New Roadmap for the Journey From Internist to Rheumatologist.

OBJECTIVE: Measurement is necessary to gauge improvement. US training programs have not previously used shared standards to assess trainees' mastery of the knowledge, skills, and attitudes necessary to practice rheumatology competently. In 2014, the Accreditation Council for Graduate Medical Education (ACGME) Next Accreditation System began requiring semiannual evaluation of all medicine subspecialty fellows on 23 internal medicine subspecialty reporting milestones. Since these reporting milestones are not subspecialty specific, rheumatology curricular milestones were needed to guide rheumatology fellowship training programs and fellows on the training journey from internist to rheumatologist. METHODS: Rheumatology curricular milestones were collaboratively composed by expanding the internal medicine reporting milestones to delineate the specific targets of rheumatology fellowship training within 6 ACGME core competencies. The 2006 American College of Rheumatology core curriculum for rheumatology training programs was updated. RESULTS: A total of 80 rheumatology curricular milestones were created, defining progressive learning through training; most focus on patient care and medical knowledge. The core curriculum update incorporates the new curricular milestones and rheumatology entrustable professional activities. CONCLUSION: Rheumatology curricular milestones are now available for implementation by rheumatology fellowship training programs, providing a clear roadmap for specific training goals and a guide to track each fellow's achievement over a 2-year training period. The comprehensive core curriculum delineates the essential breadth of knowledge, skills, and attitudes that define rheumatology, and provides a guide for educational activities during fellowship training. These guiding documents are now used to train and assess fellows as they prepare for independent rheumatology practice as the next generation of rheumatologists.

What Is a Rheumatologist and How Do We Make One?

OBJECTIVE: Graduate medical education is a critical time in the training of a rheumatologist, and purposeful evaluation of abilities during this time is essential for long-term success as an independent practitioner. The internal medicine subspecialties collectively developed a uniform set of reporting milestones by which trainees can be assessed and receive formative feedback, providing clarity of accomplishment as well as areas for improvement in training. Furthermore, the reporting milestones provide a schema for assessment and evaluation of fellows by supervisors. The internal medicine subspecialties were also tasked with considering entrustable professional activities (EPAs), which define the abilities of a subspecialty physician who has attained sufficient mastery of the field to be accountable to stakeholders and participate in independent practice. Although EPAs have been established for a few specialties, they had not yet been described for rheumatology. EPAs have value as descriptors of the comprehensive abilities, knowledge, and skills of a practicing rheumatologist. The rheumatology EPAs have a role in defining a specialist in rheumatology upon completion of training, and also represent the ways our specialty defines our abilities that are enduring throughout practice. METHODS: We describe the collaborative process of the development of both the subspecialty reporting milestones and the rheumatology EPAs. The reporting milestones evolved through discussions and collaborations among representatives from the Association of Specialty Professors, the Alliance for Academic Internal Medicine, the American Board of Internal Medicine, and the Accreditation Council for Graduate Medical Education. The EPAs were a product of deliberations by the Next Accreditation System (NAS) working group of the American College of Rheumatology (ACR) Committee on Rheumatology Training and Workforce Issues. RESULTS: Twenty-three subspecialty reporting milestones and 14 rheumatology EPAs were advanced and refined over the course of 3 subspecialty reporting milestone development summits and 3 ACR NAS working group meetings, respectively. CONCLUSION: The subspecialty reporting milestones and rheumatology EPAs presented here stipulate reasonable and measurable expectations for rheumatologists-in-training. Together, these tools aim to promote enrichment and greater accountability in the training of fellows. Additionally, the EPAs define, for all stakeholders, the expertise of a rheumatologist in practice.

Fatal antimalarial-induced cardiomyopathy: report of 2 cases.

Chloroquine and hydroxychloroquine are used to chronically treat certain rheumatologic diseases and are generally considered safe. We describe 2 patients with skeletal myopathy and fatal cardiomyopathy-uncommon and underrecognized adverse effects of these agents. Both patients developed arrhythmias and heart failure, and 1 patient had documented diaphragmatic involvement. Muscle specimens showed typical vacuolar myopathy (indicative of impaired autophagy) with myeloid bodies in both patients and curvilinear bodies in 1 patient. Antimalarial-induced cardiomyopathy should be considered in patients receiving these medications with otherwise unexplained muscle weakness or cardiac symptoms. Whether autophagy enhancers can be used to manage such myopathies merits investigation.

Methotrexate increases expression of cell cycle checkpoint genes via JNK activation.

OBJECTIVE: To assess defects in expression of critical cell cycle checkpoint genes and proteins in patients with rheumatoid arthritis (RA) relative to presence or absence of methotrexate (MTX) treatment, and to investigate the role of JNK in induction of these genes by MTX. METHODS: Flow cytometric analysis was used to quantify changes in levels of intracellular proteins, measure reactive oxygen species (ROS), and determine apoptosis in different lymphoid populations. Quantitative reverse transcription-polymerase chain reaction was used to identify changes in cell cycle checkpoint target genes. RESULTS: RA patients expressed reduced baseline levels of MAPK9, TP53, CDKN1A, CDKN1B, CHEK2, and RANGAP1 messenger RNA (mRNA) and JNK total protein. The reduction in expression of mRNA for MAPK9, TP53, CDKN1A, and CDKN1B was greater in patients not receiving MTX than in those receiving low-dose MTX, with no difference in expression levels of CHEK2 and RANGAP1 mRNA between MTX-treated and non-MTX-treated patients. Further, JNK levels were inversely correlated with C-reactive protein levels in RA patients. In tissue culture, MTX induced expression of both p53 and p21 by JNK-2- and JNK-1-dependent mechanisms, respectively, while CHEK2 and RANGAP1 were not induced by MTX. MTX also induced ROS production, JNK activation, and sensitivity to apoptosis in activated T cells. Supplementation with tetrahydrobiopterin blocked these MTX-mediated effects. CONCLUSION: Our findings support the notion that MTX restores some, but not all, of the proteins contributing to cell cycle checkpoint deficiencies in RA T cells, via a JNK-dependent pathway.

Granulomatous disorders of the nose and paranasal sinuses.

PURPOSE OF REVIEW: To review granulomatous findings in sinus and nasal tissue as part of a diagnostic indicator of various disease states, focusing on the role of further testing and evaluation to clarify this diagnosis as well as the implications for patient care. RECENT FINDINGS: Inflammatory and infectious diseases as well as neoplasms, cocaine abuse and trauma may have rhinosinus granulomatous findings as part of the disease state. The need for careful histopathologic evaluation as well as the pitfalls and caveats of laboratory testing will be reviewed in this paper. Some infections such as invasive fungal rhinosinusitis and rhinoscleroma may have a chronic granulomatous course, which may require extensive surgical and pharmacologic treatment. Treatment options for Wegener's granulomatosis have shown a lack of effect of etanercept, but hopeful alternatives to prolonged cyclophosphamide use include methotrexate and leflunomide. Cocaine-induced midline destructive lesions unfortunately have a high prevalence of cytoplasmic antineutrophil cytoplasmic antibodies, limiting this test's usefulness in distinguishing this disorder from Wegener's granulomatosis. SUMMARY: The otorhinolaryngologist must be aware of the differential diagnosis of these chronic inflammatory states to formulate an optimal course of evaluation and longitudinal management for these patients.

Color Doppler ultrasonographic findings show tendon sheath inflammation in mechanic's hands.

Two male patients with mechanics hands and concomitant interstitial lung disease, Raynaud's phenomenon, dermatomyositis-like rash, and arthritis were evaluated with gray-scale ultrasonography (US) and color Doppler US of the hands using a high-resolution hockey stick configuration 10-MHz transducer (ATL3000). Color Doppler US and gray scale US images of the flexor tendons were obtained at the level of the metacarpophalangeal joint. Initial gray-scale US demonstrated subcutaneous tissue and tendon thickening and synovial sheath effusion; color Doppler US demonstrated hyperemia of the subcutaneous tissues and tendons. Flexor tendon dysfunction on physical examination was proportional to hyperemia and thickening of structures shown by US. Follow-up examination, at a time when patients were improved, showed decreased blood flow in tendon sheaths and subcutaneous tissues and resolution of synovial sheath effusion; one patient, however, demonstrated accumulation within the sheath. Relative improvement in functional status mirrored the changes in the US. The color Doppler US finding of increased blood flow in the soft tissues of these two patients with mechanic's hands represents a physiologic anatomic abnormality amenable to treatment and provides prognostic information for these patients. Mechanic's hands and tenosynovitis occur in patients like these without muscle weakness.