Last week's articles on rheumatology topics in the major non-specialty journals.
Juvenile Idiopathic Arthritis
Among children with juvenile idiopathic arthritis (JIA) who had undergone primary immunization, a booster vaccination with live attenuated measles-mumps-rubella (MMR) vaccine did not result in worse disease, and was immunogenic. In the Netherlands, 137 patients aged 4 to 9 years old were randomized into booster vaccinations or no vaccinations. The study included 60 patients using methotrexate and 15 using biologics; the latter were discontinued before vaccination. The authors call for larger studies to clarify the effects on children taking biologics.
Endosonography vs Conventional Bronchoscopy for the Diagnosis of Sarcoidosis: The GRANULOMA Randomized Clinical Trial
JAMA, June 19, 2013 Full text $30
Endosonographic nodal aspiration resulted in greater diagnostic yield than bronchioscopic biopsy (80% vs. 53%) for patients with suspected stage I/II pulmonary sarcoidosis undergoing tissue confirmation. Three hundred and four patients were randomized to bronchoscopy or endosonography. There were two serious adverse events in the bronchoscopy group and one in the endosonography group.
Editorial: Real-world Experience in the Midst of an Exserohilum Meningitis Outbreak
JAMA, June 19, 2013., Full text $30
When screened by MRI, about one in five patients who received an injection of methylprednisolone from a contaminated lot but had not complained or presented for treatment showed probable or confirmed fungal spinal or paraspinal infection, report authors from the University of Michigan and other medical institutions in Ann Arbor. Of 172 patients screened by MRI for infection at the injection site, 36 (21%) had an abnormal MRI, and 35 had probable or confirmed infection. All 35 were treated with antifungal agents, and 24 required surgical debridement. Meningitis spiked quickly, but spinal and paraspinal infections that continue to present can be obscured when back pain or neuropathic symptoms are the same as those for which the injections were given.
The Centers for Disease Control and Prevention advises that patients with new or worsening symptoms near the injection site should undergo MRI with contrast. The authors recommend MRIs for patients with persistent pain, even if it is not worsening, if they received spinal injections from a contaminated lot. Some lots and some procedures, such as peripheral joint injections, were also associated with lower risks, however. An editorial maintains screening may not be necessary in these cases.
Race and Association With Disease Manifestations and Mortality in Scleroderma: A 20-Year Experience at the Johns Hopkins Scleroderma Center and Review of the Literature
Medicine, June 20, 2013 online only. Full text $49
Race was related to a distinct phenotypic profile in scleroderma, and a worse prognosis among African Americans, in a 20-year evaluation of 409 African American and 1,808 white patients, at Johns Hopkins. Both groups were 82% female. African American patients presented at a younger age (47 vs 53 years), and were more likely to have diffuse rather than limited cutaneous disease. Cumulative mortality at 10 years was 43% among African Americans vs 35% among whites, for a relative risk (RR) of 1.8, which was reduced to 1.3 when adjusted for other factors. Whites were more likely to have anticentromere antibody, while African Americans were more likely to have topoisomerase autoantibody. African Americans were about half again as likely to have muscle disease or cardiac, renal, or digital ischemia, and seven times as likely to have restrictive lung disease.
IgG4-Related Disease and Hypertrophic Pachymeningitis
Medicine, June 20, 2013, online only, $49
IgG4-related disease may be the most common cause of noninfectious hypertrophic pachymeningitis. Fourteen pathology specimens were reviewed according to recent consensus guidelines on histopathology. IgG4-related disease represented 29% of cases. Other cases were associated with granulomatosis with polyangiitis, lymphoma, rheumatoid arthritis, giant cell arteritis, and sarcoiditis. Clinical history, serologic tests, cerebrospinal fluid studies, and radiology alone could not identify the cause; biopsy with histopathology and immunostaining is necessary.
Prevalence of the Antiphospholipid Syndrome and Its Effect on Survival in 679 Chinese Patients With Systemic Lupus Erythematosus: A Cohort Study
Medicine, June 20, 2013, online only. Full text $49
Antiphospholipid syndrome (APS) is less common in patients with systemic lupus erythematosus (SLE) from southern China than it is among Caucasians. But when APS occurs, it more commonly manifests as arterial rather than venous thrombosis, and arterial thrombosis is more deadly. This report followed 679 southern Chinese patients with SLE (92% women) for 10 years, during which time 68 patients (10%) died. Forty-four (6.5%) met the criteria for APS, manifested by ischemic stroke, deep venous thrombosis, obstetric morbidity, cardiovascular events, and peripheral vascular disease. APS itself did not significantly affect mortality, but APS caused by arterial thrombosis was significantly associated with mortality (hazard ratio 2.3).