Objective. Although antisynthetase antibodies (ARS) are the established markers of the so-called antisynthetase syndrome (ASSD), in these patients the concomitant positivity of anti-Ro52 antibodies, reported in up to the 50% of cases, is not rare. Several studies focused on the effect of different ARS specificities on the evolution of ASSD, the most recent showing no effects. On the contrary, the role of co-occurring anti-Ro52 antibodies in ASSD is still debated. We investigated the potential of anti-Ro52 antibodies in identifying a clinical phenotype of ASSD or influencing prognosis, irrespectively to the underlying ARS specificity. Methods. Retrospective analysis of clinical, imaging and laboratory characteristics, therapeutic approaches and outcome at baseline and at last followup, of 60 ASSD patients progressively enrolled at our Hospital. Results. We identified 34 anti-Ro+ and 26 anti-Ro-ASSD patients. Classic triad prevalence at baseline was similar between the two groups, whereas interstitial lung disease (ILD) (p-value=0.01) and myositis (p-value=0.03) were significantly more prevalent in anti-Ro52+ and in anti-Ro52-patients at last follow up, respectively. No differences in therapeutic approaches, oxygen need and ILD patterns were observed. Overall mortality was 25% (15 subjects). No differences in mortality, overall and disease related, between anti-Ro52+ and anti-Ro52-patients were observed (p-value=0.764), despite the more frequent ILD occurrence in anti-Ro52+ patients. Survival curves were not different at any time point . Conclusion. Anti-Ro52 antibodies affect time course and clinical characteristics of ASSD. Although ILD is significantly more associated to anti-Ro52antibodies, no difference in mortality was observed compared to anti-Ro52 negative patients.

Objectives. The purpose of this study was to review the frequency and clinical presentation of the rarest clinical manifestations of systemic lupus erythematosus (SLE).Methods. A list of 6 rare SLE manifestations were defined: gastrointestinal, liver, pulmonary, cardiac, ocular and neurological manifestations. Each topic was assigned to a couple of authors to perform a literature search and article review. Results. In total, 149 articles were included in the literature review: 37 for gastrointestinal manifestations, 6 for liver manifestations, 27 for pulmonary manifestations, 50 for cardiac manifestations, 16 for ocular manifestations, 13 for neurological manifestations. Gastrointestinal disorders included several clinical presentations with variable frequency (from 0.5% to 10.7% of the cases); liver involvement included lupusrelated hepatitis (9.3%) and autoimmune hepatitis (2.3%). The rarest pulmonary manifestations identified were shrinking lung syndrome, described in 1.5% of patients, while interstitial lung disease and lupus pneumonia were reported in 4% and 3% of patients, respectively. Myocarditis and pulmonary hypertension were also rarely described in SLE patients although ranging from 0.4-16% and 1-14% respectively, depending on the methodology used for its identification. Ocular manifestations in SLE included some rare manifestations (reported in less than 5% of patients) and lupus retinopathy that is described in 1.2-28.8% of patients depending on methods of ascertainment. Aseptic meningitis and chorea were also confirmed as very rare manifestations being reported in less than 1% and in 0.3-2.4% of cases respectively. Conclusion. The results of this literature review provide the basis for a better understanding of some less-known manifestations of SLE and for stressing the need for a higher awareness in diagnostic and therapeutic protocols regarding these rare disease aspects.

Frozen shoulder is a common and self-limiting condition affecting the soft tissues of the shoulders, characterized by severe pain, impaired range of motion (ROM) and limitation of daily activities. Its prevalence is 5% and it occurs most commonly in the fifth and sixth decades of life; women are more affected [DePalma in Clin Orthop Relat Res 466:552–560, 2008]. It can be idiopathic or associated with other conditions such as metabolic disorders, diabetes, thyroid diseases, prolonged immobilization, trauma [DePalma in Clin Orthop Relat Res 466:552–560, 2008], or complications after vaccine administration known as SIRVA (Shoulder injury related to vaccine administration). SIRVA is not caused by the vaccine itself but by inappropriate vaccination techniques [Martín Arias et al. in Vaccine 35:4870–4876, 2017]. The natural history of the frozen shoulder is a progression through three stages based on clinical and arthroscopic presentations: freezing, frozen and thawing [DePalma in Clin Orthop Relat Res 466:552–560, 2008; Do et al. in Orthop J Sport Med 9:232596712110036, 2021]. The onset is characterized by disabling pain, that worsens at night; it is induced by inflammation and hypervascularity and lasts from 10 to 36 weeks [Do et al. in Orthop J Sport Med 9:232596712110036, 2021]. The second stage is predominated by stiffness and severe reduction of ROM. This phase typically lasts from 9 to 12 months [Do et al. in Orthop J Sport Med 9:232596712110036, 2021]. Eventually, a recovery phase occurs, with a gradual recovery of the ROM that can last between 12 and 42 months. Ultrasound is an emerging diagnostic tool that contributes to differential diagnosis and treatment [Zappia et al. in Insights Imaging 7:365–371, 2016; Ricci et al. in J Ultrasound Med 39:633–635, 2020]: signs of adhesive capsulitis consist of thickening of the inferior recess of the glenohumeral joint capsule, thickening of the coracohumeral ligament and soft tissue structures in the rotator cuff interval, with hypervascularity. An unspecific sign is increased fluid in the tendon sheath of the long head of the biceps [Martín Arias et al. in Vaccine 35:4870–4876, 2017; Tandon et al. in J Ultrasound 20:227–236, 2017].