Juvenile‐onset ankylosing spondylitis is associated with worse functional outcomes than adult‐onset ankylosing spondylitis

Objective

To compare functional outcome of patients with juvenile‐onset ankylosing spondylitis (JoAS; defined as AS with symptom onset before 16 years of age) with that of patients with adult‐onset AS (AoAS) and to identify variables associated with a poor functional outcome of JoAS.

Methods

A cross‐sectional study was performed of 326 JoAS patients who participated in a postal survey conducted by the Spondylitis Association of America. This cohort was compared with 2,021 AoAS patients who participated in the same survey. Simple and multiple logistic regression analyses were performed to identify differences with respect to clinical features, demographic features, and functional outcome (defined by the Bath Ankylosing Spondylitis Functional Index [BASFI]) between the 2 groups. A validation cohort of 255 AS patients was also surveyed.

Results

The mean ± SD BASFI score (controlled for disease duration) for JoAS was 51.3 ± 1.5 compared with 46.4 ± 0.6 for AoAS (P < 0.0001). Multiple logistic regression identified only age (P < 0.0001) and income status (P < 0.0001) as factors associated with functional impairment.

Conclusion

It appears that JoAS is a progressive disease and is associated with significant delay in diagnosis and worse functional outcome compared with AoAS. Furthermore, women do worse than men with JoAS. This would argue for the importance of early diagnosis and treatment of AS, particularly in the subgroup of patients with JoAS.

     

Late‐onset severe long QT syndrome

We report a case of torsades de pointes arrhythmia as the first manifestation of congenital Long QT syndrome in a 77‐year‐old man with family history of sudden unexplained death. This case illustrates the importance of vigilant clinical assessment and genetic counseling in families with sudden death in order to identify properly asymptomatic relatives at risk for cardiac events. It also demonstrates that Long QT syndrome can still manifest with potentially fatal arrhythmias late in life in previously asymptomatic elderly patients.     

Perinatal factors and adult‐onset lupus

Objective

Some evidence suggests that perinatal factors, including birth weight and breastfeeding, may influence the occurrence of autoimmune rheumatic diseases. However, few studies have investigated these factors in patients with systemic lupus erythematosus (SLE). Therefore, we evaluated the role of birth weight, being breastfed, and preterm birth on the incidence of SLE in participants in the Nurses' Health Study (NHS) and the Nurses' Health Study II (NHSII).

Methods

We studied 87,411 NHS participants and 98,413 NHSII participants without SLE at baseline who provided information on perinatal exposures. Among these women, during 26 (NHS) and 14 (NHSII) years of followup, 222 incident SLE cases were confirmed (136 NHS and 86 NHSII) by medical record review using American College of Rheumatology criteria. We used stratified Cox models to estimate the association of perinatal factors with SLE, adjusting for race, early passive cigarette smoke exposure, and parents' occupation. A random‐effects meta‐analysis was used to compute combined estimates across the 2 cohorts.

Results

After adjustment for multiple potential confounders, high birth weight (≥10 pounds) was associated with increased rates of SLE compared with normal birth weight (7–8.5 pounds; rate ratio [RR] 2.7, 95% confidence interval [95% CI] 1.2–5.9), as was being born ≥2 weeks preterm (RR 1.9, 95% CI 1.2–3.0); however, being breastfed was not (RR 0.8, 95% CI 0.6–1.1).

Conclusion

Birth weight ≥10 pounds and preterm birth were both positively associated with incident SLE among women.     

New‐onset diabetes after renal transplantation

Renal transplantation has important benefits in people with end‐stage renal disease, with improvements in mortality, morbidity and quality of life. Whilst significant advances in transplantation techniques and immunosuppressive regimens have led to improvements in short‐term outcomes, longer‐term outcomes have not improved dramatically. New‐onset diabetes after transplantation appears to be a major factor in morbidity and cardiovascular mortality in renal transplant recipients. The diagnosis of new‐onset diabetes after renal transplantation has been hampered by a lack of clarity over diagnostic tests in early studies, although the use of the WHO criteria is now generally accepted. HbA_1c may be useful diagnostically, but should probably be avoided in the first 3 months after transplantation. The pathogenesis of new‐onset diabetes after renal transplantation is likely to be related to standard pathogenic factors in Type 2 diabetes (e.g. insulin resistance, β‐cell failure, inflammation and genetic factors) as well as other factors, such as hepatitis C infection, and could be exacerbated by the use of immunosuppression (glucocorticoids and calcineurin inhibitors). Pre‐transplant risk scores may help identify those people at risk of new‐onset diabetes after renal transplantation. There are no randomized trials of treatment of new‐onset diabetes after renal transplantation to determine whether intensive glucose control has an impact on cardiovascular or renal morbidity, therefore, treatment is guided by guidelines used in non‐transplant diabetes. Many areas of uncertainty in the pathogenesis, diagnosis and management of new‐onset diabetes after renal transplantation require further research.     

Late‐onset acetaminophen‐induced allergic hepatitis with progression to chronicity

Acetaminophen (paracetamol), a widely used antipyretic/analgesic, is a well‐known agent causing acute hepatic injury. Whereas most cases are caused by its intrinsic hepatotoxicity, idiosyncratic hepatitis by the allergic mechanism is extremely rare. We herein report a case of late‐onset acetaminophen‐induced allergic hepatitis with progression to chronicity. This unique case extends the spectrum of acetaminophen‐induced liver injury. Clinicians should be aware of this unusual clinical manifestation. The mechanism underlying the immunological reaction to acetaminophen remains to be elucidated.     

Childhood‐onset Takayasu arteritis: an update

Childhood‐onset Takayasu arteritis (c‐TA) is a distinct subset affecting a wide age group, ranging from young infants to adolescents and it differs from adult TA in many aspects. There is scarcity of data on c‐TA worldwide. The disease is classified using the European League Against Rheumatism/Pediatric Rheumatology International Trials Organization/Pediatric Rheumatology European Society criteria. The non‐specific nature of presenting complaints and lack of appropriate biomarkers delay the early diagnosis of this illness and many children present with complications, which become irreversible once they set in. One of the largest cohorts of 40 children with c‐TA from our center reports hypertension as the commonest presenting feature. Systemic symptoms like headache, fever and weight loss are also described. Assessment of disease in c‐TA is done by correlating clinical features with raised inflammatory markers. Advanced imaging plays an important role in diagnosis. In c‐TA, the role of magnetic resonance angiography is advocated, taking into consideration the enormous amount of radiation exposure with other modalities. Complications of c‐TA include cardiovascular, pulmonary, neurological and those arising secondary to long‐term steroid and immunosuppression therapy.     

Late‐onset achalasia after esophageal atresia repair

The development of achalasia in a patient with a history of esophageal atresia (EA) is rare. Here, we report a patient who had undergone surgery for EA at birth and presented achalasia at 30 years of age. He was successfully treated with laparoscopic surgery.     

Juvenile case with the coexistence of maturity‐onset diabetes of the young 1 and later‐onset latent autoimmune diabetes in youth

We present the case of a 12‐year‐old Japanese girl, who was positive for markers of both maturity‐onset diabetes of the young and latent autoimmune diabetes in youth. She was initially diagnosed with maturity‐onset diabetes of the young 1 based on the molecular analysis, and she later developed an autoimmune response, leading to β‐cell‐associated antibody‐positive diabetes. She was treated with incretin‐associated drugs and maintained adequate glycemic control. Pathophysiologically, there was an overlap between the two different types of diabetes, because the hyperglycemia and β‐cell stress seen in non‐autoimmune diabetes can cause β‐cell autoimmunity over time.