Eric L. Matteson

An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features

Many patients with an idiopathic interstitial pneumonia (IIP) have clinical features that suggest an underlying autoimmune process but do not meet established criteria for a connective tissue disease (CTD). Researchers have proposed differing criteria and terms to describe these patients, and lack of consensus over nomenclature and classification limits the ability to conduct prospective studies of a uniform cohort. The “European Respiratory Society/American Thoracic Society Task Force on Undifferentiated Forms of Connective Tissue Disease-associated Interstitial Lung Disease” was formed to create consensus regarding the nomenclature and classification criteria for patients with IIP and features of autoimmunity. The task force proposes the term “interstitial pneumonia with autoimmune features” (IPAF) and offers classification criteria organised around the presence of a combination of features from three domains: a clinical domain consisting of specific extra-thoracic features, a serologic domain consisting of specific autoantibodies, and a morphologic domain consisting of specific chest imaging, histopathologic or pulmonary physiologic features. A designation of IPAF should be used to identify individuals with IIP and features suggestive of, but not definitive for, a CTD. With IPAF, a sound platform has been provided from which to launch the requisite future research investigations of a more uniform cohort. ERS/ATS task force provides nomenclature and classification criteria for patients with IIP and autoimmune features http://ow.ly/O7qao

The Prosorba column for treatment of refractory rheumatoid arthritis: A randomized, double-blind, sham-controlled trial

OBJECTIVEnTo evaluate the efficacy and safety of the Prosorba column as a treatment for rheumatoid arthritis (RA) in patients with active and treatment-resistant (refractory) disease.nnnMETHODSnA sham-controlled, randomized, double-blind, multicenter trial of Prosorba versus sham apheresis was performed in patients with RA who had failed to respond to treatment with methotrexate or at least 2 other second-line drugs. Patients received 12 weekly treatments with Prosorba or sham apheresis, with efficacy evaluated 7-8 weeks after treatment ended. Patients were characterized as responders if they experienced improvement according to the American College of Rheumatology (ACR) response criteria at the efficacy time point. A data safety monitoring board (DSMB) evaluated interim analyses for the possibility of early completion of the trial.nnnRESULTSnPatients in the trial had RA for an average of 15.5 years (range 1.7-50.6) and had failed an average of 4.2 second-line drug treatments prior to entry. After the completion of treatment of 91 randomized patients, the DSMB stopped the trial early due to successful outcomes. Of the 47 patients in the Prosorba arm, 31.9% experienced ACR-defined improvement versus 11.4% of the 44 patients in the sham-treated arm (P = 0.019 after adjustment for interim analysis). When results from 8 additional patients, who had completed blinded treatments at the time of DSMB action, were added to the analysis (n = 99), results were unchanged. The most common adverse events were a short-term flare in joint pain and swelling following treatment, a side effect that occurred in most subjects at least once in both treatment arms. Other side effects, although common, occurred equally as frequently in both treatment groups.nnnCONCLUSIONnApheresis with the Prosorba column is an efficacious treatment for RA in patients with active disease who have failed other treatments.

Use of Methotrexate for Autoimmune Hearing Loss

To assess the efficacy of low-dose methotrexate (MTX) given long-term for the treatment of autoimmune hearing loss, we performed a prospective open-label study of 11 patients with treatment-refractory autoimmune hearing loss. All patients had ongoing episodic worsening of hearing in 1 or both ears before enrollment despite traditional medical therapy. The MTX dose was 7.5 to 17.5 mg/wk. Hearing loss and vertigo were evaluated at baseline and at completion of the study. Hearing improvement was defined as an improvement in the pure tone threshold (PT) average of >10 dB or an increase in speech discrimination (SD) of >15%, whereas worsening was defined as a worsening of >10 dB in PT or a decrease of >15% in SD in at least 1 ear. The MTX was well tolerated. Among the 6 patients with Menieres disease, 4 had improvement or resolution of vertigo, while 2 had no improvement. Disequilibrium improved in all 3 patients with Cogans syndrome. According to the parameters defined above, hearing improved in 9 patients (82%), was unchanged in 1 patient (9%), and worsened in 1 patient (9%). Long-term low-dose MTX therapy may be a useful therapy for some patients who have hearing loss with a presumptively autoimmune-mediated component that is refractory to traditional therapies.

Presidents’ Day reflections. Why Donald Trump could not be a rheumatologist

(1) It does not pay enough. (2) We aspire to lofty ethical imperatives. (3) We seek, and are able to recognize, truth. (4) We believe in science—not Bjunk^ scienceand evidence-based practice. (5) We aim to help people. (6) We care about others, particularly the less fortunate. (7) We embrace altruism and put our patients’ needs before our own. (8) We try to think, reason, and express ourselves in more than 144 characters. (9) We strive to be humanistic, and (10) We fundamentally endeavor to respect others.

Polymyalgia Rheumatica and its Association with Cancer.

OBJECTIVEnPolymyalgia rheumatica (PMR) is a common rheumatologic disease in the elderly population. Studies on the relationship between PMR and cancer have yielded mixed results and have been limited by multiple factors. This study examined the association between PMR and development of cancer in a community cohort.nnnMETHODSnA population-based cohort of 359 patients with PMR diagnosed between 1/1/1970 and 12/31/1999 and followed to 12/31/2013 was assembled along with a comparison cohort of 357 subjects. Records of the PMR and comparator subjects were reviewed for details concerning diagnosis of cancer. The cumulative incidence of malignancy in patients with and without PMR, adjusted for the competing risk of death, was estimated and compared using methods of Gray. Cox proportional hazards models were used to assess the trends in malignancy over time.nnnRESULTSnThere was no significant difference in the prevalence of malignancy prior to PMR incidence date/index date between the two groups with prior malignancies in 41 (11%) of patients with PMR, and 50 (14%) of non-PMR subjects (p-value=0.31). As well, there was no difference in the cumulative incidence of malignancy at 10 years following PMR incidence between patients with PMR and non-PMR subjects (cumulative incidence at 10 years ± SE: PMR 13.8 ± 2.0, control 13.1 ± 2.0; p-value=0.89).nnnCONCLUSIONnThere is no increased risk of malignancy in patients who are diagnosed with PMR when compared to subjects without PMR in this population-based cohort.

Risk of Cardiovascular Disease and Venous Thromboembolism Among Patients With Incident ANCA-Associated Vasculitis: A 20-Year Population-Based Cohort Study

Objective: To assess the cardiovascular disease (CVD) and venous thromboembolism (VTE) risks among patients with newly diagnosed antineutrophil cytoplasmic antibodies (ANCA)‐associated vasculitis (AAV). Patients and Methods: A population‐based incident AAV cohort of 58 patients diagnosed between 1996 and 2015 in Olmsted County, MN, was identified by medical record review. For each patient, 3 age‐ and sex‐matched non‐AAV comparators were randomly selected from the same population and assigned an index date corresponding to the AAV incidence date. Medical records of cases and comparators were reviewed for CVD events, which included cardiac events (coronary artery disease, heart failure, and atrial fibrillation), cerebrovascular accidents (CVA), peripheral vascular disease (PVD), and VTE, which included deep vein thrombosis (DVT) and pulmonary embolism (PE). Results: Baseline total cholesterol, high‐density lipoprotein, and current smoking rate were lower in AAV than in comparators (P=.03, P=.01, and P=.04, respectively), whereas other CVD risk factors and Framingham risk score were not significantly different between the 2 groups. The CVD events developed in 13 patients and 17 comparators, corresponding to a more than 3‐fold increased risk (hazard ratio [HR], 3.15; 95% CI, 1.51‐6.57). By subtypes, risks were increased for cardiac events (HR, 2.96; 95% CI, 1.42‐6.15) and CVA (HR, 8.16; 95% CI, 2.45‐27.15), but not for PVD. The HR for VTE was 3.26 (95% CI, 0.84‐12.60), significantly increased for DVT (HR, 6.25; 95% CI, 1.16‐33.60), but not for PE (HR, 1.33; 95% CI, 0.23‐7.54). Conclusion: Despite a similar prevalence of CVD risk factors at baseline, the risk of CVD is more than 3‐fold higher and for CVA 8‐fold higher in patients with incident AAV than in matched comparator subjects.

Cardiovascular Risk in Psoriasis: A Population-Based Analysis with Assessment of the Framingham Risk Score

Objective. To examine the utility of the Framingham risk score (FRS) in estimating cardiovascular risk in psoriasis. Methods. We compared the predicted 10-year risk of cardiovascular events, namely, cardiovascular death, myocardial infarction, heart failure, percutaneous transluminal coronary angioplasty, and coronary artery bypass grafting using the FRS, to the observed risk of cardiovascular events in a population-based cohort of patients with psoriasis. Patients with incident or prevalent adult-onset psoriasis aged 30–79 years without prior history of cardiovascular disease were included. Results. Among the 1197 patients with predicted risk scores, the median FRS was 6.0%, while the observed 10-year cardiovascular risk was 6.9% (standardized incidence ratio (SIR): 1.14; 95% confidence interval (CI): 0.92–1.42). The SIR was not elevated for women nor for men. The differences between observed and predicted cardiovascular risks in patients <60 years (SIR: 1.01; 95% CI: 0.73–1.41) or ≥60 years (SIR: 1.26; 95% CI: 0.95–1.68) were not statistically significant. Conclusion. There was no apparent difference between observed and predicted cardiovascular risks in patients with psoriasis in our study. FRS reasonably estimated cardiovascular risk in both men and women as well as in younger and older psoriasis patients, suggesting that FRS can be used in risk stratification in psoriasis without further adjustment.