Table 1 Medical history, clinical and biological findings at presentation in our patients
From: Coexistent sickle-cell anemia and autoimmune disease in eight children: pitfalls and challenges
| Patient | 1 | 2 | 3 | 4 | 5 | 6 | 7 | 8 |
|---|---|---|---|---|---|---|---|---|
| Sex | M | M | F | M | M | F | F | F |
| Medical history related to SCD | ACS (3 episodes) Recurrent VOC | Recurrent polyarthralgiaa | Recurrent VOC |
Recurrent VOC Osteomyelitis right femur Chronic hepatitis B |
Recurrent VOC Acute cholecystitis, Salmonella septicemia Bilateral avascular necrosis of femoral heads | Recurrent VOC |
Recurrent VOC ACS (3 episodes) |
Recurrent VOC ACS Acute splenic sequestration Recurrent cholangitis |
| Type of AID | SLE | JIA | JIA | JIA | AIH | Sjogrën’syndrome | SLE | AIH |
| Age at onset of AID (years) | 13 | 4 | 7 | 4 | 17 | 11 | 15 | 3 |
| Age at diagnosis of AID (years) | 13 | 13 | 7 | 6 | 17 | 13 (11 for initial diagnosis of JIA) | 15 | 3 |
| Symptoms | Asthenia, denutrition polyarthritis fingers and toes hepatomegaly pericarditis |
Polyarthritis left knee and right ankle severe arthropathy at 10 y uveitis | Polyarthritis (temporo-mandibular arthritis and sacroiliitis) |
Polyarthralgia bilateral knee arthritis Asthenia, weight loss | Polyarthralgia |
Polyarthralgia (spine); knee arthritis; Secondary recurrent parotiditis; xerostomia at 13 y |
Polyarthritis; pleuritis, pericarditis, aphthous stomatitis, alopecia; pulmonary hypertension; behavior disorder |
Hepatomegaly; jaundice |
| ESR (mm/h) | 74 | 95 | 34 | 86 | 67 | 14 | 84 | 88 |
| IgG (g/l) | 38 | 26 | – | – | 35 | 23 | 30 | 26 |
| ANA pattern | 1/1280 homogeneous | 1/160 speckled | Negative |
1/80 homogeneous | 1/80 | 1/1280 |
1/80 speckled | 1/160 |
| Others immunological features |
Anti-DNA antibodies + (43 UI/ml) Coombs + | RF negative | RF positive |
Anti-U1RNP + Farr test 29% |
Anti-SSA, SSB + Anti-U1RNP + Antiβ2GP1 + Antiphospholipid antibodies + |
Anti-DNA antibodies + (44 U/l) pANCA Coombs + Antiplatelets antibodies + AntiB2GP1 + anticardiolipin + Neutropenia |
c-ANCA + Smooth muscle antibodies + (1/50) |