Authors/year Type of study Sample characteristics (sex, years in median or mean ± SD) Type of CNS involvement % ( ) of patients Type of PNS involvement Clinical associations with immunological profiles SLE patients Schneebaum et al., 1991 [9 ] Cohort 269 patients with SLE Depression 88% ( ), 45% psychosis ( ) NM The serum level of anti-P antibodies correlates with the activity of psychiatric disease
Isshi and Hirohata, 1998 [10 ] Cohort 87 SLE patients Psychosis 39% ( ), nonpsychotic CNS lupus 29,9% ( ) NM Serum anti-P levels were significantly elevated in patients with lupus psychosis compared with those with non-CNS SLE or those with nonpsychotic CNS lupus Tzioufas et al., 2000 [11 ] Cohort 28 patients with NPSLE Psychiatric disorders 25% ( ), grand mal seizures 14,2% ( ), focal signs 46,4% ( ) NM Overall prevalence of anti-P antibodies in active CNS disease patients was statistically and significantly higher, as compared to unselected SLE patients Kasitanon et al., 2002 [12 ] Cohort 91 patients with NPSLE (90 F, 1 M, 30,7 ± 10,9) Seizures 58,3% ( ), psychosis 14,3% ( ), acute confusion state 12% ( ), cerebral infraction 2,2% ( ), abnormal consciousness 6,6% ( ), transverse myelitis 6,6% ( ), aseptic meningitis 2,2% ( ) MM 3,3% ( ), polyneuropathy 2,2% ( ) Patients with NPSLE had significantly more cutaneous vasculitis and less arthritis than those without NPSLE Sanna et al., 2003 [13 ] Cohort 185 patients with NPSLE Headache 24% ( ), CD 14.5% ( ), mood disorders 16.7% ( ), cognitive disorders 10.8% ( ), seizures 8.3% ( ), psychosis 7.7% ( ), anxiety 3.7% ( ), acute confusional state 3.7% ( ) NM The presence of aPL was associated with NP manifestations Chiewthanakul et al., 2012 [14 ] Cohort 97 patients with NPSLE (84 F, 13 M, 35.1 ± 11.7) Seizures 33% ( ), CD 23,7% ( ), psychoses 24% ( ), myelopathy 6,2% ( ), headaches 2% ( ), mood disorders 1% ( ) 13 patients with polyneuropathy 77% ( ), GB 7,7% ( ), mononeuropathy 7,7% ( ), cranial neuropathy 7,7% ( ) ANA and antibodies to dsDNA did not correlate with NP manifestations
Závada et al., 2013 [15 ] Cohort 50 patients with NPSLE (5 M, 45 F 43 years (±16)) Cognitive disorder 50% ( ), mood disorder 28% ( ), CD 26% ( ), headache 26% ( ), seizure 22% ( ), psychosis 16% ( ), aseptic meningitis 4% ( ) Polyneuropathy 8% ( ), cranial neuropathy 6% ( ), mononeuropathy 4% ( ), transverse myelitis 2% ( ) NMO-IgG/AQP4-Ab in NPSLE were present only in a patient with TM and were not detectable in NPSLE patients with other neurological manifestations Hanly et al., 2004 [16 ] Cohort 111 patients with SLE (96 F, 15 M, 44.7 ± 1.2 years) Headaches 9% ( ), CD 3,6% ( ), mood disorders 3,6% ( ), cognitive dysfunction 2,7% ( ), acute confusional state 2,7% ( ), psychoses 2,7% ( ), seizures 0,9% ( ), anxiety 0,9% ( ), aseptic meningitis 0,9% ( ) Cranial neuropathy 1,8% ( ), polyneuropathy 1,8% ( ) No correlations were found Kluz et al., 2007 [17 ] Cohort 15 F mean age: 38.33 ± 11.02 years with SLE Organic brain syndrome 13,3% ( ), cranial nerve disorder 6,6% ( ), headache 6,6% ( ), seizures 6,6% ( ) NM CNS complications were associated with aPL antibodies in patients with severe disease activity and microangiopathic complications compared with those with less active disease Briani et al. 2009 [18 ] Cohort 85 SLE patients (NM sex and mean age) Headache 41,2% ( ), CD 12,3% ( ), epilepsy 11,8% ( ), psychiatric disorders 3% ( ), myelopathy 2,4% ( ) Symmetric polyneuropathy 20% ( ), mononeuropathy 15,3% ( ), median nerve 3,6% ( ) sciatic nerve 3,6% ( ) involvement, MM 2,4% ( ) Abs to ribosomal P proteins are associated with psychosis and MM Florica et al., 2011 [19 ] Case-control (retrospective) 207 SLE patients, F 86.3% (125 SLE-related PN 35.2 ± 14.4 years and 82 non-SLE-related PN 38.6 ± 15.4) NM PM 11,1% ( ), cranial neuropathy 12,5% ( ), MM 9,2% ( ), CIDP 0,9% ( ) There was no significant difference in lupus serology such as antinuclear antibody, anti-double-stranded DNA antibody, and antibodies to the extractable nuclear antigens between the two groups Hirohata et al., 2014 [20 ] Cohort 72 patients with NPSLE (49 with diffuse NPSLE 38.3 ± 14.4, 23 with neurological syndromes or peripheral neuropathy 42.0 ± 15.2) 32 M, 50 F Diffuse NPSLE: acute confusional state 38,7% ( ), anxiety 6,1% ( ), cognitive disorder 6,1% ( ), psychosis 14,2% ( ); focal NPSLE: headache 8,7% ( ), movement disorder 8,7% ( ), seizure 8,7% ( ), aseptic meningitis 4,3% ( ), demyelinating syndrome 4,3% ( ) Polyneuropathy 4,3% ( ) Anti-Sm and anti-RNP in CSF and sera were elevated in NPSLE compared with non-SLE control SS patients Spezialetti et al., 1993 [21 ] Cohort 77 patients with pSS Severe depression 13% ( ), psychosis 8% ( ), cognitive dysfunction 69% ( ) NM
No correlation between CNS diseases, including the presence of anti-ribosomal P antibodies Alexander et al., 1994 [22 ] Cohort Group 1: 52 SS patients Group 2: 49 patients Group 1: focal CNS disease 60% ( = 26/43), nonfocal CNS disease 22% ( = 2/9) Group 2: focal CNS disease 63% ( = 19/30), nonfocal CNS disease 37% ( = 7/19) Not available Anti-Ro antibodies were positive in 48% of patients with CNS compared to only 24% of all patients with pSS Delalande et al., 2004 [23 ] Cohort 82 patients (65 F, 17 M 48,6 years) Seizures 8,5% ( ), cognitive dysfunction 11% ( ), encephalopathy 2,4% ( ), optic neuropathy 15% ( ), MS 28% ( ) SMN 34,1% ( ), cranial neuropathy 19,5% ( ), MN 8,5% ( ), myositis 2,4% ( ), polyradiculoneuropathy 1,2% ( ) Anti-Ro/SSA or anti-La/SSB antibodies were more frequently observed in patients with PNS involvement than in those with CNS involvement Pittock et al., 2008 [24 ] Cohort 14 patients with SS/SLE with neurological manifestations NM NM SSA, SSA, ANA, and dsDNA antibodies were found in these patients but not NMO-IgG
Sène et al., 2011 [25 ] Cohort 120 patients with pSS (106 F, 14 M 50.4 ± 14.0) NM SMN 23% ( ), ASN 10% ( ), NSN 67% ( ) Patients with NSN with lower prevalence of ANA (60% versus 90%; ), anti-SSA (Ro) (40% versus 72%; ), anti-SSB (La) (15% versus 41%; ), RF (37% versus 67%; ), and hypergammaglobulinemia (35% versus 64%; ) Jamilloux et al., 2014 [26 ] Cohort 420 patients with pSS (377 F, 43 M, 53.6 ± 14.8) Acute ischemic stroke-like symptoms 1,4% ( ), dysarthria 0,2% ( ), seizures 0,2% ( ), cerebellar ataxia 0,4% ( ) central neuronitis 0,4% ( ), acute encephalopathy 2,1% ( ), cognitive impairment 0,4% ( ), aseptic meningitis 0,2% ( ), transverse myelitis ( ), optic neuritis 1,2% ( ), MS-like syndrome 1,2% ( ), cerebral venous thrombosis 0,2% ( ) SMN 0,6% ( ), MM 0,7% ( ), SN 4,5% ( ), SGN 2,1% ( ), DPN 0,2% ( ), cranial neuropathy 1,9% ( ) Patient with SN had more frequent cryoglobulinemia and lymphopenia ( ) but lower prevalence of anti-Ro/SSA antibodies and hypergammaglobulinemia ( ) Morreale et al. 2014 [27 ] Cohort 120 patients (12 M, 108 F; 58.3 ± 14.2 years) Headache 46.9% ( ), cognitive disorder 44.4% ( ), mood disorders 38.3% ( ) NM Headache, cognitive disorders, and psychiatric symptoms were significantly associated with anti-SSA RA
Sivri and Güler-Uysal, 1999 [28 ] Cohort 33 RA patients (28 F, 5 M; 46.7 ± 13.7 years) NM 6% ( ) carpal tunnel syndrome, 18% ( ) MM No correlation between neuropathy and RF