Marta Mosca1, Karen H Costenbader2, Sindhu R Johnson3, Valentina Lorenzoni4, Gian Domenico Sebastiani5, Bimba F Hoyer6, Sandra Navarra7, Eloisa Bonfa8, Rosalind Ramsey-Goldman9, Jorge Medina-Rosas3, Matteo Piga10, Chiara Tani1, Sara K Tedeschi2, Thomas Dörner11, Martin Aringer12, Zahi Touma3. 1. University of Pisa, Pisa, Italy. 2. Brigham and Women's Hospital and Harvard Medical School, Boston, Massachusetts. 3. Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto, Ontario, Canada. 4. Scuola Superiore Sant'Anna, Pisa, Italy. 5. Azienda Ospedaliera San Camillo-Forlanini, Rome, Italy. 6. Christian-Albrechts-University and University Hospital Schleswig-Holstein, Kiel, Germany. 7. University of Santo Tomas, Manila, Philippines. 8. Hospital das Clinicas HCFMUSP, Faculdade de Medicina da Universidade de Sao Paulo, Sao Paulo, Brazil. 9. Northwestern University Feinberg School of Medicine, Chicago, Illinois. 10. AOU University Clinic of Cagliari, Cagliari, Italy. 11. Charité University Medicine Berlin and Deutsches Rheuma-Forschungszentrum, Berlin, Germany. 12. University Medical Center and Faculty of Medicine Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany.
Abstract
OBJECTIVE: Systemic lupus erythematosus (SLE) presents with nonspecific signs and symptoms that are also found in other conditions. This study aimed to evaluate manifestations at disease onset and to compare early SLE manifestations to those of diseases mimicking SLE. METHODS: Academic lupus centers in Asia, Europe, North America, and South America collected baseline data on patients who were referred to them during the previous 3 years for possible SLE and who had a symptom duration of <1 year. Clinical and serologic manifestations were compared between patients diagnosed as having SLE and those diagnosed as having SLE-mimicking conditions. Diagnostic performance of the 1997 American College of Rheumatology (ACR) SLE classification criteria and the 2012 Systemic Lupus International Collaborating Clinics (SLICC) SLE classification criteria was tested. RESULTS: Data were collected on 389 patients with early SLE and 227 patients with SLE-mimicking conditions. Unexplained fever was more common in early SLE than in SLE-mimicking conditions (34.5% versus 13.7%, respectively; P < 0.001). Features less common in early SLE included Raynaud's phenomenon (22.1% versus 48.5%; P < 0.001), sicca symptoms (4.4% versus 34.4%; P < 0.001), dysphagia (0.3% versus 6.2%; P < 0.001), and fatigue (28.3% versus 37.0%; P = 0.024). Anti-double-stranded DNA, anti-β2 -glycoprotein I antibodies, positive Coombs' test results, autoimmune hemolytic anemia, hypocomplementemia, and leukopenia were more common in early SLE than in SLE-mimicking conditions. Symptoms detailed in the ACR and SLICC classification criteria were significantly more frequent among those with early SLE. Fewer patients with early SLE were not identified as having early SLE with use of the SLICC criteria compared to the ACR criteria (16.5% versus 33.9%), but the ACR criteria demonstrated higher specificity than the SLICC criteria (91.6% versus 82.4%). CONCLUSION: In this multicenter cohort, clinical manifestations that could help to distinguish early SLE from SLE-mimicking conditions were identified. These findings may aid in earlier SLE diagnosis and provide information for ongoing initiatives to revise SLE classification criteria.
OBJECTIVE: Systemic lupus erythematosus (SLE) presents with nonspecific signs and symptoms that are also found in other conditions. This study aimed to evaluate manifestations at disease onset and to compare early SLE manifestations to those of diseases mimicking SLE. METHODS: Academic lupus centers in Asia, Europe, North America, and South America collected baseline data on patients who were referred to them during the previous 3 years for possible SLE and who had a symptom duration of <1 year. Clinical and serologic manifestations were compared between patients diagnosed as having SLE and those diagnosed as having SLE-mimicking conditions. Diagnostic performance of the 1997 American College of Rheumatology (ACR) SLE classification criteria and the 2012 Systemic Lupus International Collaborating Clinics (SLICC) SLE classification criteria was tested. RESULTS: Data were collected on 389 patients with early SLE and 227 patients with SLE-mimicking conditions. Unexplained fever was more common in early SLE than in SLE-mimicking conditions (34.5% versus 13.7%, respectively; P < 0.001). Features less common in early SLE included Raynaud's phenomenon (22.1% versus 48.5%; P < 0.001), sicca symptoms (4.4% versus 34.4%; P < 0.001), dysphagia (0.3% versus 6.2%; P < 0.001), and fatigue (28.3% versus 37.0%; P = 0.024). Anti-double-stranded DNA, anti-β2 -glycoprotein I antibodies, positive Coombs' test results, autoimmune hemolytic anemia, hypocomplementemia, and leukopenia were more common in early SLE than in SLE-mimicking conditions. Symptoms detailed in the ACR and SLICC classification criteria were significantly more frequent among those with early SLE. Fewer patients with early SLE were not identified as having early SLE with use of the SLICC criteria compared to the ACR criteria (16.5% versus 33.9%), but the ACR criteria demonstrated higher specificity than the SLICC criteria (91.6% versus 82.4%). CONCLUSION: In this multicenter cohort, clinical manifestations that could help to distinguish early SLE from SLE-mimicking conditions were identified. These findings may aid in earlier SLE diagnosis and provide information for ongoing initiatives to revise SLE classification criteria.
Authors: Sara K Tedeschi; Sindhu R Johnson; Dimitrios T Boumpas; David Daikh; Thomas Dörner; Betty Diamond; Søren Jacobsen; David Jayne; Diane L Kamen; W Joseph McCune; Marta Mosca; Rosalind Ramsey-Goldman; Guillermo Ruiz-Irastorza; Matthias Schneider; Murray Urowitz; David Wofsy; Josef S Smolen; Raymond P Naden; Martin Aringer; Karen H Costenbader Journal: Ann Rheum Dis Date: 2019-01-28 Impact factor: 19.103
Authors: Martin Aringer; Karen Costenbader; David Daikh; Ralph Brinks; Marta Mosca; Rosalind Ramsey-Goldman; Josef S Smolen; David Wofsy; Dimitrios T Boumpas; Diane L Kamen; David Jayne; Ricard Cervera; Nathalie Costedoat-Chalumeau; Betty Diamond; Dafna D Gladman; Bevra Hahn; Falk Hiepe; Søren Jacobsen; Dinesh Khanna; Kirsten Lerstrøm; Elena Massarotti; Joseph McCune; Guillermo Ruiz-Irastorza; Jorge Sanchez-Guerrero; Matthias Schneider; Murray Urowitz; George Bertsias; Bimba F Hoyer; Nicolai Leuchten; Chiara Tani; Sara K Tedeschi; Zahi Touma; Gabriela Schmajuk; Branimir Anic; Florence Assan; Tak Mao Chan; Ann Elaine Clarke; Mary K Crow; László Czirják; Andrea Doria; Winfried Graninger; Bernadett Halda-Kiss; Sarfaraz Hasni; Peter M Izmirly; Michelle Jung; Gábor Kumánovics; Xavier Mariette; Ivan Padjen; José M Pego-Reigosa; Juanita Romero-Diaz; Íñigo Rúa-Figueroa Fernández; Raphaèle Seror; Georg H Stummvoll; Yoshiya Tanaka; Maria G Tektonidou; Carlos Vasconcelos; Edward M Vital; Daniel J Wallace; Sule Yavuz; Pier Luigi Meroni; Marvin J Fritzler; Ray Naden; Thomas Dörner; Sindhu R Johnson Journal: Arthritis Rheumatol Date: 2019-08-06 Impact factor: 15.483