Table 1.
neurological and nephrological manifestation in childhood Sjogren syndrome
| 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 |
*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