Subacute Cutaneous Lupus Erythematosus Research Articles

A Case of Terbinafine-Induced Subacute Cutaneous Lupus Erythematosus rapidly resolving with Upadacitinib.

Drug-induced subacute cutaneous lupus erythematosus (SCLE) is a lupus-like cutaneous eruption without systemic features that results due to insult from a range of chemically diverse culprit drugs. In cases where remission is not achieved with drug discontinuation, treatment options generally involve those used to treat idiopathic SCLE, including topical or systemic corticosteroids, antimalarials and/or immunosuppressive drugs. Herein we present a case of drug-induced SCLE secondary to terbinafine with a prolonged course following drug cessation that did not respond to classical treatments, but demonstrated rapid and sustained improvement within 48 hours of upadacitinib treatment.

Histopathological Granuloma Formation in a Subacute Cutaneous Lupus Erythematosus Patient

Introduction: Subacute cutaneous lupus erythematosus (SCLE) is one of the cutaneous lupus erythematous subtypes. Being an autoimmune condition in nature, its diagnosis requires compatible clinical presentation with histopathological findings. Objective: To present a subacute cutaneous lupus erythematosus with granuloma formation. Case Report: A 56- year-old woman presented with longstanding red plaques on her face, trunk, and extremities. Two skin biopsies were performed during two different visits. The results showed tuberculoid leprosy and multiple granuloma annulare. She was treated accordingly for years with no improvement in her condition. Later, the third skin biopsy with CD123 immunohistochemical stain was done. The result was most consistent with cutaneous lupus erythematosus. Together with her clinical findings, she was finally diagnosed with SCLE. Her rashes resolved after being treated with hydroxychloroquine and topical steroid. Conclusion: The diagnosis of SCLE must be made based on both clinical presentations and histopathological findings.

Paraneoplastic subacute cutaneous lupus erythematosus

The genesis of subacute cutaneous lupus erythematosus (SCLE) is multifactorial and includes idiopathic, drug-related and paraneoplastic etiologies. This article reports the case of a70-year-old female patient with paraneoplastic SCLE in whom a lung adenocarcinoma was detected during the extended examination. Aparaneoplastic SCLE should be considered when apatient with SCLE presents with lesions in regions of the skin not exposed to sunlight and beginning Bsymptoms.

Association of the Spectrum of Cutaneous Lupus Erythematosus with Disease Activity and Systemic Manifestations in Patients with Systemic Lupus Erythematosus.

Cutaneous involvement is common in systemic lupus erythematosus (SLE) patients and may be essential to the disease activity. This study aimed to describe cutaneous manifestations spectrum and determine the association of cutaneous lesions with the disease activity and systemic involvement among SLE patients in Malang, Indonesia. A cross-sectional study was conducted using 54 SLE patients from rheumatology outpatient clinic at Saiful Anwar General Hospital Malang, Indonesia. Cutaneous features were classified according to Gilliam and Sontheimer classification of cutaneous lupus. Disease activity and clinical manifestations were documented according to Mexican-SLE disease activity index (Mex-SLEDAI). Among 54 subjects, 50% of the patients had cutaneous manifestations. Subacute cutaneous lupus erythematosus (SCLE) was observed in 11.1% of patients, and malar rash in 20.4%. Subjects with cutaneous lesions had significantly higher Mex-SLEDAI scores, especially those who had SCLE (p<0.001), malar rash (p=0.002), alopecia (p=0.002), and photosensitivity (p=0.032). Six patients (11.1%) had skin infections with higher disease activity (9[8-11]vs.2[0-4];p<0.001). SCLE was significantly associated with malar rash (OR 11.7[1.8-76.5]), vasculitis (OR 43.0[4.1-445.6]), and fatigue (OR 15.0[2.1-108.8]). Malar rash was associated with photosensitivity (OR 8.4[1.6-44.0]), while oral or nasal ulcer was associated with fatigue (OR 8.6 [1.4-54.6]). Vasculitis (OR 5.9[1.0-35.1]) and nephritis (OR 11.7 [1.8-76.5]) were associated with the presence of skin infection. SCLE and malar rash are the most common cutaneous lesions among subjects. Subjects with cutaneous lesions have relatively higher disease activity. Several skin lesions are also associated with SLE patients' systemic manifestations.

Dermoscopy for the Differentiation of Subacute Cutaneous Lupus Erythematosus from Other Erythematous Desquamative Dermatoses-Psoriasis, Nummular Eczema, Mycosis Fungoides and Pityriasis Rosea.

Subacute cutaneous lupus erythematosus (SCLE) is a condition that might pose a diagnostic challenge. The aim of this study was to assess the usefulness of videodermoscopy in the differentiation of SCLE from other erythematous-desquamative dermatoses. Consecutive patients with SCLE (n = 27), psoriasis (n = 36), nummular eczema (n = 30), mycosis fungoides (n = 26), and pityriasis rosea (n = 20) referred to our Department of Dermatology were recruited for this study. A representative lesion was visualized using a Canfield D200EVO Videodermatoscope (Canfield Scientific GmbH, Bielefeld, Germany) and evaluated for the following parameters: vessels (morphology and distribution), scales (color and distribution), follicular findings, colors and morphologies, and presence of specific clues. SCLE was predominantly characterized by a polymorphous vascular pattern (92.6%) of unspecific distribution (92.6%) over a pink-red background (74.1%). Gray-brown dots were present in 10 (37.0%) cases, and pigmentation was noted in 15 (55.6%) patients, including peripheral pigmentation in 7 (25.9%) patients. Videodermoscopic evaluation showed significant differences between SCLE and psoriasis, which was characterized by regularly distributed dotted vessels. Although some common dermoscopic features with MF were noted, the presence of yellow structureless areas and red dots/globules favored the diagnosis of MF. In conclusion, a polymorphic vascular pattern, especially in association with gray-brown dots and/or peripheral pigmentation, is a valuable clue for the differentiation of SCLE from other erythematous-desquamative dermatoses.

Plasmacytoid Dendritic Cells Are Not Major Producers of Type 1 IFN in Cutaneous Lupus: An In-Depth Immunoprofile of Subacute and Discoid Lupus

The immunologic drivers of cutaneous lupus erythematosus (CLE) and its clinical subtypes remains poorly understood. We sought to characterize the immune landscape of discoid lupus erythematosus (DLE) and subacute CLE (SCLE) using multiplexed immunophenotyping. We found no significant differences in immune cell percentages between DLE and SCLE (p>0.05) with the exception of an increase in TANK binding kinase 1 (TBK1) in DLE (p<0.05). Unbiased clustering grouped subjects into two major clusters without respect to clinical subtype. Subjects with a history of smoking had increased percentages of neutrophils, disease activity, and endothelial granzyme B than non-smokers. Despite previous assumptions, plasmacytoid dendritic cells (pDCs) did not stain for type 1 interferon (IFN-1). Skin-eluted and circulating pDCs from CLE subjects expressed significantly less IFNα than healthy control pDCs upon toll like receptor (TLR) 7 stimulation ex vivo (p<0.0001). These data suggest that DLE and SCLE have similar immune microenvironments in a multiplexed investigation. Our aggregated analysis of CLE revealed that smoking may modulate disease activity in CLE via neutrophils and endothelial GZMB. Notably, our data suggest that pDCs are not the major producers of IFN-1 in CLE. Future in vitro studies to investigate the role of pDCs in CLE are needed.

51 Rapid clinical improvement of refractory subacute cutaneous lupus erythematosus with oral TYK2 inhibitor deucravacitinib: a case report

51 Rapid clinical improvement of refractory subacute cutaneous lupus erythematosus with oral TYK2 inhibitor deucravacitinib: a case report

Diffuse skin lesions in a patient with systemic lupus erythematosus: A case report

Systemic lupus erythematous (SLE) is a persistent autoimmune disease with systemic and life- threatening manifestations, in which cutaneous disease is the second most common manifestation, after joint inflammation. Skin lesions in SLE is originally divided into two groups: LE-specific lesions and LE-nonspecific lesions. The term “LE-specific lesions” includes 3 distinct categorizes: Acute cutaneous lupus erythematosus (ACLE), subacute cutaneous lupus erythematosus (SCLE) and chronic cutaneous lupus erythematosus (CCLE) [4]. By another method of classification, specific skin lesions can also be classified as localized or generalized lesions. The most common localized lesion is malar, or butterfly rash, which is reported in 20-60% of patients with SLE. In comparision to localized lesions, generalized lesions, which are indiscriminately referred to as lupus maculopapular rash, are relatively rare. This form of lesion is characterized by well-demarcated erythematous macules and/or papules located on the extensor side of limbs, sparing the knuckles. In this article, we report a case of a female patient having generalized cutaneous lesions. She was initially diagnosed with SLE and received treatment at Department of Allergy, Center of Dermato-Venerology and Allergy, 108 Military Central Hospital.

Subacute cutaneous lupus erythematosus in a patient of pancreatic cancer secondary to novel albumin-bound paclitaxel chemotherapy

Paclitaxel is commonly used for the treatment of breast, ovarian, gastrointestinal, and carcinoma of the head and neck. Paclitaxel treatment is often associated with hair loss, myalgia, acral erythema, scleroderma and Stevens–Johnson syndrome, peripheral neuropathy, nausea, vomiting, bone marrow suppression, and rarely cardiac electroconductive pathway abnormalities. However, its association with cutaneous/systemic lupus erythematosus has been sparsely described. In this case report, we are discussing a case of nab-paclitaxel-induced subacute cutaneous lupus erythematosus which has been described only once in the published literature.

The power of representation in educational materials: Teaching cutaneous lupus across skin tones in an interactive module.

Clinicians report low confidence assessing cutaneous lupus erythematosus (CLE) lesions, especially for patients who identify as Black, Indigenous, and People of Color (BIPOC), who are historically excluded from educational materials. To address this, we created an online, interactive module teaching an approach to assessing CLE across skin tones and measured its impact on medical knowledge and confidence. Our team created a module with case-based methods to introduce an approach to CLE, common mimicking rashes, and tips for photographing cutaneous lesions in BIPOC. Graduate medical trainees from five academic institutions completed the module. Using surveys and pre-post testing, we assessed changes in medical knowledge and clinical confidence along with learner satisfaction, comparing responses using Wilcoxon-signed rank tests and Chi square analysis. We assessed the module's representation of light, medium, and dark skin tones with Chi square analysis. The module represented light, medium, and dark skin tones (X2 4.788, p=0.091) among 102 images; 77.5% (79) were novel images from authors' personal libraries. Ninety-four participants completed the post-module test and evaluation survey. Analyses revealed significant improvement in medical knowledge identifying serologic studies associated with subacute CLE (Χ2 14.035, P<0.001) and describing how to photograph rashes (Χ2 38.211, P<0.001). Participants reported improved confidence across all learning objectives after module completion (p<0.001). This module is the first to introduce an approach to assessing CLE across skin tones, effectively increasing medical knowledge and confidence among graduate medical trainees.

The Possible Clinical Significance of a Decreased Serum Level of Soluble PD-L1 in Discoid Lupus Erythematosus, but Not in Subacute Cutaneous Lupus Erythematosus-A Pilot Study.

Cutaneous lupus erythematosus (CLE) is an autoimmune skin disease with various clinical forms, including the subtypes of discoid lupus erythematosus (DLE) and subacute cutaneous lupus erythematosus (SCLE). The altered function of the programmed cell death 1/programmed cell death ligand 1 (PD-1/PD-L1) axis in CLE pathogenesis has been suggested. Here, the soluble forms of PD-1 (sPD-1) and PD-L1 (sPD-L1) were explored in untreated DLE and SCLE. Levels of sPD-1 and sPD-L1 were determined by enzyme-linked immunosorbent assay in serums of 21 DLE, 18 SCLE, 13 systemic lupus erythematosus (SLE) patients and 20 healthy controls (HCs). Differences between patient groups and HCs, and the association between clinical activity of skin symptoms and sPD-1/sPD-L1 levels were analyzed with Mann-Whitney U-test and Spearmann's correlation. Regarding sPD-1 levels, no statistically significant differences were found between DLE and SCLE groups, nor compared to HCs. As for sPD-L1, a significantly lower level was found in the DLE group compared to the SCLE and HC groups (p = 0.027 and p = 0.009, respectively). In SLE, significantly higher sPD-1 was found compared to HCs (p = 0.002). No association between skin symptom activity and sPD-1/sPD-L1 levels was found in CLE. Alterations of the inhibitory effect of sPD-L1 on T-cell activity might elucidate the differences between DLE and SCLE.

Clinical and immunologic features of the phenotype of systemic lupus erythematosus combined with Sjögren's syndrome

The problem of clinical and immunological heterogeneity of systemic lupus erythematosus (SLE) is of great interest, especially the combination of SLE and Sjögren's syndrome (SjS) determines a more favorable prognosis and specifics of therapy.Objective: to characterize the clinical and immunological features of SLE combined with SjS.Material and methods. The retrospective study included 44 patients with SLE combined with SjS and 356 patients with SLE without SjS, hospitalized at the V.A. Nasonova Research Institute of Rheumatology between 2013 and 2021. The two groups of patients were compared in terms of life expectancy, clinical manifestations, laboratory parameters and therapy.Results and discussion. The following phenotypic features of the clinical and immunological variant of SLE combined with SjS were found: significantly longer duration of SLE (p&lt;0.01); higher incidence of polyarthritis (p=0.01) and Raynaud's syndrome (p&lt;0.003) at disease onset. Subacute cutaneous lupus erythematosus, Raynaud's syndrome, peripheral nervous system involvement (sensory polyneuropathy and distal sensory-motor polyneuropathy) were found more frequently in SLE combined with SjS than in SLE without SjS, p&lt;0.0001. Among laboratory abnormalities in SLE patients with SjS, leukopenia (p&lt;0.0001), antibodies to SSA/Ro, SSB/La, and rheumatoid factor (p&lt;0.0001) were observed in a greater number of cases. In the group of SLE combined with SjS, the chronic variant of SLE course according to the classification of V.A. Nasonova, and lower activity were observed, which didn't not require therapy with high doses of glucocorticoids; in this group, rituximab (p&lt;0.01), cyclophosphamide and methotrexate were used significantly more often.Conclusion. Thus, significant clinical and laboratory differences were found between SLE with SjS and without SjS. They determine prognosis, approaches to pathogenetic therapy and monitoring.

Autoimmune hepatitis overlapping with subacute cutaneous lupus erythematosus: A case report

Autoimmune hepatitis (AIH) is an autoimmune liver disorder which rarely coexists with systemic lupus erythematosus (SLE) and presents as an overlap syndrome affecting multiple organs. Herein, we report a case of a 35-year-old healthy male who was evaluated for bilateral leg oedema and was diagnosed with AIH 5 years back. Four years later he developed fever and multiple photosensitive skin rashes, worsening leg oedema and proteinuria to which a diagnosis of coexisting SLE was made. He was diagnosed with the rare entity of AIH subacute cutaneous lupus erythematosus overlap syndrome. He was medically managed with immunosuppressive medication to which he responded.

Normocomplementemic urticarial vasculitis simulating subacute cutaneous lupus erythematosus

Urticarial vasculitis is a cutaneous disease characterized by recurring wheals along with histopathological evidence of leukocytoclastic vasculitis, along with potential systemic complications. Urticarial vasculitis clinically presents with erythematous wheals. The measurement of complement levels is useful in classifying urticarial vasculitis. Here, we describe a rare case of normocomplementemic urticarial vasculitis clinically mimicking subacute cutaneous lupus erythematosus in a young female.

Subacute cutaneous lupus erythematosus following ribociclib therapy for metastatic breast cancer.

Drug-induced subacute cutaneous lupus erythematosus (DI-SCLE) has been associated with drugs with different mechanisms of action, including anti-hypertensives, tumour necrosis factor-α inhibitors and even some chemotherapy medicines. In the last years, a few reports have been described in patients treated with cyclin-dependent kinase (CDK) 4/6 inhibitors, palbociclib and abemaciclib. Here, we describe a case of DI-SCLE in association with ribociclib and exemestane in a woman diagnosed with metastatic breast cancer. Topical mometasone was prescribed for two weeks with complete resolution of lesions, also abemaciclib was substituted for ribociclib, and the patient had stable disease with no relapse of DI-SCLE. To our knowledge, this is the first report of ribociclib-induced SCLE but based on the DI-SCLE reported cases associated others CDK4/6 inhibitors, the role of this family of drugs in dermatopathology must be further investigated.

Cutaneous lupus erythematosus is associated with an increased risk of cardiac and vascular diseases: a large-scale, propensity-matched global retrospective cohort study.

Autoimmune skin diseases can expedite various systemic sequelae involving other organs. Although limited to the skin, cutaneous lupus erythematosus (CLE) was noted to be associated with thromboembolic diseases. However, small cohort sizes, partially discrepant outcomes, missing data on CLE subtypes, and incomplete risk assessment limits these findings. The Global Collaborative Network of TriNetX provides access to medical records of more than 120 million patients worldwide. We used TriNetX to elucidate the risk for cardiac and vascular diseases after diagnosis of CLE, and its subtypes chronic discoid (DLE) and subacute cutaneous lupus erythematosus (SCLE). We included 30,315 CLE, 27,427 DLE, and 1613 SCLE patients. We performed propensity-matched cohort studies determining the risk to develop cardiac and vascular diseases (ICD10CM:I00-99) following diagnosis of CLE, DLE, or SCLE. Patients with systemic lupus erythematosus were excluded. We document that CLE and its subtype DLE but less so SCLE are associated with a higher risk for various cardiac and vascular diseases. This included predominantly thromboembolic events such as pulmonary embolism, cerebral infarction, and acute myocardial infarction, but also peripheral vascular disease and pericarditis. For example, the hazard ratio of arterial embolism and thrombosis was 1.399 (confidence interval: 1.230-1.591, p<0.0001) following CLE diagnosis. The study is limited by retrospective data collection and reliance on ICD10-disease classification. CLE and its major subtype DLE are associated with an increased risk for the development of a wide range of cardiac and vascular diseases. This research was funded by Deutsche Forschungsgemeinschaft (EXC 2167, CSSL/CS01-2022) and the Excellence-Chair Program of the State of Schleswig-Holstein.

CPC05 A case of extensive hypertrophic lupus erythematosus responsive to topical steroids

Abstract Hypertrophic lupus erythematosus (HLE) is a rare variant of lupus erythematosus, occurring in 2% of cases of chronic cutaneous lupus erythematosus (CCLE). It is an exaggerated proliferative epithelial response manifesting as verrucous-appearing hypertrophic indurated lesions occurring on sun-exposed sites. We present a case of the development of extensive HLE in a patient with subacute cutaneous lupus erythematosus (SCLE) with underlying pulmonary malignancy. A 74-year-old woman with Sjögren syndrome presented to dermatology with a 2-month history of a rash affecting her face and décolletage. Laboratory studies illustrated elevated antinuclear and anti-Ro antibodies. Biopsy from her chest confirmed SCLE. Malignancy screening revealed T1aN0 pulmonary lepidic adenocarcinoma, which was successfully managed with a lower lobectomy. Hydroxychloroquine was initially trialled but required discontinuation following intolerable side-effects. Mepacrine was then initiated, which allowed clearance of the rash, alongside yellow discoloration of the skin. Six months later, she developed a raised pruritic rash on her arms, legs and buttocks, resistant to current management alongside a trial of mycophenolate mofetil. Examination revealed violaceous hypertrophic plaques affecting 40% of her body-­surface area. The differential included hypertrophic lichen planus, nodular prurigo and HLE. Repeat lesional biopsies highlighted a lichenoid inflammatory infiltrate with overlying scale, favouring HLE. Owing to patient choice, topical mometasone furoate 0.1% was used to manage cutaneous disease, with a reduction in lesion size. HLE is a distinct subset of CCLE, regarded as a separate entity to SCLE. Twenty per cent of patients with SCLE also exhibit other types of cutaneous lupus erythematosus (CLE) lesions. No cases in the literature describe progression from SCLE to CCLE, or HLE, in particular. Hallmarks of HLE include verrucous lesions, chronicity and treatment resistance. Early recognition and follow-up are essential as malignancy may arise in long-standing lesions. Lung adenocarcinomas and hypertrophic lupus are both associated with p53 mutations and decreased granzyme B expression. Mutated p53 results in constitutive cell proliferation and DNA damage can accumulate in cells. Granzyme B is a protease in cytotoxic T cells involved in immune-mediated cell lysis. Both processes result in the potential to produce exaggerated hyperproliferative lesions, such as those observed in HLE and the lepidic adenocarcinomas. HLE is a rare clinical entity that can be easily misdiagnosed clinically and histopathologically. Increased awareness of this condition is essential to highlight the variety of clinical presentations of uncommon variants of CLE, to ensure they are considered while approaching clinical diagnosis.

P95 A single-centre retrospective study of the association between subacute cutaneous lupus erythematosus and malignancy

Abstract Subacute cutaneous lupus erythematosus (SCLE) is a rare photosensitive dermatosis that has been classically categorized as idiopathic or drug-induced. A proliferation of case reports and small case series support the hypothesis that the presentation of new-onset idiopathic SCLE can be a facultative paraneoplastic phenomenon. There remains a lack of consensus regarding the investigations that this dermatosis should initiate, and in which patients. The aims were to investigate the proportion of patients with biopsy-consistent SCLE who had an underlying malignancy, and to analyse the demographic, clinical and laboratory characteristics associated with this. We conducted a histopathology database word search of skin biopsy reports from 1995 to 2021. We performed a retrospective chart review of electronic and paper records and analysed demographic, clinical and laboratory data of patients with possible SCLE. This study received full ethical approval from the relevant local research ethics committee. Of 64 patients initially identified, 35 had SCLE following clinicopathological correlation and had sufficient data available for inclusion. In this cohort, a total of 16 (46%) patients had a malignancy, either preceding or following SCLE diagnosis. In eight (23%) patients, malignancy was diagnosed within 1 year of SCLE onset. SCLE was considered drug induced in 14 (40%) of cases. Eleven (31%) cases were considered to be idiopathic; of these, five (45%) were subsequently diagnosed with malignancy. Malignancies subsequent to the diagnosis of idiopathic SCLE all occurred in women (aged 66–74 years). There were no significant differences in the autoantibody profiles between patients with malignancy and the overall SCLE cohort. Malignancies were detected, on average, 41 days (range 11–74) after the diagnosis of SCLE. Subtypes of cancer diagnosed post-SCLE were lung (n = 4, all current or ex-smokers) and colorectal (n = 1, a never smoker). In four of these cases, investigation for malignancy was specifically advised by dermatology. This is the first reported retrospective study to explore the under-recognized association between SCLE and malignancy. Clinicians should be aware of the potential paraneoplastic nature of SCLE, especially in the context of risk factors such as smoking history and an older age profile.

BI16 (P41) An emerging role of interferon-targeted therapy for the treatment of cutaneous systemic lupus erythematosus: a prospective real-world evaluation of anifrolumab for refractory cutaneous lupus

Abstract Cutaneous lupus erythematosus (CLE), particularly discoid lupus erythematosus (DLE), is frequently unresponsive to standard therapies, including antimalarial and immunosuppressive therapies. Recent evidence has identified the importance of type-1 interferon (IFN-1) in the pathogenesis of disease. Anifrolumab is a monoclonal antibody that targets IFN-1 and has been licensed for the treatment of systemic lupus erythematosus (SLE). It is unclear which clinical subtypes of CLE are associated with an IFN-1 signature and may be responsive to anifrolumab therapy. We sought to evaluate the efficacy of anifrolumab on (i) DLE and (ii) rituximab-refractory CLE; and (iii) to compare IFN-specific biomarkers and (iv) early and late immunophenotypic and clinical responses. Anifrolumab was available through an early access to medicines scheme. Patients with SLE for whom the phenotype was predominantly cutaneous were treated with anifrolumab 300 mg intravenously every 4 weeks. The Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) and Dermatology Life Quality Index (DLQI) were reported. Seven patients were enrolled (five with DLE, one with chilblain and one with subacute CLE). All patients were female (median age 46 years; median disease duration 17 years). The median number of failed therapies was six: rituximab in six, belimumab in two and thalidomide in four patients. Four patients required long-term oral prednisolone &amp;gt; 10 mg daily. Median baseline CLASI activity score was 17 and DLQI was 16/30. Median CLASI score reduced after 4 weeks to 6 (range 0–13; P = 0.016) with a median decrease from baseline of 60% (range 19–100%), which was maintained at 6 months. Clinical responses were associated with significant improvements in DLQI (P &amp;lt; 0.001) and EQ-5D visual analogue scale (P = 0.002) by 3 months. All patients had a marked suppression of IFN-stimulated genes at 4 weeks (mean difference 2.92; P &amp;lt; 0.01). Treatment was generally well tolerated with the exception of one patient who had shingles complicated by sensorineural hearing loss while on anifrolumab and prednisolone &amp;gt; 15 mg daily. This required prolonged aciclovir for effective management. This case series highlights the potential role for anifrolumab in managing treatment-resistant CLE with particular responses observed in DLE and chilblain lupus. The need for enhanced awareness of viral infections, particularly varicella-zoster virus, needs to be considered in higher-risk patients.

P41 An emerging role of interferon targeted therapy for the treatment of cutaneous systemic lupus erythematosus: a prospective real-world evaluation of anifrolumab for refractory cutaneous lupus

Abstract Cutaneous lupus erythematosus (CLE), particularly discoid lupus erythematosus (DLE), is frequently unresponsive to standard therapies, including antimalarial and immunosuppressive therapies. Recent evidence has identified the importance of type-1 interferon (IFN-1) in the pathogenesis of disease. Anifrolumab is a monoclonal antibody that targets IFN-1 and has been licensed for the treatment of systemic lupus erythematosus (SLE). It is unclear which clinical subtypes of CLE are associated with an IFN-1 signature and may be responsive to anifrolumab therapy. We sought to evaluate the efficacy of anifrolumab on (i) DLE and (ii) rituximab-refractory CLE and (iii) to compare IFN-specific biomarkers and (iv) early and late immunophenotypic and clinical responses. Anifrolumab was available through an early access to medicines scheme. Patients with SLE for whom the phenotype was predominantly cutaneous were treated with anifrolumab 300 mg intravenously every 4 weeks. The Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) and Dermatology Life Quality Index (DLQI) were reported. Seven patients were identified (five with DLE, one with chilblain and one with subacute CLE were enrolled). All patients were female (median age 46 years; median disease duration 17 years). The median number of failed therapies was six: rituximab in six, belimumab in two and thalidomide in four patients. Four patients required long-term oral prednisolone &amp;gt; 10 mg daily. The median baseline CLASI activity score was 17 and DLQI was 16/30. The median CLASI score reduced after 4 weeks to 6 (range 0–13; P = 0.016) with a median decrease from baseline of 60% (range 19–100%), which was maintained at 6 months. Clinical responses were associated with significant improvements in DLQI (P &amp;lt; 0.001) and the EQ-5D visual analogue scale (P = 0.002) by 3 months. All patients had a marked suppression of IFN-stimulated genes at 4 weeks (mean difference 2.92; P &amp;lt; 0.01). Treatment was generally well tolerated with the exception of one patient who had shingles complicated by sensorineural hearing loss while on anifrolumab and prednisolone &amp;gt; 15 mg daily. This required prolonged aciclovir for effective management. This case series highlights the potential role for anifrolumab in managing treatment-resistant CLE with particular responses observed in DLE and chilblain lupus. The need for enhanced awareness of viral infections, particularly varicella-zoster virus, needs to be considered in higher-risk patients.