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Table 1 neurological and nephrological manifestation in childhood Sjogren syndrome

From: Nephrological disorders and neurological involvement in pediatric primary Sjogren syndrome:a case report and review of literature

Case 1 2 3 4 5 6 7 8 9 10 11
References/Year Matsui [2] Kornitzer [3] Arabshahi [4] Bogdanovic [5] Kumon [6] Pessler [7] Jung [8] Igarashi [9] Gottfried [10] Wong [11] Kagan [12]
Age (Gender) 9/F 6/F 11/F 13/F 14/F 6/F 11/F 12/F 9/F 16/F 12/F
Symptoms at onset fever fever headache optic neuritis nephrocalcinosis fever arthralgia parotitis hematuria
proteinuria
parotitis fever
conjunctivitis
headache AKI
Dry eyes or mouth NM N NM NM NM NM Y Y Y N NM
Parotitis NM NM NM Y N NM N Y Y Y Y
Neurologic manifestation mental disorder
gaze deviation
ambulation difficulty
mental disorder
muscle weakness
muscle weakness
urinary incontinence
N arm numbness
hypalgesia
thermohypoesthesia
N N N Ambulation
difficulty
ptosis
leg numbness
dysarthria,
ataxia
N
Renal damage RTA N N dRTA
diabetes insipidus
N RTA dRTA
hematuria
proteinuria
proteinuria N N hematuria
proteinuria
ANA Pos Pos Pos Pos Pos Pos Pos Pos Pos Pos Pos
Anti-Ro/SSA Pos Pos Pos Pos Pos Pos Pos Pos Pos Pos Pos
Anti-Lo/SSB Neg Neg Pos Neg Pos Pos Neg Pos Neg Pos Neg
ESR NM NM NM NM Pos Pos NM Pos Neg Pos NM
RF Pos Neg Neg Pos Pos Pos NM NM Neg NM Neg
Hyperglobulinemic IgG IgM NM NM NM IgG NM N IgG IgA IgM NM NM NM
Schirmer test Neg Neg Pos Neg Neg NM Pos Pos Neg NM NM
CSF Pos Pos Pos NM Pos NM NM NM Neg Pos NM
Renal biopsy NM NM NM TIN NM GS TIN IgA TIN NM NM crescentic GN
TIN
Salivary gland biopsy Pos Pos Pos NM Pos NM NM Pos Pos NM NM
Immunomodulatory therapy GC GC AZA RTX GC CTX GC AZA MMF GC GC CTX
MMF
GC GC GC GC GC AZA
Outcome residual dysphagia right-side&facial
nerve weakness
vision loss stable renal
function
sensory disturbance stable renal
function
recover stable residual ptosis
facial diplegia
recover normal renal
function
Follow up (yr) 7 m 3 0.5 6 NM 7 4 m NM 6 m 3w 1
Case 12 13 14 15 16 17 18 19 20 21
References/Year DeGuzman [13] Skalova [14] Yoshida [15] Berman [16] Zawadzki [17] Kobayashi [18] Chang [19] Ohlsson [20] Hoshina [21] Present patient
Age (Gender) 14/M 16/F 13/M 10/F 15/F 10/F 11/F 15/F 8/F 16/F 12/F
Symptoms at onset fever seizure muscular weakness hematuria
proteinuria
fever headache
dizziness vomiting
gait disturbances uveitis fever weakness arthritis fever headache
vomiting
joint pain
Dry eyes or mouth N N N NM NM N N Y NM Y Y
Parotitis N NM N NM NM Y Y Y NM Y N
Neurologic manifestation muscle weakness
slurred speech
muscle weakness N dysmetria weakness
proprioception
N N N N N N superficial sensation
impaire
Renal damage Neg dRTA
proteinuria
hematuria
proteinuria
N dRTA dRTA N dRTA dRTA
proteinuria
N glucosuria
ANA Neg Pos Pos Pos Pos Pos Pos Pos Pos Pos Pos
Anti-Ro/SSA Neg Pos Pos Pos Pos Pos Pos Pos Pos Pos Neg
Anti-Lo/SSB Neg Pos Pos Pos Pos Neg Pos Neg Pos Pos Pos
ESR NM Pos NM Pos NM Pos Pos Pos Pos NM Pos
RF NM Pos NM Pos NM Pos Pos Pos Pos Pos Neg
Hyperglobulinemic IgG IgG N IgG IgG IgA IgM IgG IgM IgG IgA IgG1 IgG N
Schirmer test NM NM NM NM Neg Pos Neg Pos NM Pos Pos
CSF Pos NM NM Pos NM NM Pos NM NM Pos Neg
Renal biopsy NM TIN MGN NM TIN TIN NM NM NM NM TIN
Salivary gland biopsy Pos NM Pos Pos Pos Pos NM Neg NM NM Pos
Immunomodulatory therapy GC CTX GC CSA GC GC CTX GC CTX GC CTX GC NM GC HCQ MTX GC TAC HCQ GC
Outcome lethargic
visual impairment
difficulty walking
recover urine protein
hematuria
stable tubular acidosis stable stable NM synovitis symptoms
improved
glucosuria
Follow up (yr) 3 1 m 2 m 11 m 14 m 5 3 3 2 m NM 1.5
  1. *F female; M male; Y yes; N no; m months; w weeks; NM not mention; Neg negative; Pos positive; GC glucocorticoid; AZA azathioprine; RTX rituximab; HCQ hydroxychloroquine; CTX cyclophosphamide; MMF mycophenolate mofetil; MTX methotrexate; CSA cyclosporine A; TAC tacrolimus