) in the Italian prospective cohort study P-RHEUM.it., Methods: Pregnant women with different ARD were enrolled for up to 20 gestational weeks in 29 Rheumatology Centres for 5 years (2018-2023). Maternal and infant information were collected in a web-based database., Results: We analysed 866 pregnancies in 851 patients (systemic lupus erythematosus was the most represented disease, 19.6%). Maternal disease flares were observed in 135 (15.6%) pregnancies. 53 (6.1%) pregnancies were induced by assisted reproduction techniques, 61 (7%) ended in miscarriage and 11 (1.3%) underwent elective termination. Obstetrical complications occurred in 261 (30.1%) pregnancies, including 2.3% pre-eclampsia. Two cases of congenital heart block were observed out of 157 pregnancies (1.3%) with anti-Ro/SSA. Regarding treatments, 244 (28.2%) pregnancies were treated with glucocorticoids, 388 (44.8%) with hydroxychloroquine, 85 (9.8%) with conventional synthetic disease-modifying anti-rheumatic drugs and 122 (14.1%) with biological disease-modifying anti-rheumatic drugs. Live births were 794 (91.7%), mostly at term (84.9%); four perinatal deaths (0.5%) occurred. Among 790 newborns, 31 (3.9%) were small-for-gestational-age and 169 (21.4%) had perinatal complications. Exclusive maternal breast feeding was received by 404 (46.7%) neonates. The Edinburgh Postnatal Depression Scale was compiled by 414 women (52.4%); 89 (21.5%) scored positive for emotional distress., Conclusions: Multiple factors including preconception counselling and treat-to-target with pregnancy-compatible medications may have contributed to mitigate disease-related risk factors, yielding limited disease flares, good pregnancy outcomes and frequency of complications which were similar to the Italian general obstetric population. Disease-specific issues need to be further addressed to plan preventative measures., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Pregnancy requires a special management in women with inflammatory rheumatic diseases (RDs), with the aim of controlling maternal disease activity and avoiding fetal complications. Despite the heterogeneous course of RDs during pregnancy, their impact on pregnancy largely relates to the extent of active inflammation at the time of conception. Therefore, accurate evaluation of disease activity is crucial for the best management of pregnant patients. Nevertheless, there are limitations in using conventional measures of disease activity in pregnancy, as some items included in these instruments can be biased by symptoms or by physiological changes related to pregnancy and the pregnancy itself may influence laboratory parameters used to assess disease activity. This article aims to summarize the current literature about the available instruments to measure disease activity during pregnancy in RDs. Systemic lupus erythematosus is the only disease with instruments that have been modified to account for several adaptations which might interfere with the attribution of signs or symptoms to disease activity during pregnancy. No modified-pregnancy indices exist for women affected by other RDs, but standard indices have been applied to pregnant patients. The current body of knowledge shows that the physiologic changes that occur during pregnancy need to be either adapted from existing instruments or developed to improve the management of pregnant women with RDs. Standardized instruments to assess disease activity during pregnancy would be helpful not only for clinical practice but also for research purposes., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Objectives: Poor information on long-term outcomes and costs on tumour necrosis factor (TNF) inhibitors in psoriatic arthritis (PsA) are available. Our aim was to evaluate long-term costs and benefits of TNF- inhibitors in PsA patients with inadequate response to conventional treatment with traditional disease-modifying anti-rheumatic drugs (tDMARDs)., Methods: Fifty-five out of 107 enrolled patients included in the study at one year, completed the 5-year follow-up period. These patients were enrolled in 8 of 9 centres included in the study at one year. Patients aged older than 18 years, with different forms of PsA and failure or intolerance to tDMARDs therapy were treated with anti-TNF agents. Information on resource use, health-related quality of life (HRQoL), disease activity, function and laboratory values were collected at baseline and through the 5 years of therapy. Costs (expressed in Euro 2011) and utility (measured by EQ-5D instrument) before TNF inhibitor therapy and after 1 and 5 years were compared., Results: The majority of patients (46 out of 55; 83.6%) had a predominant or exclusive peripheral arthritis and 16.4% had predominant or exclusive axial involvement. There was a statistically significant improvement of the most important clinical variables after 1 year of follow-up. These improvements were maintained also after 5 years. The direct costs increased by approximately €800 per patient-month after 1 year, the indirect costs decreased by €100 and the overall costs increased by more than €700 per patient-month due to the cost of TNF inhibitor therapy. Costs at 5 year were similar to the costs at 1 year. The HRQoL parameters showed the same trends of the clinical variables. EQ-5D VAS, EQ-5D utility and SF-36 PCS score showed a significant improvement after 1 year, maintained at 5 years. SF-36 MCS showed an improvement only at 5 years., Conclusions: The results of our study suggest that TNF blockers have long-term efficacy. The higher cost of TNF inhibitor therapy was balanced by a significant improvement of HRQoL, stable at 5 years of follow-up. Our results need to be confirmed in larger samples of patients.

Objectives: To establish how many children with HLA B27-positive juvenile undifferentiated spondyloarthritis (JuSpA) living in southern Italy develop axial disease after 5 years of disease., Methods: All children with B27-positive enthesitis-related arthritis (ERA) consecutively seen in a 7-year period were entered in a special register and were followed prospectively. Each patient was examined at 6-month intervals, even if asymptomatic. In patients with inflammatory spinal pain and/or buttock pain, MRI of the sacroiliac joints and spine was performed. Five years after inclusion, sacroiliac joint plain radiographs were obtained and read blindly after being mixed with those of control subjects., Results: Thirteen children, 9 boys and 4 girls, with B27-positive ERA and one girl with B27-positive isolated SpA dactylitis were seen in the study period. Their median age at disease onset and at our first examination were 10 (range 2-16) and 12 years (range 3-16), respectively. During follow-up, only one patient had axial symptoms, i.e. alternate buttock pain. MRI revealed moderate bone oedema at both sacroiliac joints. After five years of disease, no patient showed reduced spinal movement. No sign of sacroiliitis was seen in any patient and control on plain films. A new MRI of the sacroiliac joints of the patient who showed bone oedema in the first years of disease was normal., Conclusions: This study confirms that the onset of axial involvement in Italian Caucasian HLA-B27 positive children with ERA is rare in the first five years of disease.

In about 20% of patients with psoriatic arthritis (PsA) the rheumatological manifestations precede the onset of the cutaneous lesions. If there is a family history of psoriasis these patients are diagnosed as having psoriatic arthritis sine psoriasis. In the past, they were also classified among patients with undifferentiated spondyloarthritis. The clinical spectrum of PsA sine psoriasis is wide and identified by dactylitis and/or distal interphalangeal arthritis, HLA-Cw6, and a family history of psoriasis. The ClASiffication of Psoriatic ARthritis (CASPAR) criteria of PsA include PsA sine psoriasis.

Objective: To determine the sensitivity of the CASPAR criteria in patients with early psoriatic arthritis (PsA)., Methods: Consecutive patients with a clinical diagnosis of PsA and a disease duration < 12 months were enrolled for study. The proportion of patients meeting the criteria (i.e., the sensitivity) was determined., Results: Forty-four patients with early PsA (23 women, 21 men; mean age 51 yrs, range 16-90) were enrolled. Mean disease duration (+/- SD) was 15.8 +/- 14.3 weeks (range 0.1-50.9 wks). Thirty-four patients satisfied the criteria at the first visit (sensitivity 77.3%). Most patients met the skin and laboratory criterion, i.e., they were rheumatoid factor-negative, while only 2 satisfied the radiologic criterion., Conclusion: Our findings suggest a less satisfactory performance of the CASPAR criteria when applied in early PsA. Lower sensitivity could mainly depend on the small proportion of patients fulfilling the radiologic criterion.

Objective: To evaluate costs, benefits and cost-effectiveness of anti-TNF agents in PsA patients with inadequate response to conventional treatment., Methods: A total of 107 patients, from nine Italian rheumatology centres, with different forms of PsA were given anti-TNF treatment, mainly etanercept (87%). Information on resource use, health-related quality of life, disease activity, function and laboratory values were collected at baseline and through out the 12 months of therapy. Cost (expressed in euro 2007) and utility (measured by EuroQol) before and after anti-TNF therapy initiation were compared in order to estimate the incremental cost per quality-adjusted life year (QALY) gained, and cost-effectiveness acceptability curve was calculated., Results: At the end of 12 months, there was a significant increase in direct cost due to an increase of drug cost caused by TNF inhibitors that was only partially offset by the decrease in indirect cost. In the last 6 months of therapy, the direct cost increased by euro5052, the cost for the National Health System (NHS) by euro5044 and the social cost by euro4638. However, a gain of 0.12 QALY resulted in a cost per QALY gained of euro40 876 for the NHS and of euro37 591 for the society. The acceptability curve showed that there would be a 97% likelihood that anti-TNF therapy would be considered cost-effective at willingness-to-pay threshold of euro60 000 per QALY gained., Conclusion: Cost-effectiveness ratios are within the commonly accepted willingness-to-pay threshold. These results need to be confirmed in larger samples of patients.

Objectives: To describe a case-series of patients who presented with the typical postural abnormalities of long-standing advanced ankylosing spondylitis (AS) but were instead found to suffer from diffuse idiopathic skeletal hyperostosis (DISH)., Methods: We enrolled consecutive patients who showed postural abnormalities, which at first suggested to us the diagnosis of long-standing advanced AS, although the diagnostic process led us to the correct diagnosis of DISH. Each patient had a complete physical examination and radiographs of the spine and pelvis, and was investigated for HLA-B27 locus typing., Results: From 15 June 1998 to 15 June 2006, 15 patients with DISH were seen who presented with the typical postural abnormalities of long-standing advanced AS. All patients were males with a median age of 69 yrs (range 51-91). All lacked HLA-B27 and denied personal or family history of spondyloarthritis. All measurements assessing cervical, thoracic and lumbar spinal movement were abnormal., Conclusions: Patients suffering from DISH can occasionally have severe limitations of spinal mobility, along with postural abnormalities that resemble long-standing advanced AS. Thus, the differential diagnosis between DISH and advanced AS is not limited to the radiological findings and can also extend to the clinical findings in the two diseases, as is highlighted by our report.