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

Wang, Jing; Wen, Yubing; Zhou, Mengyu; Shi, Xiaoxiao; Jiang, Lanping; Li, Mingxi; Yu, Yang; Li, Xuemei; Li, Xuewang; Zhang, Wen; Lundquist, Andrew L.; Chen, Limeng

doi:10.1186/s13075-017-1317-x

Arthritis Research & Therapy

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	Died^a
	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	Improved^f
Ram et al. [28]	Paralysis	+	2.1 mg/dl	1.3	Dense lymphocytic interstitial infiltrate	Supportive only	Improved
Celik et al. [23]	Paralysis, cardiac arrest^c	+	1.1 mg/dl	1.1	n.a.	Prednisone 40 mg/d iv. in acute phase	Improved^f
Shi and Chen [33]	Proteinuria, glycosuria^d	+		3.07	TIN	Methylprednisolone	Improved^e
Saeki et al. [34]	Renal dysfunction	+	1.07 mg/dl	3.7	TIN	Prednisolone 40 mg/day	Improved^e
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	Improved^f
	Fatigue, polyuria, anorexia, osteopathy	+	88 μmol/L	2.1	Focal TIN, focal tubule atrophy	Prednisone 40 mg/day	Improved^f
	Fatigue, anorexia, osteopathy	+	176 μmol/L	3.3	Diffuse TIN, diffuse tubule atrophy, lymphocyte infiltration	Prednisone 50 mg/day	Improved^e
	Fatigue, anorexia	+	305 μmol/L	2.7	Focal TIN, focal tubule atrophy	Prednisone 45 mg/day	Improved^e
	Fatigue, anorexia, polyuria	–	72 μmol/L	2.53	Mild tubulitis	Supportive only	Improved^f
	Fatigue, anorexia, polyuria	+	184 μmol/L	3.0	n.a.	Prednisone 35 mg + cyclophosphamide 0.2 g qod	Improved^e
	Polyuria	–	120 μmol/L	3.4	n.a.	Prednisone 60 mg/day	Improved^e
	Fatigue, polyuria, anorexia, osteopathy	+	202 μmol/L	2.62	n.a.	Prednisone 55 mg/day	Improved^e
	Osteopathy	–	110 μmol/L	3.2	n.a.	Prednisone 55 mg/day + metrotraxate 10 mg qw	Improved^f
	Fatigue, polyuria, osteopathy	+	75 μmol/L	2.88	n.a.	Prednisone 40 mg/day + cyclophosphamide 0.2 g qod	Improved^f
	Hypokalemic paralysis, osteopathy	+	65 μmol/L	2.1	n.a.	Supportive	Improved^f
	polyuria, osteopathy	+	71 μmol/L	2.34	n.a.	Prednisone 30 mg/day + metrotraxate 10 mg qw	Improved^f

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

Back to article page

ISSN: 1478-6362

Contact us

General enquiries: journalsubmissions@springernature.com