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Table 2 Comparison of clinical profile between our cases and pSS-related Fanconi syndrome reported in the literature

From: Ectopic germinal center and megalin defect in primary Sjogren syndrome with renal Fanconi syndrome

Reference Presenting symptoms RTA Cr K (mmol/L) Histology Treatment Outcome
Shearn and Tu [29] Polyuria + n.a. 3.8 TIN, tubular atrophy n.a. n.a.
Walker et al. [30] Paralysis, polyuria + n.a. n.a. TIN Prednisolone 10 mg/day n.a.
Kamm and Fischer [24] Polyuria, nocturia, weight loss + 2.7 mg/dl 2.9 Diffuse TIN Supportive only Improved
Matsumura et al. [26] n.a. n.a. 2.7 mg/dl n.a. TIN, tubulitis n.a. n.a.
Ardiles et al. [21] Muscle weakness + 1.3 mg/dl 2.5 n.a. Prednisolone “low dose” Improved
Bridoux et al. [22] Weight loss + 1.8 mg/dl 3.5 Diffuse TIN, proximal tubulitis Supportive only Dieda
  Polyuria + 1.6 mg/dl 2.4 Diffuse TIN, proximal tubulitis Prednisolone 10 mg/day Improved
Kobayashi et al. [25] Muscle weakness + 1.3 mg/dl 2.7 Diffuse TIN, proximal tubule atrophy Prednisolone 30 mg/day, 6 months later 12.5 mg/day Improved
Ren et al. [32]b n.a.
Yang et al. [31] Muscle weakness, respiratory distress + 1.4 mg/dl 2.7 n.a. Supportive only n.a.
Nakamura et al. [27] Renal dysfunction, organizing pneumonia, multiple bone fracture + 1.3 mg/dl 3.0 n.a. Mizoribine 50 mg/day n.a.
Wang et al. [6] Hypokalemic paralysis + 2.2 mg/dl 1.6 Diffuse TIN Mycophenolate mofetil 1 g/day Improvedf
Ram et al. [28] Paralysis + 2.1 mg/dl 1.3 Dense lymphocytic interstitial infiltrate Supportive only Improved
Celik et al. [23] Paralysis, cardiac arrestc + 1.1 mg/dl 1.1 n.a. Prednisone 40 mg/d iv. in acute phase Improvedf
Shi and Chen [33] Proteinuria, glycosuriad +   3.07 TIN Methylprednisolone Improvede
Saeki et al. [34] Renal dysfunction + 1.07 mg/dl 3.7 TIN Prednisolone 40 mg/day Improvede
Kong et al. [35] Weakness, osteodynia, impaired mobility _ n.a. 1.3 n.a. Prednisone 30 mg/day  
Our cases Fatigue, anorexia + 151 μmol/L 3.4 Diffuse TIN, diffuse tubule atrophy, lymphocyte infiltration Prednisone 50 mg/day Improvedf
  Fatigue, polyuria, anorexia, osteopathy + 88 μmol/L 2.1 Focal TIN, focal tubule atrophy Prednisone 40 mg/day Improvedf
  Fatigue, anorexia, osteopathy + 176 μmol/L 3.3 Diffuse TIN, diffuse tubule atrophy, lymphocyte infiltration Prednisone 50 mg/day Improvede
  Fatigue, anorexia + 305 μmol/L 2.7 Focal TIN, focal tubule atrophy Prednisone 45 mg/day Improvede
  Fatigue, anorexia, polyuria 72 μmol/L 2.53 Mild tubulitis Supportive only Improvedf
  Fatigue, anorexia, polyuria + 184 μmol/L 3.0 n.a. Prednisone 35 mg + cyclophosphamide 0.2 g qod Improvede
  Polyuria 120 μmol/L 3.4 n.a. Prednisone 60 mg/day Improvede
  Fatigue, polyuria, anorexia, osteopathy + 202 μmol/L 2.62 n.a. Prednisone 55 mg/day Improvede
  Osteopathy 110 μmol/L 3.2 n.a. Prednisone 55 mg/day + metrotraxate 10 mg qw Improvedf
  Fatigue, polyuria, osteopathy + 75 μmol/L 2.88 n.a. Prednisone 40 mg/day + cyclophosphamide 0.2 g qod Improvedf
  Hypokalemic paralysis, osteopathy + 65 μmol/L 2.1 n.a. Supportive Improvedf
  polyuria, osteopathy + 71 μmol/L 2.34 n.a. Prednisone 30 mg/day + metrotraxate 10 mg qw Improvedf
  1. Mean age of patients with pSS-related Fanconi syndrome reported in the literature is 47.4 ± 13.2, with a female ratio of 93.3%
  2. n.a. not available, pSS primary Sjogren syndrome, qop every other day, qw every week, RTA renal tubule acidosis, TIN tubulointerstitial nephritis
  3. aProbable cardiovascular event
  4. bFour cases reported in a retrospective study of 130 cases, no detailed information
  5. cAlso diagnosed of brucellic disease
  6. dWith autoimmune thyroiditis
  7. eImprovement of renal function, and stable during follow-up
  8. fCorrection of electrolyte derangement, and relief of symptoms