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 |