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OBJECTIVE: While the incidence and type of blood malignancies are well documented amid primary Sjögren's syndrome patients (pSS), data focusing on solid neoplasms are more conflicting. We aimed to describe clinical, pathological, and immunological characteristics of pSS patients with cancers, along with the chronological interplay between the two conditions. METHODS: Outcomes concerning both pSS and cancer were retrospectively collected from Montpellier University Hospital (tertiary center) between 2019 and 2020. pSS characteristics were compared to a control group of pSS patients without cancer. RESULTS: A total of 165 patients with pSS were included: 55 patients with cancer (52 female, mean age 58.4 ± 10.4 years at pSS diagnosis; mean follow-up 10.5 ± 10.1 years, 12 patients had multiple cancers) and 110 controls without cancer. Characteristics of pSS patients with cancers were different from controls mostly for lymphoma prognosis factors. Among the 70 cancers, we recorded 55 solid neoplasms (whom 27 breast cancers and 8 lung cancers, and 82% of adenocarcinomas), with no evidence of disease at the end of follow-up in 85% of them. Among the 15 recorded blood malignancies, ten were lymphomas with an excellent prognosis. Regarding chronological interplay between cancer and pSS, most cancers (43%) were diagnosed close (± 5 years) to pSS diagnosis. Breast cancers were diagnosed before or close to pSS diagnosis (mean delay - 1.8 ± 13.0 years), at an early stage, with only two relapses (no cancer-related death), while lung cancers were diagnosed late after. CONCLUSIONS: The tight chronological interplay between breast cancer and pSS and the intriguing pathological and immunological pattern of pSS in these patients suggest a hypothesis of immune control of cancer.
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Neoplasias Pulmonares , Linfoma , Síndrome de Sjogren , Humanos , Feminino , Pessoa de Meia-Idade , Idoso , Síndrome de Sjogren/complicações , Síndrome de Sjogren/diagnóstico , Estudos Retrospectivos , Recidiva Local de Neoplasia , Linfoma/terapiaRESUMO
BACKGROUND: Inflammation of unknown origin (IUO) is a challenging situation in internal medicine. OBJECTIVES: To describe the final diagnoses in IUO and assess the helpfulness of 18 F-fluorodesoxyglucose positron emission tomography with computerized tomography (18 F-FDG-PET/CT) in the diagnosis strategy. RESULTS: A total of 317 IUO patients with 18 F-FDG-PET/CT were enrolled. A diagnosis was reached in 228 patients: noninfectious inflammatory diseases (NIID) (37.5%), infectious diseases (18.6%), malignancies (7.9%), and non-systemic-inflammatory miscellaneous diseases (7.9%). The two leading causes of NIID were polymyalgia rheumatica and giant cell arteritis. 18 F-FDG-PET/CT results were classified as true positive in 49.8% of patients and contributory in 75.1% of overall IUO patients (after the complete investigation set and a prolonged follow-up). In multivariate analysis, only C-reactive protein minimum level (≥50 mg/L) was associated with the contributory status of 18 F-FDG-PET/CT. CONCLUSION: Within the wide spectrum of IUO underlying diseases, 18 F-FDG-PET/CT is helpful to make a diagnosis and to eliminate inflammatory diseases. Obese patients constitute a specific group needing further studies.
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Febre de Causa Desconhecida , Doenças não Transmissíveis , Febre de Causa Desconhecida/diagnóstico por imagem , Febre de Causa Desconhecida/etiologia , Fluordesoxiglucose F18 , Humanos , Inflamação/complicações , Inflamação/diagnóstico por imagem , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada/métodos , Tomografia por Emissão de Pósitrons/efeitos adversosRESUMO
OBJECTIVES: To describe the characteristics, treatment and outcome of patients with immune thrombocytopenia with clinical significance (ITPCS) associated with SLE. METHODS: This retrospective multicentre study included SLE patients who experienced ≥1 ITPCS (defined as ITP with attributable bleeding disorders and/or a platelet count <30×109/l). Other causes of secondary thrombocytopenia were excluded. Major bleeding event (MBG) was defined as Khellaf score >8 and/or WHO score >2. RESULTS: A total of 90 patients were included, the median (range) follow-up duration was 80 (6-446) months. ITP was diagnosed before SLE in 25 patients. They presented a high rate of autoimmune haemolytic anaemia (15%), antiphospholipid antibody (62%) and antiphospholipid syndrome (19%). The 25 (28%) patients who experienced MBG had significantly more bleedings at ITP diagnosis and higher bleeding scores, and serositis and thrombosis during follow-up. They required significantly more treatment lines, transfusions and hospitalizations. The 11 (12%) patients who experienced no bleeding event presented a significantly more restricted SLE phenotype (cutaneous and/or articular). Patients received a mean (range) of 4.2 (1-11) treatment lines. Corticosteroids and HCQ allowed ITPCS overall response in one-third of patients. The median relapse-free survival of rituximab (n = 34), AZA (n = 19), MMF (n = 8), thrombopoietin-receptor agonists (n = 16) and splenectomy (n = 19) were 53, 31.5, 61, 24.5 and 78 months, respectively. Four patients experienced thrombotic events after splenectomy and one occurred under thrombopoietin-receptor agonist treatment. CONCLUSION: SLE-ITCS patients displayed a high rate of haematological abnormalities and MBG patients exhibited higher morbidity. Management of thrombocytopenia was highly heterogeneous and many options seem viable.
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Lúpus Eritematoso Sistêmico , Púrpura Trombocitopênica Idiopática , Trombocitopenia , Trombose , Humanos , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Púrpura Trombocitopênica Idiopática/complicações , Púrpura Trombocitopênica Idiopática/epidemiologia , Púrpura Trombocitopênica Idiopática/terapia , Receptores de Trombopoetina/agonistas , Estudos Retrospectivos , Trombocitopenia/tratamento farmacológico , Trombose/tratamento farmacológicoRESUMO
OBJECTIVE: Angioimmunoblastic T-cell lymphoma (AITL) is frequently associated with autoimmune cytopenia (AIC). Whether such patients have a particular phenotype and require particular management is unclear. METHOD: Angioimmunoblastic T-cell lymphoma patients from the multicentric database of the Lymphoma Study Association presenting with AIC during disease course were included and matched to AITL patients without AIC (1/5 ratio). RESULTS: At diagnosis, AIC patients (n = 28) had more spleen and bone marrow involvement (54% vs 19% and 71% vs 34%, P < 0.001), Epstein-Barr virus replication (89% vs 39%, P < 0.001), gamma globulin titers (median 23 vs 15 g/L, P = 0.002), and proliferating B cells and plasmablasts in biopsies, as compared to control patients (n = 136). The 28 AIC patients had 41 episodes of AIC, diagnosed concomitantly with AITL in 23 (82%) cases. After a median follow-up of 24 months (range 3-155), 10 patients relapsed, all associated with AITL relapse. CONCLUSION: Our results provide new insight into AIC associated with AITL by highlighting the significant interplay between AITL and B-cell activation leading to subsequent autoimmunity.
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Doenças Autoimunes/complicações , Linfadenopatia Imunoblástica/diagnóstico , Linfadenopatia Imunoblástica/terapia , Linfoma de Células T/diagnóstico , Linfoma de Células T/terapia , Pancitopenia/complicações , Adulto , Idoso , Idoso de 80 Anos ou mais , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Doenças Autoimunes/diagnóstico , Biópsia , Gerenciamento Clínico , Suscetibilidade a Doenças , Feminino , Humanos , Linfadenopatia Imunoblástica/etiologia , Linfadenopatia Imunoblástica/mortalidade , Imunoglobulinas Intravenosas/uso terapêutico , Linfoma de Células T/etiologia , Linfoma de Células T/mortalidade , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Pancitopenia/diagnóstico , Fenótipo , Estudos Retrospectivos , Avaliação de Sintomas , Resultado do TratamentoAssuntos
Medula Óssea/irrigação sanguínea , Medula Óssea/patologia , Granulomatose com Poliangiite/patologia , Anemia/sangue , Anemia/etiologia , Angiodisplasia/complicações , Angiodisplasia/patologia , Angiodisplasia/terapia , Anticorpos Anticitoplasma de Neutrófilos/imunologia , Epistaxe/etiologia , Hemorragia Gastrointestinal/complicações , Hemorragia Gastrointestinal/patologia , Hemorragia Gastrointestinal/terapia , Granulomatose com Poliangiite/sangue , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/diagnóstico por imagem , Hemostase Endoscópica , Humanos , Masculino , Mastoidite/diagnóstico por imagem , Mastoidite/etiologia , Pessoa de Meia-Idade , Mieloblastina/imunologia , Necrose , Trombocitopenia/sangue , Trombocitopenia/etiologiaAssuntos
Anemia Falciforme , Reação Transfusional , Anemia Falciforme/terapia , Hemólise , Humanos , IsoanticorposAssuntos
Fístula Cutânea/tratamento farmacológico , Glucocorticoides/uso terapêutico , Mastite Granulomatosa/tratamento farmacológico , Prednisona/uso terapêutico , Adulto , Aleitamento Materno , Fístula Cutânea/diagnóstico por imagem , Fístula Cutânea/patologia , Progressão da Doença , Feminino , Mastite Granulomatosa/diagnóstico por imagem , Mastite Granulomatosa/patologia , Humanos , Período Pós-Parto , Gravidez , Complicações na Gravidez/diagnóstico por imagem , Complicações na Gravidez/patologia , CicatrizaçãoRESUMO
OBJECTIVES: We performed a systematic review and meta-analysis of randomised controlled trials (RCTs) of psoriatic arthritis (PsA) to examine the effect of tumour necrosis factor (TNF) blockers on radiographic progression, and to determine whether treatment combining TNF blocker with methotrexate (MTX) was superior to TNF-blocker monotherapy. METHODS: We systematically reviewed articles published up to December 2012 in Embase and Medline, and from the two last EUropean League Against Rheumatism (EULAR) and American College or Rheumatology (ACR) meetings. The primary endpoint was the proportion of patients with no radiographic progression (non-progressors) at treatment week 24 (defined by change in modified total Sharp score (mTSS) ≤0.5). The Mantel-Haenszel method was used to estimate ORs and 95% CIs of the effect of TNF blockers (with or without MTX) versus placebo (with or without MTX). Statistical heterogeneity was assessed by χ² test. RESULTS: The search retrieved 207 articles; 5 (1110 patients) met the meta-analysis criteria. For patients receiving TNF blockers, 494/584 (84.5%) were considered non-progressors at treatment week 24 vs 362/526 (68.8%) receiving placebo (OR 2.68 (95% CI 1.99 to 3.60) p<0.001), without significant heterogeneity (I(2)=3%; p=0.39). Only three RCTs provided data on potential additional efficacy of MTX: two did not find significant difference, one suggested a benefit of combined therapy. CONCLUSIONS: For patients with PsA, control of structural damage is better at week 24 with TNF blockers than placebo. Due to the limited data, we were unable to conclude on the potential additional effect of MTX on structural damages.
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Antirreumáticos/uso terapêutico , Artrite Psoriásica/tratamento farmacológico , Produtos Biológicos/uso terapêutico , Ensaios Clínicos Controlados Aleatórios como Assunto , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Artrite Psoriásica/diagnóstico por imagem , Progressão da Doença , Quimioterapia Combinada , Humanos , Metotrexato/uso terapêutico , Radiografia , Resultado do TratamentoRESUMO
BACKGROUND: auto-antibodies against the potassium channel inward rectifying potassium channel 4.1 (Kir4.1) have previously been identified in 46% of patients with multiple sclerosis (MS). OBJECTIVES: to confirm these findings. METHODS: we evaluated the presence of anti-Kir4.1 antibodies by enzyme-linked immunosorbent assay (ELISA) and immunofluorescence in 268 MS patients, 46 patients with other neurological diseases (OND) and 45 healthy controls. RESULTS: anti-Kir4.1 antibodies were found in 7.5% of MS patients, 4.3% of OND patients and 4.4% of healthy controls. Immunofluorescence analysis did not identify any specific staining. CONCLUSIONS: we confirmed the presence of anti-Kir4.1 antibodies in MS patients, but at a much lower prevalence than previously reported.
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Autoanticorpos/sangue , Esclerose Múltipla/imunologia , Canais de Potássio Corretores do Fluxo de Internalização/imunologia , Adulto , Autoanticorpos/imunologia , Autoantígenos/imunologia , Biomarcadores/análise , Ensaio de Imunoadsorção Enzimática , Feminino , Imunofluorescência , Humanos , Masculino , Esclerose Múltipla/sangueRESUMO
OBJECTIVE: The objective of this study was to evaluate the association between frailty status and risk of readmissions, inpatient death, and cost of admission among patients with systemic lupus erythematosus (SLE). METHODS: We conducted a retrospective cohort study using the National Readmissions Database. Using International Statistical Classification of Diseases, Tenth Revision codes, we identified individuals >18 years of age who had a primary or secondary diagnosis of SLE and were hospitalized between January and June 2018. Using the validated claims-based Hospital Frailty Risk Score, we categorized individuals as frail (score ≥ 5) or nonfrail (score < 5) at the time of index hospitalization. Our primary outcome was readmission rates post discharge from index hospitalization. Secondary outcomes were rates of inpatient mortality and the total cost of hospitalizations. Cox proportional hazard models were used to estimate the association between frailty and risk of readmissions, with adjustment for age, sex, insurance type, household income, and Elixhauser Comorbidity Index score. RESULTS: A total of 39,738 patients with SLE met eligibility criteria. Over a median follow-up of eight months, frail patients with SLE (n = 18,385) had higher Elixhauser Comorbidity Index scores and longer length of stay compared to nonfrail patients with SLE (n = 21,353). Frail patients with SLE had higher readmission rates, a higher proportion of prolonged hospitalizations, and higher costs per hospitalization. Frailty was independently associated with a 10% higher risk of readmission after adjustment for covariates. CONCLUSION: Among hospitalized adults with SLE, presence of frailty was associated with higher readmission and inpatient mortality rates. Our results highlight that frailty status can help risk stratify patients with SLE at increased risk for readmissions and other adverse health outcomes.
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INTRODUCTION: Involvement of subcutaneous tissue in idiopathic inflammatory myopathies (IIM) is poorly known. METHODS: We conducted a systematic review of the literature regarding panniculitis and lipodystrophy/lipoatrophy in juvenile and adult IIM via PubMed/Medline, Embase and Scopus databases. Three local observations are included in this review. Epidemiological, clinical, paraclinical and therapeutic data were collected. RESULTS: Panniculitis appears to be more common in adults than in juveniles. It was mainly localised in the upper and lower limbs. Panniculitis improved in most cases with steroids and panniculitis and myositis had a similar course in 83.3% and 72.2% of cases in juveniles and adults, respectively. Lipodystrophy appeared to be more frequent in juveniles and was only observed in dermatomyositis in both juveniles and adults. Lipodystrophy was mainly partial in juveniles and adults. The median time from myositis to the diagnosis of lipodystrophy was 6 years [0-35] and 2.5 years [0-10] in juveniles and adults, respectively. Lipodystrophy was associated with anti-TIF1 gamma auto-antibody positivity, a polycyclic/chronic course of myositis and the occurrence of calcinosis and might be an indicator of poor disease control. CONCLUSION: Adipose tissue involvement, particularly lipodystrophy, occurs almost exclusively in dermatomyositis. The insidious onset and lack of awareness of the diagnosis may underestimate its prevalence. Larger studies are needed to identify possible risk factors in these patients, to better potential underlying pathophysiological process, in order to discuss potential therapeutic targets.
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Dermatomiosite , Lipodistrofia , Miosite , Paniculite , Adulto , Humanos , Dermatomiosite/complicações , Tela Subcutânea , Autoanticorpos , Paniculite/complicações , Lipodistrofia/complicaçõesRESUMO
OBJECTIVE: Giant cell arteritis (GCA) has a relapsing-remitting course and is associated with a high burden of comorbidities, leading to repeated hospitalizations. This study was undertaken to investigate the burden, risk factors, causes, and outcomes of hospitalization and readmission in GCA patients in a US national cohort. METHODS: Using the 2017 US National Readmission Database, we identified adults ≥50 years of age hospitalized with GCA between January and June 2017, with at least 6 months of follow-up. We estimated the burden of hospitalization including 6-month risk of readmission, total days spent in hospital, and costs, annually. We examined patient-, hospital-, and index hospitalization-related factors for 6-month readmission and total days of hospitalization using binomial logistic regression. RESULTS: Our study included 1,206 patients hospitalized with GCA (70% women, median age 77 years), with 13% of patients experiencing GCA-related ophthalmologic complications at index hospital admission. On follow-up, 3% died, and 34% of patients were readmitted within 6 months, primarily for infections (23%) and cardiovascular diseases (CVDs) (15%). Charlson comorbidity index (CCI) of ≥1, smoking, and obesity were associated with readmission. GCA patients spent a median of 5 days/year in hospital (interquartile range [IQR] 3-11), with those in the top quartile spending 19 days/year in hospital (IQR 14-26). CONCLUSION: GCA patients frequently experience unplanned health care utilization, with 1 in 3 patients experiencing readmission within 6 months, and 3% dying within the follow-up period. Infection and CVDs are common causes of readmission and may be related to glucocorticoid exposure. Population health management strategies are required in these vulnerable GCA patients.
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Arterite de Células Gigantes , Adulto , Humanos , Feminino , Idoso , Masculino , Estudos de Coortes , Arterite de Células Gigantes/epidemiologia , Arterite de Células Gigantes/terapia , Arterite de Células Gigantes/complicações , Estudos Retrospectivos , Hospitalização , Fatores de Risco , Readmissão do PacienteRESUMO
INTRODUCTION: Internal medicine departments manage patients referred by emergency departments or private practitioners. Considering the overcrowding of emergency departments and lack of beds for inpatients, this specialty must be part of an "ambulatory shift", particularly by strengthening the links between community and hospital medicine. Our objective was to evaluate a new care pathway in internal medicine at Nîmes university hospital. METHODS: Our department has developed the RAPIDO project (Réseau d'Aide à la PrIse en Charge Diagnostique et d'Orientation). The referring general practitioner contacts a senior internist on a dedicated phone line. After careful evaluation, he may offer a consultation within 15 days. A summary report is then given to the patient. RESULTS: Between November 2020 and November 2021, 254 patients were seen via RAPIDO. The average call-consultation time period was 6.4 (±4.5) days, for symptoms lasting for 2 weeks to 3 months in 43% (n=109) of cases. The reason for the call was a suspicion of systemic disease in 28% of cases (n=84), or a dysfunction of an organ in 16%. A diagnosis was made in 89% of cases. The budget of the whole procedure was balanced. CONCLUSION: A quick internal medicine consultation pathway for general practitioners seems to be a relevant, feasible and economically viable healthcare trajectory, which can be transposed to any type of healthcare institution, as soon as sufficient human resources are dedicated.
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Clínicos Gerais , Medicina Interna , Masculino , Humanos , Medicina Interna/métodos , Hospitais Universitários , Prática Privada , Serviço Hospitalar de EmergênciaRESUMO
Objective: To identify the key coding genes underlying the biomarkers and pathways associated with giant cell arteritis (GCA), we performed an in situ spatial profiling of molecules involved in the temporal arteries of GCA patients and controls. Furthermore, we performed pharmacogenomic network analysis to identify potential treatment targets. Methods: Using human formalin-fixed paraffin-embedded temporal artery biopsy samples (GCA, n = 9; controls, n = 7), we performed a whole transcriptome analysis using the NanoString GeoMx Digital Spatial Profiler. In total, 59 regions of interest were selected in the intima, media, adventitia, and perivascular adipose tissue (PVAT). Differentially expressed genes (DEGs) (fold-change > 2 or < -2, p-adjusted < 0.01) were compared across each layer to build a spatial and pharmacogenomic network and to explore the pathophysiological mechanisms of GCA. Results: Most of the transcriptome (12,076 genes) was upregulated in GCA arteries, compared to control arteries. Among the screened genes, 282, 227, 40, and 5 DEGs were identified in the intima, media, adventitia, and PVAT, respectively. Genes involved in the immune process and vascular remodeling were upregulated within GCA temporal arteries but differed across the arterial layers. The immune-related functions and vascular remodeling were limited to the intima and media. Conclusion: This study is the first to perform an in situ spatial profiling characterization of the molecules involved in GCA. The pharmacogenomic network analysis identified potential target genes for approved and novel immunotherapies.
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Arterite de Células Gigantes , Humanos , Arterite de Células Gigantes/patologia , Artérias Temporais , Remodelação Vascular , Artérias/patologia , Biomarcadores/metabolismoRESUMO
We aimed to assess the factors associated with mortality in patients treated with tocilizumab for a SARS-CoV-2 pneumonia due to the delta or omicron variants of concern (VOC) and detect an effect of tocilizumab on mortality. We conducted a prospective cohort study in a tertiary hospital from 1 August 2021 to 31 March 2022 including patients with severe COVID-19, treated with tocilizumab. Factors associated with mortality were assessed in a Cox model; then, the 60-day mortality rates of COVID-19 patients treated with standard of care (SoC) +/- tocilizumab were compared after 1:1 propensity score matching. The mortality rate was 22% (N = 26/118) and was similar between delta and omicron cases (p = 0.6). The factors independently associated with mortality were age (HR 1.06; 95% CI (1.02-1.11), p = 0.002), Charlson index (HR 1.33; 95% CI (1.11-1.6), p = 0.002), WHO-CPS (HR 2.56; 95% CI (1.07-6.22) p = 0.03), and tocilizumab infusion within the first 48 h following hospital admission (HR 0.37, 95% CI (0.14-0.97), p = 0.04). No significant differences in mortality between the tocilizumab plus SoC and SoC alone groups (p = 0.5) were highlighted. However, the patients treated with tocilizumab within the 48 h following hospital admission had better survival (p = 0.04). In conclusion, our results suggested a protective effect on mortality of the early administration of tocilizumab in patients with severe COVID-19 regardless of the VOC involved.
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OBJECTIVES: CTLA4 deficiency (CTLA4d) is a disease with multisystem autoimmune features, including neurologic manifestations. We aimed to describe neurologic involvement in these patients. METHODS: We performed a cross-sectional observational study using the French Reference Centre for Primary Immunodeficiencies (CEREDIH) registry plus a surveillance in national society networks. Participants with confirmed CTLA4d and neurologic involvement were included. Clinical, laboratory, and radiologic features were collected, as well as treatments. Available MRI was double-reviewed. RESULTS: Among 70 patients with CTLA4d, 13 patients (21%) had neurologic involvement. Neurologic symptoms began at a median age of 18 [15-45] years, mostly occurring after systemic manifestations (median delay: 8.5 [4.5-10.5] years). Main symptoms included headaches, focal deficit (54% each), and seizures (38%). MRI detected at least 1 large contrast-enhancing lesion in 8 patients. Lesions reminiscent of multiple sclerosis lesions were found in 6 patients. Cerebellar (6 patients) and large spinal cord lesions (3 patients) were common. Ten patients were treated with abatacept, of whom 9 (90%) showed good clinical and radiologic response. DISCUSSION: Neurologic involvement is common among patients with CTLA4d. Despite its rarity, and considering the suspected efficacy of abatacept, neurologists should be aware of the characteristics of CTLA4d neurologic involvement.
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Esclerose Múltipla , Doenças da Medula Espinal , Humanos , Adolescente , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Antígeno CTLA-4/genética , Abatacepte/uso terapêutico , Estudos TransversaisRESUMO
OBJECTIVES: Systemic sclerosis (SSc) can cause malnutrition due to frequent gastrointestinal involvement. However, prevalence of malnutrition in SSc is poorly known. The aim of this study was to evaluate the prevalence of malnutrition in SSc and its potential associations with disease features in patients from a tertiary referral center. METHODS: All patients meeting American College of Rheumatology/European Alliance of Associations for Rheumatology criteria for SSc followed between January 1, 1985, and January 1, 2019, at the Department of Internal Medicine, Saint Eloi University Hospital, were included. Malnutrition was assessed using the 2020 French recommendations for SSc and the malnutrition universal screening tool score. Severe malnutrition was defined via the French Haute Autorité de Santé (National Health Authority) 2007 criteria. RESULTS: A total of 120 patients were included, with mean age 64 (± 15) y and a female-to-male sex ratio of 5:1. According to 2020 French recommendations, 71 patients (59.2%) were malnourished and 30 (25%) had at least one criterion of severe malnutrition. With the malnutrition universal screening tool score, 41.7%, 20%, and 38.3%, respectively, had low, medium, and high risk of malnutrition. Multivariate analysis revealed the following results: 1) malnutrition was associated with cardiac involvement (P < 0.01); 2) a high malnutrition universal screening tool score was also associated with specific cardiac involvement (P < 0.01); and 3) severe malnutrition was strongly correlated with interincisal distance <35 mm (P = 0.02). CONCLUSIONS: Malnutrition affects more than half of SSc patients and is associated with specific cardiac involvement. Interincisal distance <35 mm could be a red flag for severe malnutrition in SSc.