Characteristics of the three cases
| Case | 1 | 2 | 3 | ||
| Case characteristics | Sex | F | F | F | |
| Ethnicity | Caucasian | North African | Caucasian | ||
| Tobacco use | Weaned (10 PY) | No | No | ||
| Follow-up (years) | 30 | 10 | 21 | ||
| Age at diagnosis (years) | 49 | 56 | 48 | ||
| Elements of 2016 ACR/EULAR classification criteria form primary Sjögren syndrome | Labial salivary gland biopsy | Chisholm score | Chisholm 1 | Chisholm 3 | Chisholm 4 |
| Focus score | – | 1.6 | 8 | ||
| Anti-SSA (positivity) | Yes | Yes | Yes | ||
| Schirmer test | – | 0 mm (RE and LE) | – | ||
| Unstimulated salivary flow rate (mL/min) | – | 0.3 | – | ||
| Cryoglobulinaemia | Type of cryoblobulinaemia | Type II | Type II | Type II | |
| Clinical manifestations | Articular involvement | Yes | Yes | No | |
| Synovitis | Yes | No | No | ||
| Cutaneous involvement | Yes | Yes | Yes | ||
| Type | Purpura | Purpura | Purpura | ||
| Pulmonary involvement | No | No | No | ||
| Renal involvement | Yes | No | Yes | ||
| Type | Glomerular | – | Glomerular | ||
| Renal biopsy | MPGN I | – | No | ||
| Creatinine rate (μmol/L) | 73 | 77 | 75 | ||
| GFR in CKD-EPI (mL/min) | 68 | 70 | 69 | ||
| Proteinuria (g/24 hours) | 0.68 | 0.14 | 0.66 | ||
| Haematuria | Yes | No | – | ||
| Muscular involvement | No | Yes | No | ||
| Type | – | Neurogenic atrophy | – | ||
| Biopsy | – | Yes | – | ||
| PNS involvement | Yes | Yes | Yes | ||
| EMG | Axonal polyneuropathy | Axonal polyneuropathy | Axonal polyneuropathy | ||
| Nerve biopsy | No | Yes | No | ||
| CNS involvement | No | No | No | ||
| History of treatments | First line | CYC | RTX | RTX | |
| Second line | RTX | CYC | RTX+CYC | ||
| Third line | RTX+CYC | MMF | |||
| Fourth line | RTX | Ofatumumab+CYC | |||
| Fifth line | RTX followed by AZA | ||||
| Sixth line | RTX+CYC | ||||
| Seventh line | CYC+RTX followed by MMF | ||||
| Caracteristics before treatment by anti-CD20 | ESSDAI | Constitutional involvement | 0 | 0 | 0 |
| Lymphadenopathy | 0 | 0 | 0 | ||
| Glandular involvement | 0 | 0 | 3 | ||
| Articular involvement | 0 | 0 | 0 | ||
| Cutaneous involvement | 3 | 3 | 3 | ||
| Pulmonary involvement | 0 | 0 | 0 | ||
| Renal involvement | 1 | 0 | 1 | ||
| Muscular involvement | 0 | 0 | 0 | ||
| PNS involvement | 0 | 0 | 3 | ||
| CNS involvement | 0 | 0 | 0 | ||
| Haematological involvement | 0 | 1 | 2 | ||
| Biological | 2 | 2 | 2 | ||
| Total | 16 | 13 | 42 | ||
| ESSPRI | VAS dryness | – | 7/10 | 5/10 | |
| VAS fatigue | – | 8/10 | 1/10 | ||
| VAS pain | – | 7/10 | 6/10 | ||
| Total : | – | 23/30 | 12/30 | ||
| B cells (/μL) | Before treatment | 43 | 19 | 6 | |
| After treatment | ND | 0 | 0 | ||
ACR, American College of Rheumatology; AZA, azathioprine; CKD-EPI, chronic kidney Disease Epidemiology collaboration; CNS, central nervous system; CYC, cyclophosphamide; EMG, electromyogram; ESSDAI, EULAR Sjögren's syndrome disease activity index; ESSPRI, EULAR Sjogren's syndrome patient reported index; EULAR, European League Against Rheumatism; F, female; GFR, glomerular filtration rate; LE, left eye; MMF, mycophenolate mofetil; MPGN, membranoproliferative glomerulonephritis; ND, not done; PNS, peripheral nervous system; PY, pack-year; RE, right eye; RTX, rituximab; SSA, Sjögren’s-syndrome-related antigen A; VAS, visual analog scale.