Background. Pulmonary involvement in systemic lupus erythematosus (SLE) is a heterogeneous and usually non-severe complication, affecting 50–70% of patients (and up to 4–5% at disease onset). Pleuro-parenchymal manifestations include pleuritis (30%), interstitial lung disease (ILD, 1–15%), and lupus pneumonitis (1–4%), the latter often requiring differential diagnosis from infectious forms. Vascular manifestations comprise pulmonary arterial hypertension (PAH, 0.5–17.5%), diffuse alveolar hemorrhage (DAH, 1–5.4%), and venous thromboembolism (VTE, 9%; 35–42% in the presence of antiphospholipid antibodies), as well as acute reversible hypoxemia (ARH). Rare forms include airway disease and shrinking lung syndrome. Anifrolumab, a monoclonal antibody that blocks the type I interferon receptor, has shown efficacy in moderate to severe SLE, particularly for mucocutaneous, articular, and serological manifestations. According to European guidelines, Anifrolumab is recommended as first-line therapy for extra-renal (non–major organ) manifestations, especially cutaneous, and as add-on therapy for severe disease. We report a case of SLE with lupus pneumonitis and antiphospholipid syndrome (APS) successfully treated with Anifrolumab. Materials and Methods. A 45-year-old Caucasian man, with no significant past medical history, experienced three episodes of bilateral pneumonia (bibasilar consolidations) since mid-2022, all requiring hospitalization. One episode was complicated by pulmonary embolism and another by a generalized rash, both treated with antibiotics and corticosteroids. Laboratory findings revealed positivity for ANA (1:2560, homogeneous nuclear pattern), anti-dsDNA, anti-Ro52, anti-nucleosome, anti-histone antibodies, rheumatoid factor, and lupus anticoagulant. Rheumatologic assessment revealed Jaccoud arthropathy and widespread erythematous maculopapular lesions on the trunk, back, and limbs. Given the absence of infectious findings (including negative BAL), the pulmonary abnormalities were attributed to autoimmune disease. A diagnosis of SLE with cutaneous involvement (histologically confirmed subacute cutaneous lupus erythematosus), pulmonary and articular manifestations, and associated APS was established. The patient started oral anticoagulation, high-dose corticosteroids, hydroxychloroquine (discontinued due to cutaneous reaction), and rituximab (1 g × 2), achieving initial improvement. Rituximab was discontinued after the second course due to an adverse reaction, followed by recurrence of bilateral pneumonia and cutaneous flare. Consequently, Anifrolumab 300 mg every 4 weeks was initiated. Results. After six months of Anifrolumab therapy, HRCT showed marked improvement, inflammatory markers normalized, and the patient reported substantial improvement in respiratory, cutaneous, and articular symptoms, allowing progressive corticosteroid tapering. Conclusions. Anifrolumab represents a promising therapeutic option for patients with severe SLE manifestations who are refractory to or intolerant of conventional treatments. Modulation of the type I interferon signaling pathway may play a key role in achieving systemic disease control. In patients with pulmonary involvement, a multidisciplinary approach and careful differentiation from infectious causes are essential.

ObjectivesTo study the prevalence of SLE arthritis subtypes, deforming and non-deforming arthritis, and determine the association with clinical features, serology, and the influence of type I interferon.MethodsThis is a retrospective study of patients with arthritis defined by the ACR or EULAR/ACR SLE classification criteria at presentation and SLEDAI 2K over follow-up identified from a single-center SLE database (July 1970-Aug 2024) from both inception and prevalent cohorts. Demographic, clinical, laboratory (including interferon signature), radiographic features, and treatment variables were retrieved from the database. Descriptive statistics were used to outline features across 3 subtypes of arthritis: non-deforming arthritis (determined by clinical examination), arthritis with reducible deformities or Jaccoud’s Arthropathy (JA), and arthritis with non-reducible deformities or rhupus. Factors associated with deforming arthritis were determined using multivariate Fine and Gray modeling for the inception cohort.ResultsArthritis was observed in 1,248 of 2264 (55.12%) patients. 908 (72.6%) had non-deforming and 340 (27.2%) had deforming arthritis- 239 (19.2%) had JA, 101 (8.1%) had rhupus. The median age at diagnosis of SLE was comparable, though a higher proportion of females was observed in JA (p=0.03). The distribution of organ involvement and antibodies was similar across the 3 subtypes, except nervous system involvement(p=0.03) and anti-Ro antibodies (p=0.04) being more frequent in rhupus. There was a trend toward higher mean SLEDAI-2K scores in JA (p=0.07), and the SDI was highest in rhupus (p<0.01). The distribution of rheumatoid factor and anti-CCP positivity did not differ significantly. The proportion of patients with high interferon signature was the greatest in JA, followed by non-deforming arthritis, and lastly, rhupus (p<0.01). Radiographs (n, 95) revealed erosive disease in 10 of 43 (23.2%) with JA, 12 of 36 (33.3%) with rhupus, and 2 of 16 (12.5%) with non-deforming arthritis. The use of glucocorticoids, mycophenolate, and belimumab was most prevalent in JA, while methotrexate was higher in rhupus (Table 1). In the multivariate analysis, JA was associated with higher average mean SLEDAI 2K [1.09(1.01-1.19)] and females [3.3(1.14-12.5)]. No associations were observed with rhupus.Table 1:Baseline demographic, clinical, laboratory, and treatment characteristics of patients with arthritis (n=1248)ConclusionArthritis was observed in half the cohort, with the majority being non-deforming (72.6%). Among deforming arthritis, JA (19%) was more common than rhupus (8%). JA was associated with a high interferon signature, high disease activity, and female sex compared to rhupus. This sheds light on 2 different mechanisms for deforming arthritis with JA associated with SLE disease burden in contrast to rhupus. Erosions were observed in both types of deforming arthritis blurring the line of radiologic differences historically outlined between them.

O031 / #611Topic:AS23 - SLE-Diagnosis, Manifestations, & OutcomesABSTRACT CONCURRENT SESSION 05: EMERGING INSIGHTS ON THE MANAGEMENT OF LUPUS MANIFESTATIONS AND COMORBIDITIES23-05-2025 1:40 PM - 2:40 PMBackground/PurposeMusculoskeletal involvement in Systemic Lupus Erythematosus (SLE) is one of the most prominent manifestations of the disease, featuring in both the classification criteria and disease activity assessments. It is presently unclear if specific subtypes of lupus arthritis—nondeforming nonerosive (NDNE), Jaccoud’s arthropathy (JA), and rhupus, are associated with specific clinical associations. We aimed to study the prevalence of subtypes of lupus arthritis and determine their association with clinical features, serology, and type I interferon signature.MethodsThis is an observational cohort study of patients with arthritis (defined by the ACR or EULAR/ACR SLE classification criteria at presentation and SLEDAI 2K over follow-up) identified from a single-center SLE database (July 1970-Aug 2024) from both inception and prevalent cohorts. Demographic, clinical, laboratory (including interferon signature), radiographic features, and treatment variables were retrieved from the database. Descriptive statistics were used to outline features across 3 subtypes of arthritis; nondeforming arthritis (determined by clinical examination), arthritis with reducible deformities or JA, and arthritis with nonreducible deformities or rhupus. In the inception cohort, time to deformities was studied using cumulative incidence survival analysis. Factors associated with deforming arthritis were determined using multivariate Fine and Gray modeling as a time-to-event analysis for the inception cohort using age, sex, smoking, adjusted mean SLEDAI-2K, autoantibodies, complements, antimalarial, glucocorticoid, and immunosuppression use.ResultsArthritis was observed in 1,248 of 2264 (55.12%) patients. 908 (72.6%) had nondeforming and 340 (27.2%) had deforming arthritis–239 (19.2%) had JA, 101 (8.1%) had rhupus. The median age at diagnosis of SLE was comparable, though a higher proportion of females was observed in JA (p=0.03). The distribution of organ involvement and antibodies was similar across the 3 subtypes, except nervous system involvement (p=0.03) and anti-Ro antibodies (p=0.04) being more frequent in rhupus. There was a trend toward higher mean SLEDAI-2K scores in JA (p=0.07), and the modified SDI (excluding musculoskeletal component) was the highest in rhupus (p<0.01). The distribution of rheumatoid factor and anticitrullinated protein antibody positivity did not differ significantly across the 3 groups. The proportion of patients with high interferon signature was the greatest in JA, followed by nondeforming arthritis, and lastly, rhupus (p<0.01). Radiographs (n, 95) revealed erosive disease in 10 of 43 (23.2%) with JA, 12 of 36 (33.3%) with rhupus, and 2 of 16 (12.5%) with nondeforming arthritis. The use of glucocorticoids, mycophenolate mofetil, belimumab, and other biologics was most prevalent in JA, while methotrexate was higher in rhupus. (Table 1) In the inception cohort, the cumulative incidence plot showed a shorter time to the development of JA of 2.07 [1.00, 15.76] as compared to 4.64 [1.00, 9.78] years for rhupus. In the multivariate analysis, JA was associated with a higher adjusted mean SLEDAI 2K [1.09(1.01-1.19)] and female sex [3.3(1.14-12.5)]. No significant associations were observed with rhupus.Table 1:Baseline demographic, clinical, laboratory, and treatment characteristics of patients with arthritis (n=1248)ConclusionsArthritis was observed in half the cohort, with the majority being nondeforming (72.6%). Among deforming arthritis, JA (19%) was more common than rhupus (8%). JA was associated with a high interferon signature, high disease activity, female sex, and a shorter time to development as compared to rhupus. These sheds light on 2 different mechanisms for deforming arthritis, with JA associated with SLE disease burden in contrast to rhupus. Erosions were observed in both types of deforming arthritis, blurring the line of radiologic differences historically outlined between them.

Correction of non-traumatic extensor tendon dislocation and ulnar drift at the metacarpophalangeal joint by the modified Dell technique

Jaccoud's arthropathy is a deforming, nonerosive form of arthritis initially described in patients with rheumatic fever. However, it has been recently observed more frequently in those with systemic lupus erythematosus. Cases of Jaccoud's arthropathy have also been described to be associated with other conditions. Herein, we describe the case of a 64-year-old Brazilian man who exhibited Jaccoud's arthropathy associated with leprosy. To the best of our knowledge, this is the first report on this association.

Joint involvement in SLE is the most frequent manifestation and shows a wide heterogeneity. It has not a valid classification and it is often underestimated. Subclinical inflammatory musculoskeletal involvement is not well known. We aim to describe the prevalence of joint and tendon involvement in hand and wrist of SLE patients, either with clinical arthritis, arthralgia or asymptomatic and compare it with healthy subjects using contrasted MRI. SLE patients fulfilling SLICC criteria were recruited and classified as follows: group (G) 1: hand/wrist arthritis, G2: hand/wrist arthralgia, G3: no hand/wrist symptoms. Jaccoud arthropathy, CCPa and RF positivity, hand OA or surgery were excluded. Healthy subjects (HS) were recruited as controls: G4. Contrasted MRI of non-dominant hand/wrist was performed. Images were evaluated following RAMRIS criteria extended to PIP, Tenosynovitis score for RA and peritendonitis from PsAMRIS. Groups were statistically compared. A total of 107 subjects were recruited (G1: 31, G2:31, G3:21, G4:24). Any lesion: SLE patients 74.7%, HS 41.67%; P 0.002. Synovitis: G1: 64.52%, G2: 51.61%, G3: 45%, G4: 20.83%; P 0.013. Erosions: G1: 29.03%; G2: 54.84%, G3: 47.62%; G4: 25%; P 0.066. Bone marrow oedema: G1: 29.03%, G2: 22.58%, G3: 19.05%, G4: 0.0%; P 0.046. Tenosynovitis: G1: 38.71%; G2: 25.81%, G3: 14.29%, G4: 0.0%; P 0.005. Peritendonitis: G1: 12.90%; G2: 3.23%, G3: 0.0%, G4: 0.0%; P 0.07. SLE patients have a high prevalence of inflammatory musculoskeletal alterations confirmed by contrasted MRI, even if asymptomatic. Not only tenosynovitis but peritendonitis is also present.

Toll-like receptor 9 (TLR9) is an important component of the innate immune system and have been associated with several autoimmune diseases, such as Systemic Lupus Erythematosus (SLE). The aim of this study was to investigate polymorphisms in TLR9 gene in a Brazilian SLE patients group and their association with clinical manifestation, particularly Jaccoud's arthropathy (JA). We analyzed DNA samples from 204 SLE patients, having a subgroup of them presenting JA (n=24). A control group (n=133) from the same city was also included. TLR9 single nucleotide polymorphisms (SNPs) (-1237 C>T and +2848 G>A) were identified by sequencing analysis. The TLR9 gene genotype frequency was similar both in SLE patients and the control group. In the whole SLE population, an association between the homozygosis of allele C at position -1237 with psychosis and anemia (p < 0.01) was found. Likewise, the homozygosis of allele G at position +2848 was associated with a discoid rash (p < 0.05). There was no association between JA and TLR9 polymorphisms. These data show that TLR9 polymorphisms do not seem to be a predisposing factor for SLE in the Brazilian population, and that SNPs are not associated with JA.

Jaccoud's arthropathy (JA) is a condition characterized by joint deformities that have a "reducible" pattern, that is, they return to the normal appearance with a passive manoeuvre. JA was described in patients with rheumatic fever (RF) more than a century ago, and presently, the majority of the patients have systemic lupus erythematosus (SLE). The aim of this review is to draw one attention to the epidemiology, pathogenesis, histopathology, clinical features, imaging, and management of JA in patients with SLE (Jaccoud-type lupus arthropathy). The search strategy included articles retrieved from PubMed utilizing the terms "lupus arthropathy", "lupus deforming arthropathy", "lupus hand", "lupus foot", "chronic postrheumatic arthropathy", "Jaccoud's" and "Jaccoud" from 1950 until March 2021, with no language restriction. The prevalence of Jaccoud-type arthropathy in SLE is approximately 5%. The aetiopathogenic mechanisms of JA are not yet known. The most common joint deformities of JA, are ulnar deviation, swan neck, and the "z" of the thumb. Unfortunately, none of the proposed classification criteria for JA have been validated so far. Characteristically, there is no bone erosion on plain radiographs of the joints, but more sensitive imaging methods, magnetic resonance imaging or high-performance ultrasound may reveal small bone erosions. There is no preventive measure against JA development, specific clinical treatment or convincing surgical approach for correcting the deformities. As daily activities and quality of life are compromised in patients with JA, other studies are urgently needed in this area.

Occupational therapy for spontaneous repair of the extensor digit tendons in left middle finger with Jaccoud arthropathy due to systematic lupus erythematosus:

Neuropsychiatric manifestations are commonly seen in lupus and limb paresis is commonly due to noncompressive spinal etiology. Here, we report a case of a 50-year-old female with Jaccoud's arthropathy who had presented with quadriparesis for 3 months. She was diagnosed with lupus during her hospital stay. Her magnetic resonance imaging of the spine showed the presence of a soft-tissue mass in the cervical spine with atlantoaxial dislocation causing severe cord compression. This case highlights the importance of suspecting compressive myelopathy secondary either to infection or inflammation due to disease process in the cervical spine of patients with lupus, especially with Jaccoud's arthritis, presenting with weakness of limbs.

We aimed to investigate the prevalence of US findings in the hand joints and related tendons and explore clinical and laboratory associations in SLE patients of the typical lupus clinic. One hundred consecutive SLE patients were enrolled in the study. Using B-mode and Doppler US, bilateral wrist, metacarpophalangeal and proximal interphalangeal joints were examined for synovitis and erosions, as well as for signs of hand tenosynovitis. US detected synovitis (grade 1-3) in 75% and erosive changes in 25% of the cohort. We found that clinical examination underestimated grade ≥2 synovitis by 13%, while US detected SH grade ≥2 in 10% of asymptomatic patients. The overall inflammatory burden, reflected by the US score, was associated with disease activity (respectively with CPR, SELENA-2K, MS-BILAG, and hypocomplementemia), as well as the presence of bone erosions. Rhupus patients had higher inflammatory markers, significantly more synovial hypertrophy, more erosions, more grade 3 tenosynovitis, and were more likely to receive methotrexate (p<0.001) than patients with SLE arthritis, while patients with Jaccoud's arthropathy were more likely to accumulate damage. The dominant hand exhibited more inflammatory changes (respectively synovial hypertrophy grade ≥2) at both the wrist and MCP joints; however, handedness was not associated with structural damage. In conclusion: 1. joint involvement in SLE is frequent and underacknowledged; 2. the overall inflammatory burden is associated with systemic disease activity and joint damage; (3) destructive arthritis is more likely to occur in the context of concomitant RA or within an "RA-like" subtype of SLE arthropathy; 4. hand dominance is associated with synovitis, but not structural changes; 5. US assessment may help tailor the management of joint involvement, thus preventing joint damage and disability in SLE patients.

Instability of the extensor digitorum tendons in Jaccoud arthropathy assessed by semi-dynamic MRI of the metacarpophalangeal joints

Metalloproteinase (MMP)-3 and MMP-12 are proteolytic enzymes especially implicated in joint inflammation. This study aims to evaluate their association with arthritis features and hand MRI abnormalities in patients with SLE. Fifty SLE patients, with a mean (s.d.) age of 48.1 (14.6) years were tested for MMP-3 and MMP-12 serum levels, then further classified according to the presence of X-ray erosions and joint deformities. Eighteen RA patients aged 47.9 (11.8) and 14 healthy people aged 46.0 (11.0) were enrolled as control groups. A subgroup of 28 SLE patients underwent a dominant-hand MRI; the detected changes were classified and semi-quantitatively scored as capsular swelling, synovitis, edematous or proliferative tenosynovitis, bone oedema, bone erosions. Statistical analysis was performed using multiple regression models. MMP-3 were significantly higher in patients with Jaccoud's arthropathy (JA) (22.1 ng/ml, P < 0.05) and independently associated with hsCRP serum levels (B-coeff 0.50; r = 0.30; P < 0.05). MMP-12 serum levels were significantly lower in patients with JA (0.18 ng/ml, P < 0.05) and inversely associated with the prednisone daily dose (B-coeff -0.03; r = -0.44; P < 0.01). Capsular swelling and edematous tenosynovitis, the most prevalent hand MRI changes in patients with JA, associated with higher MMP-3 (B-coeff 0.12; r = 0.66; P < 0.01 and B-coeff 0.08; r = 0.59; P < 0.01, respectively) and lower MMP-12 serum levels (B-coeff -7.4; r = -0.50; P < 0.05 and B-coeff -5.2; r = -0.44; P = 0.05, respectively). Imbalanced MMP-3 and MMP-12 serum levels are influenced by inflammation and glucocorticoids in SLE patients and associated with JA and distinctive hand MRI changes.

Jaccoud arthropathy (JA) is a nonerosive and deforming arthropathy experienced frequently by patients with systemic lupus erythematosus (SLE). Although genetic polymorphisms are associated with SLE development, the association between genetic polymorphisms and JA has not been studied to date. The main objective of this study was to evaluate an association between HLA, STAT4, IRF5, and BLK polymorphisms and the presence of JA in Brazilian individuals with SLE. Patients were selected from a cohort of individuals with SLE followed at 2 rheumatology reference centers in Salvador, Bahia, Brazil. The JA diagnosis was based on clinical and radiological criteria. The participants were genotyped for rs9271100, rs7574865, rs10488631, and rs13277113 polymorphisms in the HLA, STAT4, IRF5, and BLK genes, respectively, using real-time polymerase chain reaction. The presence of JA was correlated with allele frequencies, and clinical and laboratory data. One hundred forty-four individuals with SLE (38 with JA and 106 with SLE without JA) were studied. The mean age of the patients was 45 ± 12 years; the majority were women and had brown skin. Patients with JA had a longer disease duration than patients without JA. Serositis and neuropsychiatric manifestations were more frequent in the JA population. The A allele of rs13277113 in the BLK gene was associated with the presence of JA. The rs13277113 polymorphism in the BLK gene was found to be a possible genetic risk for JA development. However, further studies in larger populations should be performed to confirm this finding.

Systemic lupus erythematosus (SLE) is an autoimmune disease that can present changes in blood vessels, which can be evaluated by periungual nailfold videocapillaroscopy (VCP). This technique is important for the diagnosis of systemic sclerosis and to identify individuals with Raynaud phenomenon at higher risk of developing systemic sclerosis. This study aims to describe the videocapillaroscopic profile of a series of SLE patients and to investigate if the VCP pattern is different among those with Jaccoud arthropathy (JA) compared with those without. Between September 2014 and March 2015, the patients in this study underwent VCP, clinical evaluation, and laboratory tests. The capillaroscopic patterns were defined as minor, major, and scleroderma (SD). The presence of capillaroscopic findings, such as elongated capillaries, tortuosity, ectasia, prominent venous plexus, neoangiogenesis, hemorrhage, and megacapillaries, were also observed. Associations were calculated using the χ2, Fisher exact, or Student t test. In a population of 113 females with SLE (67 without JA and 46 with JA), at least 1 alteration was observed in VCP in 89.40% of them, among which "nonspecific changes" were the most prevalent. Minor changes were seen in 39 (58.2%) and 26 (56.5%), major changes in 21 (31.3%) and 11 (23.9%), and SD pattern in 2 (3.0%) and 3 (6.5%), in the patients without and with JA, respectively (p > 0.05). The majority of patients with SLE demonstrated changes in the VCP examination, but this tool did not allow discrimination between those with or without JA.

FRI0175 SEMI-DYNAMIC MRI OF THE EXTENSOR DIGITORUM TENDONS IN JACCOUD ARTHROPATHY

Joint deformities in Jaccoud arthropathy (JA), associated with systemic lupus erythematosus (SLE), can lead to a reduction in articular function and an impaired quality of life. There is controversy in the literature as to whether hand dominance contributes to deformities observed in rheumatoid arthritis and other forms of rheumatism. The purpose of this study was to assess whether hand dominance has any influence on the degree of deformity or joint range of motion in patients with JA associated with SLE. This was a pilot cross-sectional study of 22 female patients (mean age, 46.18 ± 12.61 years) with both SLE and JA. Each patient's hand grip and hand and wrist joint range of motion were assessed by the same examiner. The Wilcoxon test was used to compare the median values of the angles and muscular strength found between the sides. All the study participants were right-side dominant. The median muscle strength of the right and left hands was 13.25 kilogram-force (KgF) (range, 7.00-18.00 KgF) and 10.50 KgF (range, 7.50-17.25 KgF), respectively. Both the fingers of the right and left hands had a median ulnar drift of 10.00°; however, their ranges differed (right, 4.50° to 20.00°; left: 0.00° to 15.50°). No statistically significant differences were found in the extension angulation of the proximal interphalangeal joints or the flexion of the distal interphalangeal joints of the second to fifth fingers, between the dominant and nondominant hands. Hand dominance does not seem to influence the degree of JA deformities in patients with SLE.

Efficacy of High-intensity Laser in Jaccoud's Arthropathy in Systemic Lupus Erythematosus

ObjectivesThis article estimates the frequency of polyautoimmunity and associated factors in a large retrospective cohort of patients with SLE.MethodsRELESSER (Spanish Society of Rheumatology Lupus Registry) is a nationwide multicentre, hospital-based registry of SLE patients. This is a cross-sectional study. The main variable was polyautoimmunity, which was defined as the co-occurrence of SLE and another autoimmune disease, such as autoimmune thyroiditis, RA, scleroderma, inflammatory myopathy and MCTD. We also recorded the presence of multiple autoimmune syndrome, secondary SS, secondary APS and a family history of autoimmune disease. Multiple logistic regression analysis was performed to investigate possible risk factors for polyautoimmunity.ResultsOf the 3679 patients who fulfilled the criteria for SLE, 502 (13.6%) had polyautoimmunity. The most frequent types were autoimmune thyroiditis (7.9%), other systemic autoimmune diseases (6.2%), secondary SS (14.1%) and secondary APS (13.7%). Multiple autoimmune syndrome accounted for 10.2% of all cases of polyautoimmunity. A family history was recorded in 11.8%. According to the multivariate analysis, the factors associated with polyautoimmunity were female sex [odds ratio (95% CI), 1.72 (1.07, 2.72)], RP [1.63 (1.29, 2.05)], interstitial lung disease [3.35 (1.84, 6.01)], Jaccoud arthropathy [1.92 (1.40, 2.63)], anti-Ro/SSA and/or anti-La/SSB autoantibodies [2.03 (1.55, 2.67)], anti-RNP antibodies [1.48 (1.16, 1.90)], MTX [1.67 (1.26, 2.18)] and antimalarial drugs [0.50 (0.38, 0.67)].ConclusionPatients with SLE frequently present polyautoimmunity. We observed clinical and analytical characteristics associated with polyautoimmunity. Our finding that antimalarial drugs protected against polyautoimmunity should be verified in future studies.

Musculoskeletal manifestations are extremely common in patients with systemic lupus erythematosus. Transient and migratory arthralgia is frequently reported even without clinical signs of joint or tendon inflammation. In less than 15% of patients, joints may be more severely affected by deforming (Jaccoud's arthropathy) and/or erosive arthropathy (Rhupus syndrome). In recent years, ultrasound has emerged as a promising imaging technique for the assessment of musculoskeletal involvement in systemic lupus erythematosus, having demonstrated the ability to detect inflammation and structural damage both at articular and periarticular level. Recent ultrasound studies have also revealed new insights into musculoskeletal involvement in patients with systemic lupus erythematosus, some of them questioning the traditional concepts of systemic lupus erythematosus arthropathy, with potential clinical, prognostic and therapeutic implications. In daily clinical practice, the use of ultrasound in the assessment of joint and tendon involvement in patients with systemic lupus erythematosus is still limited. Several methodological issues encountered in ultrasound studies evaluating musculoskeletal involvement in systemic lupus erythematosus patients need to be addressed in order to improve both the reliability and clinical usefulness of ultrasound findings. This paper reviews ultrasound studies assessing musculoskeletal involvement in patients with systemic lupus erythematosus, highlighting certainty, limits, potential applications and future perspectives of ultrasound use in systemic lupus erythematosus patients.