Table 2.

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

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

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%

n.a. not available, pSS primary Sjogren syndrome, qop every other day, qw every week, RTA renal tubule acidosis, TIN tubulointerstitial nephritis

^aProbable cardiovascular event

^bFour cases reported in a retrospective study of 130 cases, no detailed information

^cAlso diagnosed of brucellic disease

^dWith autoimmune thyroiditis

^eImprovement of renal function, and stable during follow-up

^fCorrection of electrolyte derangement, and relief of symptoms