Table 3 Clinical features of pSS patients with renal involvement.
N | Sex/age* | Renal presentation | Renal biopsy findings | Serological status | Treatment | eGFR (ml/min/1.73 m2) | F/U (years) | Comments | |
|---|---|---|---|---|---|---|---|---|---|
Ro/La/RF | Initial | Last | |||||||
1 | F/34 | Hypokalemia, RTA (I), nephrocalcinosis | ND | +/−/+ | NaHCO3, K citrate, PD 5–7.5 mg/day | 105 | 111 | 6.4 | MALToma (Neck, 5 years after renal involvement) |
2 | F/79 | Proteinuria (PCR 0.3 g/g) | TIN | +/−/+ | MPD (62.5 mg IV) taper, HCQ | 90 | 81 | 5.4 | |
3 | F/34 | Left frank pain, RTA (I), nephrocalcinosis | ND | +/+/+ | Amiloride (for 19 years) | 73 | 90 | 30.3 | |
4 | F/39 | Hypokalemia, RTA (I), nephrocalcinosis | ND | +/+/+ | None | 67 | 80 | 0.8 | Lymphocytic interstitial pneumonia |
5 | F/46 | Renal colic pain, nephrocalcinosis, RTA (I) | ND | +/+/+ | MPD 4 mg, HCQ, Nephrolithotomy | 91 | 73 | 4.0 | |
6 | F/63 | Dyspnea, hypokalemia, RTA (I) | ND | +/+/− | NaHCO3, K citrate, PD 10 mg | 52 | 30 | 9.3 | Cholangiocarcinoma (2 years before pSS diagnosis) |
7 | F/30 | Hypokalemia, RTA (I), dyspnea | ND | +/+/+ | K citrate | 118 | 92 | 0.5 | Transferred to another hospital |
8 | F/47 | Renal colic pain, nephrocalcinosis, RTA (I) | ND | +/ND/ND | NaHCO3, K citrate, HCQ, ARB | 108 | 106 | 6.1 | |
9 | F/47 | Hypokalemic paralysis, RTA (I) | ND | +/+/+ | NaHCO3, K citrate, HCQ | 75 | 74 | 5.5 | |
10 | F/72 | RTA (I) | ND | +/+/+ | K citrate, PD 2.5 mg, ARB | 64 | 33 | 9.7 | ITP, died (Aspiration pneumonia) |
11 | F/53 | Decreased eGFR, RTA (I) Further decreased eGFR (4 years later), proteinuria (PC ratio 0.5 g/g) | TIN (1st), FSGS (2nd) | +/+/+ | PD 15 mg → 5 mg (tapering for 3 mo.), K citrate (for TIN) PD 125 mg for 3 days → 5 mg (tapering for 1 year) (for GN) | 42 | 27 | 6.8 | Lymphocytic interstitial pneumonia, hypergammaglobulinemia, hypothyroidism |
12 | F/44 | Hypokalemic paralysis, RTA (I), nephrocalcinosis | ND | +/+/+ | K citrate, spironolactone | 85 | 94 | 2.1 | Renal involvement was confirmed after 17 years of pSS diagnosis |
13 | F/71 | Non-oliguric AKI, RTA (I) | ND | +/+/+ | NaHCO3 | 18 | 32 | 0.5 | eGFR 84 ml/min/1.73 m2 (diagnosis with pSS) AKI event developed 5 years later |
14 | F/40 | Peripheral edema, RTA (1), nephrocalcinosis | ND | +/+/ND | K citrate | 46 | 59 | 3.7 | |
15 | F/36 | Hypokalemia (cardiac arrest), RTA (1) | ND | +/+/ ND | K citrate | 49 | 44 | 24.8 | |
16 | F/32 | Hypokalemia, RTA (1), decreased eGFR, nephrocalcinosis | ND | +/+/ND | K citrate | 67 | 63 | 3.5 | |
17 | F/41 | RTA (1), nephrocalcinosis | ND | +/ND/ND | PD 5 mg, HCQ | 77 | 57 | 5.2 | |
18 | F/31 | Hypokalemia, RTA (1) Proteinuria (7.9 g/day) | MGN (Stage I) | +/−/− | PD 60 mg taper to 5 mg, K citrate | 118 | 105 | 14.8 | Hyperthyroidism |
19 | F/23 | Proteinuria (PCR 3.2 g/g) | MGN (Stage III) | +/+/+ | PD 15 mg tapering, ARB | 115 | 105 | 5.0 | Ovarian cancer.6 months before pSS |
20 | F/63 | Generalized edema, hypertension, Proteinuria (254 mg/day) | Diffuse proliferative GN | +/−/+ | MMF, NaHCO3, ARB, PD 7.5 mg | 38 | 57 | 2.3 | Hypergammaglobulinemia, Cryoglobulinemia |
21 | F/62 | Decreased eGFR | FSGS | +/+/+ | HCQ | 67 | 65 | 2.4 | |
22 | F/47 | Proteinuria (1.4 g/day) | MGN (Stage II) | +/−/− | PD 80 mg taper | 101 | 103 | 14.1 | |
23 | F/58 | Proteinuria (3.3 g/day) | MGN (Stage I) | +/−/− | PD 30 mg tapering, cyclosporine | 102 | 48 | 7.3 | |
24 | F/ 39 | Microscopic hematuria, proteinuria (PCR 1.6 g/g) | IgAN, MPGN pattern (VI) | +/−/+ | MMF, PD 10 mg | 58 | 68 | 3.0 | Lymphomatoid granulomatosis (Brain) and thyroid cancer–3 years after renal involvement |
25 | F/49 | Proteinuria (1.5 g/day) | MGN (advanced) | +/+/+ | Cyclophosphamide 100 mg/day (1 year), HCQ, PD 5 mg | 117 | 112 | 4.5 | Biopsy also showed mild focal tubular atrophy |
26 | F/71 | Hematuria, Proteinuria (284 mg/day) | FSGS | +/−/− | ARB, PD 5 mg | 44 | 66 | 1.6 | Hypergammaglobulinemia |
27 | F/29 | Proteinuria (103 mg/day) | FSGS | +/−/ND | None | 105 | 119 | 3.0 | |
28 | F/47 | Decreased eGFR | FSGS | +/+/+ | NaHCO3 | 19 | 11 | 4.5 | |
29 | F/54 | Proteinuria (2.1 g/day) | MGN (Stage III) | +/+/+ | PD 60 mg taper to 5 mg, ARB | 76 | 101 | 10.1 | Light chain MGUS (3 years before pSS) |
30 | M/38 | Proteinuria (1.6 g/day) | MGN (Stage III) | +/−/− | PD 50 mg taper to 10 mg | 90 | 55 | 12.6 | Acute B-lymphoblastic leukemia (5 years before pSS) |
31 | F/60 | Proteinuria (0.9 g/day) | FSGS | +/+/ND | ARB | 64 | 50 | 3.4 | |
32 | F/55 | Proteinuria (PCR, 0.5–1.0 g/g) | ND | +/−/− | ARB | 100 | 97 | 9.6 | Hypocomplementemia |
33 | F/51 | Tubular proteinuria (Urine β2-microglobulin, 8.72 mg/L), Alkaline urine | ND | +/−/+ | None | 58 | 50 | 2.9 | Hypergammaglobulinemia, No acidosis |
34 | F/57 | Decreased eGFR | ND | +/+/+ | HCQ | 28 | 39 | 12.3 | Decreased in kidney size in ultrasound |
35 | F/72 | Decreased eGFR | ND | +/+/+ | None | 58 | 47 | 7.1 | Hypocomplementemia |
36 | F/48 | Decreased eGFR | ND | +/+/+ | ARB, PD 5 mg | 39 | 43 | 5.9 | Hypergammaglobulinemia, Hypocomplementemia |
