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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)
  1. VOC vaso-occlusive crises, ACS acute chest syndrome, AID autoimmune disease, SLE systemic lupus erythematosus, JIA juvenile idiopathic arthritis, AIH autoimmune hepatitis, M male, F female, ESR erythrocyte sedimentation rate, ANA antinuclear antibodies, RF rheumatoid factor
  2. afirst ascribed to SCD but probably related to JIA