Background Pregnant women with autoimmune disease (AID), particularly systemic lupus erythematosus (SLE), have increased risks of disease exacerbation, pre/eclampsia and other maternal and fetal complications. Low birth weight (LBW) for gestational age, preterm delivery and neonatal lupus (NL), which occurs in 1.5% of newborns of mothers with SLE, are some of the associated fetal complications. Objectives To report the maternal and fetal morbidities associated with deliveries by women with AID at a regional hospital. Methods Retrospective data were collected between 2003 and 2010 at a regional hospital. The reference area included 300,000 inhabitants. We analyzed the clinical and laboratory characteristics of mothers with AID and the complications experienced by their newborns. We recorded the maternal and neonatal disorders arising from this disease. Results Over this 7-year period, 29 mothers were analyzed. There were 52 pregnancies, which resulted in 39 newborns. There were 13 miscarriage (45%), all of them in mothers with antiphospholipid syndrome (APS). The AIDs of the mothers were 19 SLE (67%), 3 cutaneous lupus, 3 primary Sjögren’s syndrome, 2 mixed connective tissue disease, 1 primary APS and 1 systemic sclerosis. There were 8 instances of maternal complications (26%) during the pregnancies including 1 digital vasculitis, 1 pancreatitis, 1 outbreak of glomerulonephritis, 1 gestational diabetes, 3 preeclampsia and 1 eclampsia. One patient experienced significant improvement of her arthritis. During the postpartum period, there was 1 case of lupus fever and 1 case of eclampsia, pneumonitis and respiratory failure with a favorable outcome. Among the newborns, 7 were preterm (21%), 8 had LBW (35%) and 4 exhibited the transplacental passage of maternal antibodies (1anti-Sm [1], 1 anti-Ro, 1 anti-Ro plus anti-La and 1 anti-Ro plus anti-RNP). One newborn developed transient cutaneous NL with the transplacental passage of maternal anti-Sm antibodies [1]. Electrocardiography (ECG) was performed on 50% of the babies, and no abnormal results were observed. One of the daughters born to a mother with SLE was diagnosed with ANA negative self-limited oligoarthritis at 12 months of age. A son born to a mother with primary APS exhibited idiopathic thrombocytopenic purpura. Conclusions In our hospital, the rates of miscarriage, prematurity and LBW among the newborns of mothers with AID are similar to those reported in the literature. The observation of a case of NL with the transplacental passage of anti-Sm*, not previously reported in the literature, is remarkable. References Ortiz-Santamaria V, Olive A, Martinez-Cáceres E, Coll MT, Codina X, Surís X. Lupus 2010; 19: 659-61. Disclosure of Interest None Declared

Background Kikuchi-Fujimoto disease (KFD) is known as histiocytic necrotizing lymphadenitis. The most important signs and symptoms are cervical lymph node enlargement and fever. This disease is usually benign and self-limiting. The cause of this disease is not known, although infectious and autoimmune etiologies such as Systemic Lupus Erythematosus (SLE) have been proposed. Objectives To report clinical manifestations, laboratory findings and follow-up of patients with Kikuchi-Fujimoto disease. Methods We report 4 cases of KFD collected in a county hospital. The observation period was 1990-2010. The reference area had a population of 300.000 inhabitants. We analyzed the clinical features, laboratory findings, clinical course and its relation to SLE. Results The cases of KFD included 4 females with an average age of 23 years old (13-40) at the time of the diagnosis (see Table). One of the patients (case 3) presented at an unusually young age with atypical lymph-node localization: supraclavicular, inguinal and axillary. During follow-up, we confirmed the diagnosis of SLE in 2 patients. These diagnoses changed the therapeutic strategy in these cases. The other 2 cases are being monitored closely, due to the high probability that they will develop an autoimmune disease. Conclusions The importance of a diagnosis of KFD lies in the possible association with an autoimmune disease. KFD can precede, coincide or appear during the development of autoimmune diseases, usually SLE. A diagnosis of KFD requires histological confirmation. Disclosure of Interest None Declared

Sir, Neonatal lupus syndrome (NLS), first described in 1954 by McCuistion and Schoch, is a rare autoimmune disorder in newborns associated with antibodies against Ro, La or rarely U1RNP antigens. The most common clinical manifestations are transient cutaneous lesions and/or congenital heart block. We report a case of transient cutaneous neonatal lupus in a patient with the transplacental passage of maternal anti-Sm antibodies. To the best of the authors’ knowledge, this is the first case reported in the literature. The patient was a newborn girl, born at term by Caesarean section, weighing 2360 g and measuring 44 cm in length. Physical examination at 2 weeks of age revealed an infant in a good general condition, however a scaly erythematous rash involving the cheeks, forehead, ‘owl-like’ periocular involvement and scalp was present (Figure 1). She had no other clinical manifestations. The patient’s complete blood cell count and urinalysis were normal. The electrocardiogram was normal without any conduction abnormality. Laboratory tests revealed an antinuclear antibody (ANA) titre of 1:2560 speckled pattern (Hep2 substrate, Bio Rad). Anti-dsDNA (Crithidia, Bio Rad), anti-Ro/SSA and anti-La/SSB were negative, while anti-U1RNP/Sm antibodies and anti-Sm antibodies were positive, with an index of 5.4 and 5.1, respectively, and consequently the subtraction gave anti-U1RNP negative, index 0.3 enzyme-linked immunosorbent assay (ELISA) (index normal value <1) (INOVA Diagnostics, Inc., San Diego, CA, USA). At 5 months of age the cutaneous lesions disappeared completely and the serological tests were negative (Table 1). The infant’s mother was diagnosed of systemic lupus erythematosus 3 years before because of malar rash, photosensitivity, oral ulcers, non-erosive symmetrical arthritis involving wrists and metacarpophalangeal joints, leucopaenia (3100/mm) and lymphopaenia (800/mm). She was positive for ANA 1:2560 speckled, anti-dsDNA antibodies 1:80, anti-U1RNP/Sm antibodies index 6.48, anti-Sm antibodies index 6.24, analysed by ELISA and the subtraction gave anti-U1 RNP negative, index 0.24 (ELISA, Orgentec, Mainz, Germany). Anti RNP-A, anti-SmBB’ and anti-SmD were positive, analysed by immunoblot with recombinant antigens (Inno-lia ANA Update, Innogenetics, Heiden-Westfalen, Germany) (Table 1). Antiphospholipid antibodies were negative. She was treated with low dose corticosteroids (deflazacort 6mg/day) and antimalarials (hydroxichloroquine 200mg/day) before, during and after pregnancy. During her pregnancy she presented digital vasculitis, non-haemolytic anaemia and hypocomplementaemia. NLS is an autoimmune disease caused by the transplacental passage of maternal antibodies that occurs in about 1–2% of babies born from mothers with or without autoimmune disorders. Congenital heart block results in either fetal death due to cardiac failure or permanent injury to the conduction system, frequently requiring lifelong use of a pacemaker. NLS most commonly appears as transient cutaneous lesions without heart or other organ system involvement. The rash tends to develop after exposure to UV light. The typical cutaneous manifestations are erythematous annular lesions or arcuate macules with slight central atrophy and a raised active margin on exposed areas, mostly peri-orbital. The lesions are usually self-limiting and resolve by 6–8 months of age simultaneously with the clearance of the maternal antibodies. The antibodies most frequently associated with NLS are antibodies directed against Ro antigens. Rarely, NLS occurs in the absence of Ro antibodies, although only Laor U1RNP-positive 4,7,8 NLS have been reported. The strong association with maternal anti-Ro antibodies suggests a role for these antibodies in the pathogenesis of NLS, but the presence of these antibodies is not in itself sufficient to induce illness. Anti-RNP antibodies may also be implicated in the pathogenesis of NLS. It is not known if the presence of anti-U1RNP antibody may determine a different clinical subset of NLS. NLS with positive anti-U1RNP usually has cutaneous and haematological manifestations without cardiac involvement. It has a better prognosis than Roor La-positive NLS and does not show a female preponderance. Correspondence to: Vera Ortiz-Santamaria, Rheumatology Section, Granollers General Hospital, Francesc Ribas Avenue, 08400 Granollers, Barcelona, Spain. E-mail: 33144vos@comb.cat Received 17 March 2009; accepted 25 August 2009

A prospective study was made of all patients with normal CSF counts and positive cultures for Neisseria meningitidis diagnosed in “El Vallés” County, Barcelona between January 1987 and December 1990. Meningococcal meningitis was documented in 82 patients, eight of whom (seven children, five boys and two girls with a mean age of 5·6 ± 3·3 years, and a 69-year-old male patient) had no apparent CSF abnormalities in the initial lumbar puncture. At the time of admission all patients had fever (mean 39·1 °C) of 10·8 ± 5·6 hour duration and petechial rash which had been present for a mean of 3·6 ± 3·3 hours. Signs of meningeal irritation were not found. A 4-month-old infant with symptoms of circulatory collapse, intracranial hypertension and impairment of consciousness subsequently died of septicemia in 48 hours. Group B N. meningitidis was isolated in six cases (reduced penicillin-susceptibility in two cases) and group C N. meningitidis in the remaining two (reduced penicillin-susceptibility in one case). Patients without pleocytosis did not differ in a statistically significant fashion from the patients with high pleocytosis in the duration of temperature, and petechial rash, leukopenia, positive blood culture and fatal outcome.

The incidence and characteristics of invasive Haemophilus influenzae disease were studied in 43 adult patients admitted to the acute care hospitals in El Vallés County (Barcelona, Spain) between January 1987 and June 1992. The annual incidence of Haemophilus influenzae disease was 1.2 per 100,000 inhabitants. Pneumonia occurred in 24 patients, meningitis in five, intraabdominal infections in three, obstetric infections in two, epiglottitis in two and cellulitis in one. In six patients the source of infection was unknown. Ten (23%) of the infections were hospital acquired. Underlying conditions were diagnosed in 30 (70%) patients. Nontypeable Haemophilus influenzae strains predominated in all adult age groups. Sixty-one percent of type b and 34% of nontypeable strains were ampicillin resistant (p = 0.08). Multiple antibiotic resistance was also high among type b (53%) and nontypeable (18%) strains. The mortality rate was significantly higher in patients with pneumonia, bacteremia from an unidentified focus or shock at presentation.