Combined Medicine-Pediatrics Fellowships: A Guide for Fellowship Directors and Residents.

Dual training in Internal Medicine-Pediatrics (MedPeds) was recognized by the American Board of Medical Specialties in 1967. Residents complete 24 months each in Internal Medicine and Pediatrics and are board-eligible for both at the conclusion of training. Graduates are eligible for fellowships in either or both fields. Many graduates pursue fellowship training. A small absolute number of graduates apply for dual training in adult and pediatric subspecialties, but those that do bring direct, in-depth clinical experience across the lifespan, and familiarity with care in both pediatric and adult settings. As such, they contribute unique perspectives and capabilities to their fellowship and future practice. This includes the ability to provide subspecialty care in settings with limited resources, where they are able to address needs without age restrictions, and in the transition of subspecialty care for emerging adults with childhood-onset conditions. Due to the small number of applicants pursuing joint adult and pediatric fellowships, many fellowship directors may have limited experience with dual fellowships but may want to create opportunities for these unique trainees. This summary was developed jointly by residents, fellows, MedPeds program directors, and fellowship directors in Pediatrics and Internal Medicine subspecialties, and approved by their respective leadership councils to offer some key points on common questions, suggest additional resources, and share best practices, with a goal of facilitating this process for fellowship programs and residents alike.

Antisynthetase syndrome presenting as rheumatoid-like polyarthritis.

BACKGROUND: The antisynthetase syndrome is a systemic inflammatory disease associated with anti-tRNA synthetase antibodies and consisting of the clinical features of inflammatory myopathy arthritis, interstitial lung disease (ILD), fever, Raynaud syndrome, and rash. It rarely presents with symmetric arthritis as the initial manifestation of the disease. OBJECTIVE: The aim of the study was to describe the clinical, laboratory, and radiographic characteristics of patients with antisynthetase syndrome who presented with symptoms of inflammatory arthritis, mimicking rheumatoid arthritis (RA) at the time of initial evaluation. METHODS: Six cases derived from a single university-based rheumatology clinic in Wisconsin are presented. The major clinical, laboratory, radiographic, and histopathologic data are described. RESULTS: All 6 patients demonstrated symmetric synovitis involving the hands. Five patients met the American College of Rheumatology classification criteria for RA. Three patients had nail-fold capillary abnormalities, and 4 patients were observed to have Raynaud phenomenon. Three patients demonstrated a cytoplasmic pattern when testing for antinuclear antibodies by immunofluorescent assay, and all had t-RNA synthetase antibodies. Two patients had positive rheumatoid factors, but none had strongly positive cyclic citrullinated peptide antibodies. None of the patients demonstrated radiographic erosions. All patients had evidence of ILD by imaging or pulmonary function testing. Prognosis was generally favorable, although disease severity and treatment varied considerably. CONCLUSION: In patients who present with features mimicking but atypical for RA, such as early ILD, nail-fold capillary abnormalities, Raynaud phenomenon, cytoplasmic antinuclear antibody pattern, negative cyclic citrullinated peptide antibody status, and nonerosive arthritis, the antisynthetase syndrome should be considered.

Outcome measurements in scleroderma: results from a delphi exercise.

OBJECTIVE: To obtain a consensus on the minimal clinically relevant treatment effect in various scleroderma disease outcome measures to be used in future clinical trials. METHODS: A Delphi consensus building exercise using a survey was sent out to members of the Scleroderma Clinical Trials Consortium (SCTC). The 65 SCTC members were divided into 2 groups. Group 1 was informed, in a cover letter, of the usual American College of Rheumatology 20% response results in randomized trials using effective biologic treatments for rheumatoid arthritis, while Group 2 was not. The first round of the exercise presented the scleroderma experts with a survey composed of 95 questions/clinical scenarios divided into 8 categories. These included situations where the treatment group improved, or worsened, or where some outcome measures improved, while others worsened. From the responses of this first round, a mean, mode, median, and range of responses for each of the 95 questions was obtained. This information was sent out, in the second round of the Delphi exercise, only to those respondents who answered the first round. The respondent's previous answer and the mean and range from the first round were provided for each question. It gave respondents the option to change any of their initial responses. The median of their responses in the second round was used to calculate the values for the minimal clinically relevant treatment effect. RESULTS: Thirty-two of the 65 SCTC members returned the first round of the Delphi exercise. Twenty-eight members returned the second round. Intraclass correlation coefficients between responses to round 1 and 2 were calculated for the questions. These varied from 0.99 (excellent agreement) to 0.02 (poor agreement). The p value was under 0.09 for 9 questions and under 0.19 for 20 questions. Standard deviations (SD) were calculated and were found to be lesser for each of the questions in round 2 when compared to the SD in responses from round 1, thus indicating a movement towards a consensus by the second round. An average of 33% of the responses were changed by the respondents in the second round of the Delphi exercise to a value closer to the median/average of the first round's responses. A range in required values for the minimal clinically relevant treatment effect for Modified Rodnan skin score is 3 to 7.5 units, Health Assessment Questionnaire Disability Index (HAQ-DI) 0.2 to 0.25 units, HAQ pain 0.2 to 0.3 units, MD global (100 mm visual analog scale) 8 to 13, patient global assessment 10 to 12, and diffusing capacity (percentage predicted) 9 to 10. The scenarios were especially weighted towards overall disease modification, thus organ-specific measures, such as 6 minute walk time (which has been used in many pulmonary artery hypertension trials), forced vital capacity, and a dyspnea rating (which may be important in scleroderma lung trials), were not included in the survey. CONCLUSION: Our study begins to address the current deficiency in our knowledge of appropriate values for the minimal clinically relevant treatment effect in various scleroderma disease outcome measures. A consensus could be achieved, or at least a range of minimal clinically relevant treatment effect values could be found for several outcome measurements. Of course, this consensus statement will be modified by evidence as it accrues in each consensus area.

Myasthenia gravis in a patient with pauciarticular juvenile chronic arthritis.

There have been reported cases of children with histories of pauciarticular juvenile chronic arthritis (JCA) later developing myasthenia gravis (MG) as young adults. This is intriguing because it had been considered rare to diagnose a second autoimmune disease in a patient with pauciarticular JCA, unlike in those with adult-onset rheumatoid arthritis. We report a case of MG in a 20-year-old woman with a history of pauciarticular JCA. She presented with bilateral ptosis, weakness, and a history of dysphagia. The diagnosis was confirmed with positive serum acetylcholine receptor antibodies (2000 nm/L) and electromyography showing a decremental response to repetitive muscle stimulation. The patient's inflammatory arthritis was quiescent at diagnosis. The patient underwent a surgical thymectomy and was treated with pyridostigmine, intravenous immunoglobulin, and corticosteroids with a fluctuating clinical course. Previous cases have been reported of MG associated with this subtype of JCA, suggesting a connection in autoimmune pathology. The earlier recognition and management of MG in a patient with pauciarticular JCA presenting with weakness may improve the prognosis of this disease.