Table 1 Demographic, biochemical and immunological data for the 12 patients with pSS
Patient | Age | Sex | Presentation/reason for referral | Extra-renal clinical features | ANA | Anti-Ro | Anti-La | RF (0–20 IU/ml) | C3 (70–165 mg/dL) | C4 (16–54 mg/dL) | IgG (7-16 g/L) | Serum protein electrophoresis | ESR (mm/hr) | Creatinine (μmol/L) | Urine PCR (mg/mmol) |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
1 | 54 | Female | Renal impairment Urinary Symptoms | Sicca, Non-specific (generally unwell, fatigue, poor energy) | positive (>1/1000 fine speckled) | Positive | Negative | 145 | 128 | 15 | 24.9 | Polyclonal increase in immunoglobulins | 107 | 168 | 0 |
2 | 52 | Female | Hypokalaemic acidosis with paralysis | Sicca, Non-specific (generally unwell, fatigue, poor energy), arthralgias | positive (>1/1000 fine speckled) | Positive | Positive | N/A | 137 | 29 | 27.5 | Polyclonal increase in immunoglobulins | 88 | 97 | 27 |
3 | 36 | Female | Hypokalaemic acidosis | Sicca, Non-specific (generally unwell, fatigue, poor energy), arthralgias, parotitis, low mood. | positive (>1/1000 fine speckled) | Positive | Positive | 718 | 148 | 21 | 24.8 | Polyclonal increase in immunoglobulins | 35 | 88 | 102 |
4 | 45 | Female | Renal impairment | Sicca | positive (>1/1000 fine speckled) | Positive | Positive | 743 | 139 | 15 | 37.6 | Polyclonal increase in immunoglobulins | 121 | 124 | N/A |
5 | 71 | Female | Hypokalaemic acidosis with paralysis | Sicca, Non-specific (generally unwell, fatigue, poor energy), arthralgias | positive (>1/1000 fine speckled) | Positive | Negative | 618 | 124 | 21 | 21.3 | Polyclonal increase in immunoglobulins | 86 | 106 | 101 |
6 | 54 | Female | Renal impairment Stones | Sicca, Non-specific (generally unwell, fatigue, poor energy) | positive (>1/1000 fine speckled) | Positive | Positive | 69 | 89 | 16 | 18.5 | N/A | 5 | 186 | 50 |
7 | 48 | Female | Renal impairment Nephrocalcinosis | Sicca, Non-specific (generally unwell, fatigue, poor energy), rash | positive (>1/1000 fine speckled) | Positive | Positive | 93 | N/A | N/A | 14 | Polyclonal increase in immunoglobulins | 33 | 133 | 127 |
8 | 43 | Female | Renal impairment Nephrocalcinosis | Sicca, Non-specific (generally unwell, fatigue, poor energy) | positive (>1/1000 fine speckled) | Positive | Positive | <20 | 108 | 21 | 19.7 | Polyclonal increase in immunoglobulins | N/A | 141 | 46 |
9 | 51 | Female | Renal impairment | Sicca, Non-specific (generally unwell, fatigue, poor energy) | positive (>1/1000 fine speckled) | Positive | Negative | <20 | 70 | 16 | 19.9 | N/A | 5 | 168 | 0 |
10 | 61 | Female | Renal impairment | Sicca, non-specific (generally unwell, fatigue, poor energy) | positive (>1/1000 fine speckled) | Positive | Positive | 158 | 85 | 20 | 13.7 | Polyclonal increase in immunoglobulins | 25 | 115 | 0 |
11 | 72 | Female | Renal impairment | Sicca, non-specific (generally unwell, fatigue, poor energy), arthralgia and GI upset. | positive (>1/1000 fine speckled) | Positive | Negative | N/A | 88 | 14 | 17.6 | Paraprotein with immunoparesis | N/A | 133 | N/A |
12 | 53 | Male | Renal impairment | Sicca, neuropathy, vasculitic rash, cryoglobulinaemia | positive (>1/1000 fine speckled) | Positive | Negative | 1370 | 96 | 3 | 12.4 | Type 1 IgM kappa cryoglobulin | 65 | 133 | 40 |