Intravenous immunoglobulins for adult Still's disease and pregnancy
SIR, We report a case of adult Still's disease (ASD) in a pregnant patient successfully treated with intravenous immunoglobulins (IVIg). To our knowledge, the effect of IVIg in this situation has never been reported.
A 28-year-old woman, in the 10th week of her first pregnancy, was referred to us for a high spiking fever, sore throat, cervical lymph node enlargement, nonpruritic evanescent measles-like diffuse rash with typical Koebner's sign, and painful polyarthritis with palpable synovitis affecting both wrists and the left knee, mediotarsal and metacarpophalangeal joints.
Laboratory studies showed an elevated erythrocyte sedimentation rate (120 mm/h), high C-reactive protein (174mg/1) and ferritin (2960ng/ml; normal: <240) levels, anaemia (haemoglobin level 9.7 g/dl), elevated white blood cell count (12 100 x 10^sup 9^/l with 73% PMN) and platelet (547 000 x 10^sup 9^/1) counts. AST was N x 1.5-fold and ALT was N x 1.7-fold. Serum rheumatoid factor and antinuclear antibody were negative. Blood cultures and an exhaustive serological investigation for bacterial, viral and parasitic aetiologies of ASD-like syndromes yielded repeatedly negative results. A therapeutic test with ampicillin was unsuccessful. A diagnosis of ASD was therefore made.
Her symptoms improved slightly with salicylates 3 g/24 h, but the inflammatory anaemia worsened (haemoglobin level 8.4 g/dl) and the treatment was tapered by half because of hepatic injury (AST and ASD rise up to N x 6-fold). From the 14th week, the fever abated, the articular symptoms gradually improved and the ferritin level decreased to 12 ng/ml.
A severe relapse occurred at the 22nd week. Salicylates were again increased to 3 g/24 h and a first course of IVIg, I g/kg/day for 2 days, was administered with excellent tolerance. At the 25th week, the articular symptoms had partially subsided, with persistent stable anaemia. Fever, rash and sore throat subsequently disappeared, but some degree of synovitis persisted and a second identical course of IVIg was given at the 31st week, again well tolerated. During gestation, obstetric examination was normal and ultrasonography showed normal growth of the fetus. At the 36th week, while all symptoms of ASD had fully cleared for 1 month, she became pre-eclamptic due to a retroplacentar haematoma and was urgently delivered by Caesarean section, giving birth to a 2.6-kg-weight healthy female whose subsequent growth appeared to be normal. Salicylates were discontinued. Six days post-partum, the mother was reoperated on with haemorrhagic collapse and an 8 cm ruptured ovarian serous cyst was removed. Two months later, there were no clinical or biological signs of disease activity. After a disease-free interval of 5 months, she suffered a severe relapse and received both a third course of IVIg and pulse methylprednisolone ( 15 mg/kg), followed by prednisone 15 mg/day, prudently tapered. Ten months post-partum, while still taking low-dose prednisone (8 mg/day), her only complaints are minor sporadic arthralgias.
Our case met all the criteria proposed by Yamaguchi et al. [ 1] for definite ASD. To date, 33 pregnancies have been reported in 24 patients with ASD [2.14]. The precise role of pregnancy in the onset and course of ASD is unclear since improvement, flare or indifference toward ASD have been described during both pregnancy and post-partum, even in the same multiparous patient.
Corticosteroids, the mainstay of treatment in ASD, proved efficacious and relatively safe in pregnancyassociated ASD. Our patient, however, posed a therapeutic challenge: firstly, she experienced unacceptable hepatic toxicity of salicylates; secondly, because she suffered excessive weight gain early in pregnancy, the use of corticosteroids was hampered by a high risk of developing diabetes gestationis. Recent favourable results obtained with IVIg in ASD [15, 16], even in cases previously refractory to NSAIDs, encouraged us to treat this patient with IVIg. IVIg, alone or in combination with short-pulse high-dose steroids, resulted in complete remission of ASD with excellent safety for the mother and the baby. IVIg might be considered a potential alternative to NSAIDs and steroids in pregnant women with ASD.
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E. LIOZON, K. LY, Y. AUBARD1, E. VIDAL
Departments of Internal Medicine and 'Obstetrics and Gynaecology, Dupuytren's University Hospital, 87042 Limoges, France
Accepted 28 April 1999
Correspondence to: E. Liozon, Service de Medecine Interne A, Hopital Universitaire Dupuytren, 2 avenue Martin Luther-King, 87042 Limoges, France
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