Allergist-immunologists face significant challenges as experts in an ever-evolving field of neuroimmunology. Among these challenges is the increasingly frequent need to counsel patients with suspected mast cell activation disorders about perceived comorbidities, which may include hypermobile Ehlers-Danlos syndrome, amplified pain syndrome, fibromyalgia, burning sensation syndromes, migraines, irritable bowel syndrome, and postural orthostatic tachycardia syndrome. Patients may experience comorbid anxiety, panic disorder, and depression associated with disturbed sleep, fatigue, and cognitive impairment that often worsen when their physical symptoms increase in severity. These conditions may mimic mast cell activation disorders and are emotionally taxing for patients and clinicians because they are often accompanied by vague diagnostic courses, perceived unmanageability, social stigma, and significant impairment in quality of life. Combined with relatively poorly researched therapies, it is no surprise that clinicians may feel overwhelmed or find it difficult to provide consistently compassionate care for this population. In this article, we review available therapies for these conditions, which run the gamut from physical therapy to antidepressants to multimodal pain control. We highlight the benefit of multidisciplinary care within the primary care home, which includes an important role by the allergist-immunologist. By outlining simple approaches to initial treatment, we hope to empower clinicians with the tools needed to curb emotional burnout and embrace this patient population with compassion.
Nathan Zvaifler was one of the most original thinkers in the field of rheumatology contributing to modern understanding of the role of complement in mediating inflammation and the role of synoviocytes in the pathogenesis of the destructive changes of rheumatoid arthritis. His clinical acumen was unparalleled and his students are still in awe of the breadth of his knowledge.
Given the significant advance of virtual care in the past year and half, it seems timely to focus on quality frameworks and how they have evolved collaboratively across health care organizations. Massachusetts General Hospital’s (MGH) Center for TeleHealth and Mass General Brigham's (MGB) Virtual Care Program are committed to hosting annual symposia on key topics related to virtual care. Subject matter experts across the country, health care organizations, and academic medical centers are invited to participate. The inaugural MGH/MGB Virtual Care Symposium, which focused on rethinking curriculum, competency, and culture in the virtual care era, was held on September 2, 2020. The second MGH/MGB Virtual Care Symposium was held on November 2, 2021, and focused on virtual care quality frameworks. Resultant topics were (1) guiding principles necessary for the future of virtual care measurement; (2) best practices deployed to measure quality of virtual care and how they compare and align with in-person frameworks; (3) evolution of quality frameworks over time; (4) how increased adoption of virtual care has impacted patient access and experience and how it has been measured; (5) the pitfalls and barriers which have been encountered by organizations in developing virtual care quality frameworks; and (6) examples of how quality frameworks have been applied in various use cases. Common elements of a quality framework for virtual care programs among symposium participants included improving the patient and provider experience, a focus on achieving health equity, monitoring success rates and uptime of the technical elements of virtual care, financial stewardship, and clinical outcomes. Virtual care represents an evolution in the access to care paradigm that helps keep health care aligned with other modern industries in digital technology and systems adoption. With advances in health care delivery models, it is vitally important that the quality measurement systems be adapted to include virtual care encounters. New methods may be necessary for asynchronous transactions, but synchronous virtual visits and consults can likely be accommodated in traditional quality frameworks with minimal adjustments. Ultimately, quality frameworks for health care will adapt to hybrid in-person and virtual care practices.
Rheumatologists encounter patients with psychiatric illnesses daily in their practice; however, formal training in rheumatology does not sufficiently equip rheumatologists with guidance for managing common psychiatric illnesses. This study reviews common clinical situations involving psychiatric symptoms, their relationship with rheumatologic conditions, and their effects on clinical presentation and management. We illustrate key principles in a case-based format and reflect on the management of psychiatric components. Based on these discussions and a brief review of the epidemiology of psychiatric illnesses, we emphasize the prevalence and significance of these problems in daily practice.
OBJECTIVE:Telehealth is an essential facet of care delivery for patients with rheumatic diseases. The Association of American Medical College's (AAMC) telehealth competencies (TCs) define the skills required for delivering general telehealth care across the range of clinician experience. In this study, the American College of Rheumatology's (ACR) TCs working group aimed to adapt the AAMC TCs to rheumatology, outlining the skills acquisition unique to rheumatology with a focus on knowledge, skills, and behaviors expected of recent rheumatology fellowship graduates.METHODS:Through a collaborative process, the working group adapted the AAMC TCs to the training structure and practice of rheumatology. The rheumatology TCs underwent peer review among recipients of the Clinician Scholar Educator Award and attendees at the ACR 2021 Convergence conference.RESULTS:The rheumatology TCs define 24 essential skills required for synchronous telehealth care of patients with rheumatic diseases. The working group adapted the AAMC's 20 TCs organized within 6 domains, added 2 skills to the AAMC's domains of patient safety and appropriate use, and data collection and assessment, and created a novel domain of systems-based requirements with 2 competencies. The rheumatology TCs define expected skill levels for recent rheumatology fellowship graduates and experienced rheumatology clinicians.CONCLUSION:The rheumatology TCs represent the first adaptation of the AAMC TCs to subspecialty care, expanding the scope to include rheumatology fellowship graduates and additional domains of rheumatology practice. These competencies can guide curricular innovations and measurements of proficiency in telehealth care delivery among rheumatology trainees and experienced clinicians, enhancing the care provided to patients with rheumatic diseases.
Background/Objective Sarcoidosis is a granulomatous disorder. Rarely, malignancy is accompanied by a granulomatous reaction. This retrospective study addressed whether cancer can masquerade as sarcoidosis. Methods TriNetX identified 1029 newly diagnosed cases of sarcoidosis between October 2015 and September 2020, of whom 50 had a subsequent diagnosis of malignancy. Results In 36 cases, the malignancy was unrelated to sarcoidosis. In 7 cases, the diagnosis of sarcoidosis delayed the eventual cancer diagnosis by a range of 1 month to 2 years. In 7 cases, the subsequent diagnosis of cancer might have been related to the therapy for sarcoid. Conclusions Cancer masquerading as sarcoidosis is very rare occurring in 7 (0.7%) of our 1029 cases. In no case was lung cancer masquerading as sarcoidosis. Searching for occult malignancy in patients with a known diagnosis of sarcoidosis is rarely indicated.
Centers for Disease Control and Prevention published a case definition for the multisystem inflammatory syndrome in children in May 2020 when reports started pouring in about a clinical syndrome in children which was temporally associated with coronavirus disease 2019 infection. It has also been referred to as pediatric inflammatory multisystemic syndrome temporally associated with severe acute respiratory syndrome coronavirus 2. Most of these patients test positive for severe acute respiratory syndrome coronavirus 2 serology or reverse transcription-polymerase chain reaction, although a small number of patients could test negative which would require an epidemiological link to the coronavirus disease 2019 infection. The initial clinical presentation could overlap with Kawasaki disease, severe coronavirus disease 2019 infection, toxic shock syndrome, and macrophage activation syndrome. While multisystem inflammatory syndrome in children is characterized by multisystem involvement with hyper inflammation and severe clinical presentation initially, the prognosis is generally good. Since it was first described, there have been multiple studies describing the demographic characteristics, laboratory features, and treatment paradigm.
The paradigm that autoimmune diseases are abberations in the adaptive immune system is over 50 years old, but recent data suggest a multitude of abnormalities in the innate immune system in lupus and other autoimmune diseases. This viewpoint elaborates the reasons that I think it is time to reexamine this paradigm and shift our research focus to the innate immune system in lupus and other prototypic autoimmune diseases.
Pachydermodactyly is an uncommon, progressive, nontender thickening of the fingers with prominent involvement of the proximal interphalangeal joints. Pachydermodactyly mimics inflammatory arthritis but plain film radiography is normal in this condition. Pachydermodactyly has been previously described in workers performing manual labor. Mechanical stimulation has been identified as a predisposing factor in the majority of cases. We present three cases in adolescent males arising in association with excessive computer gaming.
There are more than 10,000 articles in the literature published since 1999 that appear in a search of hyperuricemia and hypouricemia for vascular events. Systematic reviews were reviewed for this time frame, numbering approximately 300 articles in addition to more than 400 reports of randomized clinical trials published since 2017. In summary, the epidemiologic associations of hyperuricemia and hypouricemia with vascular disease are confounded by comorbid conditions. The interventional data are suggestive of a relationship of gout and vascular disease and to a lesser extent hyperuricemia and hypertension; however, more interventional studies are necessary to confirm these relationships.
Objective: The Periodic fever syndromes (PFS) are a group of disorders of the innate immune system. We investigated patients diagnosed with PFS at the Dartmouth Hitchcock Pediatric Rheumatology Clinic. Methods: Case acquisition was performed by reviewing ICD 9/10 coded records for familial Mediterranean fever (ICD 9 277.31), laboratory test records for PFS genetic screening, and clinic records between 1/1/2011 and 12/31/2017. Results: Twenty-seven cases had clinical evaluations including PFS genetic screening. Clinical diagnoses included familial Mediterranean fever (FMF) (10 cases), Muckle-Wells (2 cases), tumor necrosis factor receptor associated periodic syndrome (TRAPS) (4 cases), hyper IgD syndrome (HIDS) (1 case), Crohn's Disease (1 case), systemic onset juvenile idiopathic arthritis (SoJIA) (1 case), fever of unknown origin (FUO) (1 case), periodic fever adenitis pharyngitis aphthous ulcer (PFAPA) (6 cases), and cold-induced urticaria (1 case). Fifteen cases were associated with a genetic cause. Seven of the 10 FMF cases were confirmed genetically and were either heterozygous or compound heterozygotes. Both cases of Muckle-Wells had either a compound heterozygote for CIAS 1 or a NOD gene mutation. Both TRAPS cases presented atypically with patients developing systemic lupus erythematosus (SLE) or being asymptomatic. Two patients had novel syndromes. One FMF patient had a TRNT1 gene mutation who responded to intravenous immunoglobulin (IVIg) and colchicine after failing multiple treatments. The other had SoJIA with a LPIN 2 gene mutation but responded to colchicine. Only one of the 15 genetically proven cases had classical presentation and genetics (HIDS secondary to a mevalonate kinase (MVK) gene mutation). Conclusion: PFS screening was helpful in over half of the cases to develop therapeutic treatment plans. Given the atypical clinical presentations seen with genetically determined PFS, extensive genetic testing is indicated for all patients presenting with a PFS, excluding classical PFAPA syndrome.
Arthritis Care & ResearchVolume 71, Issue 4 p. 578-578 Letters Differences in Management Style Between Pediatric and Adult Rheumatologists: Comment on the Article by Van Mater et al Daniel Albert, Daniel Albert orcid.org/0000-0002-6269-8294 Dartmouth Hitchcock Medical Center, Lebanon, NHSearch for more papers by this author Daniel Albert, Daniel Albert orcid.org/0000-0002-6269-8294 Dartmouth Hitchcock Medical Center, Lebanon, NHSearch for more papers by this author First published: 26 February 2018 https://doi.org/10.1002/acr.23552Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat No abstract is available for this article. Volume71, Issue4April 2019Pages 578-578 RelatedInformation
Objective: Few controlled studies are available to guide treatment decisions in juvenile dermatomyositis (JDM). This study evaluated therapies received, changes of treatment over time, and factors associated with medication choices in JDM. Methods: We performed a retrospective analysis of the number and type of therapies and duration of treatment for 320 patients with JDM enrolled in a North American registry. Kaplan-Meier and logistic regression analysis were used to assess the association of demographic and clinical features and autoantibodies with medication usage. Results: High-dose oral prednisone was the primary therapy given to 99% of patients. 1997 was determined to be a threshold year for increasing usage of medications other than prednisone. The median time to half the initial oral prednisone dose was shorter in patients diagnosed after vs. before 1997 (10 vs. 19 months, P < 0.01). Patients received intravenous methylprednisolone (IVMP), methotrexate, intravenous immunoglobulin, antimalarial drugs, and combination therapy more frequently when diagnosed after 1997. IVMP was frequently received by patients with severe illness onset, anti-p155/140 (anti-TIF1) and anti-MJ (anti-NXP2) autoantibodies. Treatment with methotrexate was associated with older age at diagnosis and anti-MJ autoantibodies, while antimalarial therapy was associated with anti-p155/140 autoantibodies and mild onset severity. Conclusion: Oral prednisone has been the mainstay of therapy in JDM, and prednisone was reduced faster in patients diagnosed after 1997 when there was also an increase in other medications. Specific medications received by patients with JDM correlated with year and age at diagnosis, myositis autoantibodies, onset severity, and illness features. Published by Elsevier Inc.