{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,11]],"date-time":"2026-03-11T15:45:25Z","timestamp":1773243925135,"version":"3.50.1"},"reference-count":42,"publisher":"MDPI AG","issue":"9","license":[{"start":{"date-parts":[[2023,5,4]],"date-time":"2023-05-04T00:00:00Z","timestamp":1683158400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare autoimmune disorder of the peripheral nervous system. Diagnosis relies on clinical and electrophysiological criteria. Various disorders requiring specific treatment regimens may be associated with CIDP, including sarcoidosis (SAR-CIDP) and connective tissue disease (CTD-CIDP). Therefore, it is important to distinguish between CIDP, SAR-CIDP and CTD-CIDP. In this retrospective monocentric study, we analyzed 16 patients with SAR-CIDP and 11 with CTD-CIDP and compared them with a group of 17 patients with idiopathic CIDP. SAR-CIDP patients had a frequently acute or subacute CIDP onset. CTD-CIDPs were mostly Sj\u00f6gren\u2019s syndrome and lupus, and patients had a chronic onset. An older age at onset (64.5 vs. 54 years, p = 0.04), more atypical presentation (19\/25 (76%) vs. 6\/17 (35%), p = 0.008), acute\/subacute onset of symptoms (15\/25 (60%) vs. 1\/17 (6%), p = 0.0004) and more frequent weight loss (7\/16 (44%) vs. 0\/17 (0%), p = 0.017) were identified SAR-CIDP and CTD-CIDP groups. Response to intravenous immunoglobulin therapy was lower in the combined SAR-CIDP and CTD-CIDP group (44% versus 82%, p = 0.005). As sarcoidosis and CTDs might be associated with CIDP and require specific management, the \u201cred flags\u201d mentioned above should be kept in mind by clinicians managing patients with CIDP.<\/jats:p>","DOI":"10.3390\/jcm12093281","type":"journal-article","created":{"date-parts":[[2023,5,5]],"date-time":"2023-05-05T02:08:42Z","timestamp":1683252522000},"page":"3281","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Red Flags for Chronic Inflammatory Demyelinating Polyradiculoneuropathy Associated with Sarcoidosis or Connective Tissue Diseases"],"prefix":"10.3390","volume":"12","author":[{"given":"Cl\u00e9ment","family":"Vialatte de P\u00e9mille","sequence":"first","affiliation":[{"name":"Neurology Department, AP-HP, CHU de Bic\u00eatre, 78 Rue du General Leclerc, 94270 Le Kremlin Bicetre, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4055-617X","authenticated-orcid":false,"given":"Nicolas","family":"No\u00ebl","sequence":"additional","affiliation":[{"name":"Internal Medicine Departement, CHU de Bic\u00eatre, 78 Rue du General Leclerc, 94270 Le Kremlin Bicetre, France"},{"name":"Faculty of Medicine, Paris Saclay University, 63 Rue Gabriel Peri, 94270 Le Kremlin Bicetre, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Clovis","family":"Adam","sequence":"additional","affiliation":[{"name":"Pathology Laboratory, CHU de Bic\u00eatre, 78 Rue du General Leclerc, 94270 Le Kremlin Bicetre, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C\u00e9line","family":"Labeyrie","sequence":"additional","affiliation":[{"name":"Neurology Department, AP-HP, CHU de Bic\u00eatre, 78 Rue du General Leclerc, 94270 Le Kremlin Bicetre, France"},{"name":"French National Reference Center for Rare Neuropathies (NNERF), CHU de Bic\u00eatre, 78 Rue du General Leclerc, 94275 Le Kremlin Bicetre, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Adeline","family":"Not","sequence":"additional","affiliation":[{"name":"Neurology Department, AP-HP, CHU de Bic\u00eatre, 78 Rue du General Leclerc, 94270 Le Kremlin Bicetre, France"},{"name":"French National Reference Center for Rare Neuropathies (NNERF), CHU de Bic\u00eatre, 78 Rue du General Leclerc, 94275 Le Kremlin Bicetre, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Guillemette","family":"Beaudonnet","sequence":"additional","affiliation":[{"name":"Neurology Department, AP-HP, CHU de Bic\u00eatre, 78 Rue du General Leclerc, 94270 Le Kremlin Bicetre, France"},{"name":"French National Reference Center for Rare Neuropathies (NNERF), CHU de Bic\u00eatre, 78 Rue du General Leclerc, 94275 Le Kremlin Bicetre, France"},{"name":"Neurophysiology Department, AP-HP, CHU de Bic\u00eatre, 78 Rue du General Leclerc, 94270 Le Kremlin Bicetre, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1012-9783","authenticated-orcid":false,"given":"Andoni","family":"Echaniz-Laguna","sequence":"additional","affiliation":[{"name":"Neurology Department, AP-HP, CHU de Bic\u00eatre, 78 Rue du General Leclerc, 94270 Le Kremlin Bicetre, France"},{"name":"Faculty of Medicine, Paris Saclay University, 63 Rue Gabriel Peri, 94270 Le Kremlin Bicetre, France"},{"name":"French National Reference Center for Rare Neuropathies (NNERF), CHU de Bic\u00eatre, 78 Rue du General Leclerc, 94275 Le Kremlin Bicetre, France"},{"name":"INSERM U1195, Paris Saclay University, 94276 Le Kremlin Bicetre, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David","family":"Adams","sequence":"additional","affiliation":[{"name":"Neurology Department, AP-HP, CHU de Bic\u00eatre, 78 Rue du General Leclerc, 94270 Le Kremlin Bicetre, France"},{"name":"Faculty of Medicine, Paris Saclay University, 63 Rue Gabriel Peri, 94270 Le Kremlin Bicetre, France"},{"name":"French National Reference Center for Rare Neuropathies (NNERF), CHU de Bic\u00eatre, 78 Rue du General Leclerc, 94275 Le Kremlin Bicetre, France"},{"name":"INSERM U1195, Paris Saclay University, 94276 Le Kremlin Bicetre, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C\u00e9cile","family":"Cauquil","sequence":"additional","affiliation":[{"name":"Neurology Department, AP-HP, CHU de Bic\u00eatre, 78 Rue du General Leclerc, 94270 Le Kremlin Bicetre, France"},{"name":"French National Reference Center for Rare Neuropathies (NNERF), CHU de Bic\u00eatre, 78 Rue du General Leclerc, 94275 Le Kremlin Bicetre, France"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2023,5,4]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"3556","DOI":"10.1111\/ene.14959","article-title":"European Academy of Neurology\/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint Task Force\u2014Second revision","volume":"28","author":"Hadden","year":"2021","journal-title":"Eur. J. Neurol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1093\/qjmed\/92.2.103","article-title":"Central nervous system sarcoidosis\u2014Diagnosis and management","volume":"92","author":"Zajicek","year":"1999","journal-title":"QJM"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1016\/j.ncl.2009.09.004","article-title":"Neurological Manifestations of Systemic Lupus Erythematosus in Children and Adults","volume":"28","author":"Muscal","year":"2010","journal-title":"Neurol. Clin."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"278","DOI":"10.4103\/aian.AIAN_116_17","article-title":"Clinical Spectrum, Therapeutic Outcomes, and Prognostic Predictors in Sjogren\u2019s Syndrome-associated Neuropathy","volume":"20","author":"Sivadasan","year":"2017","journal-title":"Ann. Indian Acad. Neurol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1400","DOI":"10.1002\/art.40930","article-title":"2019 European League Against Rheumatism\/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus","volume":"71","author":"Aringer","year":"2019","journal-title":"Arthritis Rheumatol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1002\/art.39859","article-title":"2016 American College of Rheumatology\/European League Against Rheumatism Classification Criteria for Primary Sj\u00f6gren\u2019s Syndrome: A Consensus and Data-Driven Methodology Involving Three International Patient Cohorts","volume":"69","author":"Shiboski","year":"2017","journal-title":"Arthritis Rheumatol."},{"key":"ref_7","doi-asserted-by":"crossref","unstructured":"Joint Task Force of the EFNS and the PNS (2010). European Federation of Neurological Societies\/Peripheral Nerve Society Guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society\u2014First Revision. J. Peripher Nerv. Syst., 15, 1\u20139.","DOI":"10.1111\/j.1529-8027.2010.00245.x"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"264","DOI":"10.1093\/brain\/awf027","article-title":"Nerve granulomas and vasculitis in sarcoid peripheral neuropathy: A clinicopathological study of 11 patients","volume":"125","author":"Said","year":"2002","journal-title":"Brain J. Neurol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"283","DOI":"10.1111\/j.1600-0404.1986.tb03277.x","article-title":"Neurosarcoidosis: Clinical presentations and course in 50 patients","volume":"73","author":"Oksanen","year":"1986","journal-title":"Acta Neurol. Scand."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"220","DOI":"10.1378\/chest.112.1.220","article-title":"Neurosarcoidosis: A personal perspective based on the study of 37 patients","volume":"112","author":"Sharma","year":"1997","journal-title":"Chest"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"296","DOI":"10.1002\/mus.21890","article-title":"Sarcoidosis presenting as acute inflammatory demyelinating polyradiculoneuropathy","volume":"43","author":"Saifee","year":"2011","journal-title":"Muscle Nerve"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"P6.142","DOI":"10.1212\/WNL.86.16_supplement.P6.142","article-title":"Neurosarcoidosis Presenting as Acute Inflammatory Polyneuropathy (P6.142)","volume":"86","author":"Bhat","year":"2016","journal-title":"Neurology"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1016\/j.jns.2006.01.014","article-title":"The natural history and long-term outcome of 57 limb sarcoidosis neuropathy cases","volume":"244","author":"Burns","year":"2006","journal-title":"J. Neurol. Sci."},{"key":"ref_14","first-page":"3S85","article-title":"Chronic inflammatory demyelinating polyneuropathy and sarcoidosis: Fortuitous association?","volume":"163","author":"Ducray","year":"2007","journal-title":"Rev. Neurol."},{"key":"ref_15","first-page":"943","article-title":"Neurosarcoidosis. A report of 5 cases","volume":"67","author":"Elkin","year":"1985","journal-title":"South Afr. Med. J. Suid-Afr. Tydskr. Geneeskd."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1097\/CND.0b013e3181ae3be9","article-title":"Neurosarcoidosis presenting as Guillain-Barr\u00e9-like syndrome. A case report and review of the literature","volume":"11","author":"Fahoum","year":"2009","journal-title":"J. Clin. Neuromuscul. Dis."},{"key":"ref_17","first-page":"357","article-title":"Chronic polyradiculoneuritis disclosing sarcoidosis","volume":"158","author":"Ferriby","year":"2002","journal-title":"Rev. Neurol."},{"key":"ref_18","first-page":"72","article-title":"Sarcoidosis and Gullain-Barr\u00e9 syndrome","volume":"111","author":"Findik","year":"2011","journal-title":"Acta Neurol. Belg."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"765","DOI":"10.1136\/pgmj.65.768.765","article-title":"Sarcoidosis presenting with an acute Guillain-Barr\u00e9 syndrome","volume":"65","author":"Miller","year":"1989","journal-title":"Postgrad Med. J."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"P3.371","DOI":"10.1212\/WNL.86.16_supplement.P3.371","article-title":"A Case of Neurosarcoidosis Masquerading as Subacute Inflammatory Demylelinating Polyneuropathy","volume":"86","author":"Rizk","year":"2016","journal-title":"Neurology"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"664","DOI":"10.1002\/mus.24652","article-title":"Sarcoid polyneuropathy masquerading as chronic inflammatory demyelinating polyneuropathy","volume":"52","author":"Singhal","year":"2015","journal-title":"Muscle Nerve"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"131","DOI":"10.1016\/0002-9343(67)90154-4","article-title":"Sarcoidosis with a Landry-Guillain-Barr\u00e9 syndrome and clinical response to corticosteroids","volume":"43","author":"Strickland","year":"1967","journal-title":"Am. J. Med."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"96","DOI":"10.5414\/NPP27096","article-title":"Sarcoid neuropathy: Clinico-pathological study of 4 new cases and review of the literature","volume":"27","author":"Vital","year":"2008","journal-title":"Clin. Neuropathol."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"1558","DOI":"10.1212\/WNL.41.10.1558","article-title":"Sarcoid peripheral neuropathy","volume":"41","author":"Zuniga","year":"1991","journal-title":"Neurology"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1136\/jnnp.51.1.159","article-title":"Chronic relapsing inflammatory polyneuropathy complicating sicca syndrome","volume":"51","author":"Barnes","year":"1988","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"1139","DOI":"10.1038\/bmt.2013.18","article-title":"Sj\u00f6gren\u2019s syndrome associated chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) treated with autologous and subsequently allogeneic haematopoietic SCT (HSCT)","volume":"48","author":"Bregante","year":"2013","journal-title":"Bone Marrow Transpl."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"1119","DOI":"10.1177\/0961203312440346","article-title":"Successful treatment of chronic inflammatory demyelinating polyneuropathy (CIDP) in systemic lupus erythematosus (SLE) with oral cyclophosphamide","volume":"21","author":"Jasmin","year":"2012","journal-title":"Lupus"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"485","DOI":"10.1016\/j.jbspin.2017.02.013","article-title":"Lewis-Sumner syndrome in a patient with rheumatoid arthritis: Link between rheumatoid arthritis and demyelinating polyradiculoneuropathies","volume":"84","author":"Kedra","year":"2017","journal-title":"Jt. Bone Spine"},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"75","DOI":"10.22631\/rr.2017.69997.1021","article-title":"Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) as a first presentation of systemic lupus erythematosus","volume":"2","author":"Kianmehr","year":"2017","journal-title":"Rheumatol. Res."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"604","DOI":"10.1001\/archneur.1986.00520060066020","article-title":"Acute and chronic demyelinating inflammatory polyradiculoneuropathy. Association with autoimmune diseases and lymphocyte response to human neuritogenic protein","volume":"43","author":"Abramsky","year":"1986","journal-title":"Arch. Neurol."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"476","DOI":"10.4103\/0028-3886.82754","article-title":"Sj\u00f6gren\u2019s syndrome with chronic inflammatory demyelinating polyneuropathy","volume":"59","author":"Hsu","year":"2011","journal-title":"Neurol. India"},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"328","DOI":"10.1080\/03009749950155544","article-title":"Mixed connective tissue disease associated with chronic inflammatory demyelinating polyneuropathy: CASE REPORT","volume":"28","author":"Luostarinen","year":"1999","journal-title":"Scand. J. Rheumatol."},{"key":"ref_33","first-page":"146","article-title":"Sensorimotor peripheral neuropathy in rheumatoid arthritis","volume":"28","author":"McCombe","year":"1991","journal-title":"Clin. Exp. Neurol."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"142","DOI":"10.2169\/internalmedicine.41.142","article-title":"Motor dominant neuropathy in Sj\u00f6gren\u2019s syndrome: Report of two cases","volume":"41","author":"Mochizuki","year":"2002","journal-title":"Intern. Med."},{"key":"ref_35","first-page":"481","article-title":"3 cases of rare peripheral neuropathies associated with primary Gougerot-Sj\u00f6gren syndrome","volume":"149","year":"1993","journal-title":"Rev. Neurol."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"855","DOI":"10.1002\/mus.21274","article-title":"Subacute inflammatory polyradiculopathy associated with Sj\u00f6gren\u2019s syndrome","volume":"39","author":"Rigamonti","year":"2009","journal-title":"Muscle Nerve"},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"762","DOI":"10.1002\/mus.23339","article-title":"Chronic inflammatory demyelinating polyneuropathy associated with alopecia totalis and Sj\u00f6gren syndrome","volume":"45","author":"Sanvito","year":"2012","journal-title":"Muscle Nerve"},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"399","DOI":"10.1111\/j.1600-0404.1991.tb03971.x","article-title":"Serum antibodies to GM1 and GM3-gangliosides in systemic lupus erythematosus with chronic inflammatory demyelinating polyradiculoneuropathy","volume":"83","author":"Sindern","year":"1991","journal-title":"Acta Neurol. Scand."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1016\/j.semarthrit.2005.08.008","article-title":"Chronic inflammatory demyelinating polyneuropathy in patients with systemic lupus erythematosus: Prognosis and outcome","volume":"35","author":"Vina","year":"2005","journal-title":"Semin. Arthritis Rheum."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"177","DOI":"10.1159\/000117120","article-title":"Chronic inflammatory demyelinating polyneuropathy in patient with rheumatoid arthritis","volume":"35","author":"Zappia","year":"1995","journal-title":"Eur. Neurol."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"965","DOI":"10.1007\/s00296-009-1008-2","article-title":"Chronic inflammatory demyelinating polyradiculoneuropathy in a boy with systemic lupus erythematosus","volume":"30","author":"Zoilo","year":"2010","journal-title":"Rheumatol. Int."},{"key":"ref_42","first-page":"47","article-title":"Salivary gland biopsy in sarcoidosis","volume":"6","author":"Harvey","year":"1989","journal-title":"Sarcoidosis"}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/12\/9\/3281\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T19:29:14Z","timestamp":1760124554000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/12\/9\/3281"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,5,4]]},"references-count":42,"journal-issue":{"issue":"9","published-online":{"date-parts":[[2023,5]]}},"alternative-id":["jcm12093281"],"URL":"https:\/\/doi.org\/10.3390\/jcm12093281","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,5,4]]}}}