|
Case | Age at diagnosis of SLE (years) | Main manifestations of SLE | Treatments for inducing SLE remission | Maintenance treatment for SLE | Age at onset of VSRC (years) | SLEDAI 2 K at VSRC onset | Manifestations of VSRC | Treatments for VSRC with failed response | VSRC outcome, relationship with SLE activity |
|
1 | 40 | Mucocutaneous Musculoskeletal Cardiovascular (endocarditis) Immunological | Glucocorticoids Azathioprine Mycophenolate Mofetil Rituximab | Rituximab (3 cycles) | 44 | 1 | Dysuria Polyuria Nocturia | Methylprednisolone Azathioprine Fexofenadine Amitriptyline | Improvement, SLE inactive |
|
2 | 14 | Musculoskeletal Renal Pulmonary Haematologic Immunological | Glucocorticoids Mycophenolate Mofetil Cyclophosphamide Rituximab | Rituximab (6 cycles) | 21 | 2 | Dysuria Polyuria Nocturia Perineal pain Bladder pressure | Prednisolone Amitriptyline Hydroxyzine Oxybutynin Carbamazepine Gabapentin Botulinum toxin | Improvement, SLE inactive |
|
3 | 19 | Renal Musculoskeletal Haematologic Immunological | Glucocorticoids Mycophenolate Mofetil Cyclophosphamide | Mycophenolate Hydroxychloroquine Rituximab started at relapse (one cycle) | 23 | 8 | Dysuria Polyuria Severe perineal pain Bladder pressure Hypogastric pain | Methylprednisolone Amitriptyline Anti-H1 Anti-H2 : ranitidine NSAIDs Opioids Pregabalin Hyoscine bromide Intravenous gamma globulin Pudendal block Urethral infiltration (partial response) | Refractoriness to treatment, SLE inactive |
|
4 | 12 | Renal Haematologic Immunological Mucocutaneous Neurological Gastrointestinal | Glucocorticoids Azathioprine Mycophenolate Mofetil Cyclophosphamide Rituximab | Rituximab (7 cycles) | 20 | 12 | Dysuria Polyuria Nocturia | Methylprednisolone IV Instillations (heparin and lidocaine) Oxybutynin Montelukast | Refractory treatment, relapse of SLE. Death associated with infections, multisystem failure. |
|