Search

Background: In 1972, Dr Sharp and colleagues described a new connective tissue disease, characterized by overlapping features of systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and polymyositis/ dermatomyositis (PM/DM) and by the presence of antibodies against the U1 small nuclear ribonucleoprotein autoantigen (U1 snRNP). This condition was termed mixed connective tissue disease (MCTD) and proposed as a distinct disease. Later, after observing the clinical evolution of MCTD patients, Sharp himself agreed that the original concept of MCTD had to be modified and that Internal organs were at risk for serious complications; patients were not always steroid responsive; prognosis was not always benign. Materials and methods: Patients in the age group of 15-50 years diagnosed to have connective tissue diseases were included. 8 patients in the age group of 15-50 admitted in Medicine department were taken and they were evaluated for the clinical profile of sharp syndrome by thorough clinical examination, routine laboratory tests and special investigations depending on the clinical profile. Results: 8 patients with connective tissue disease attending the medicine OPD were studied. Of these 8 patients were female patients. The median age of onset was 36 years, 8 patients met criteria by sharp and Alarcon-Segovia. The clinical features of patients at presentation are Raynaud's phenomena, Puffy fingers, esophagus dysmotility, skin rash, interstitial lung disease, arthritis, pulmonary hypertension, myositis, anemia. Conclusion: SHARP syndrome is a rare condition, as evidenced by the small series of cases reported to date. Diagnosis is based on clinical and paraclinical criteria. The evolution can be interspersed with various complications that can affect the short, medium and long-term prognosis. [ABSTRACT FROM AUTHOR]

Obadah Aqtash,1 Aman Naim Ajmeri,1 Brent A Thornhill,1 Elise Anderson,2 Ryan Carroll,1 Adee Elhamdani,1 Eva Tackett1 1Internal Medicine, Joan C. Edwards School of Medicine, Marshall University, Huntington, WV 25701, USA; 2Charleston Area Medical Center, Charelston, WV 25701, USACorrespondence: Aman Naim AjmeriInternal Medicine, Joan C. Edwards School of Medicine, Marshall University, Huntington, WV 25701, USA, Email ajmeri@marshall.eduAbstract: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a potentially fatal severe adverse reaction to medications. Numerous drugs have been implicated, with carbamazepine and allopurinol being the most common. Tenofovir-induced DRESS is extremely rare. We report a case of a 65-year-old male patient with a diffuse exfoliative maculopapular rash across his entire body of five weeks of duration. The patient also had icteric sclera, abnormal liver enzymes and Raynaud’s of the tongue, nose and the left fifth finger. After discontinuation of tenofovir, the case resolved over a span of ten days. A high index of suspicion is crucial along with the prompt withdrawal of the offending medication for a good outcome.Keywords: DRESS, drug reaction with eosinophilia and systemic symptoms, tenofovir, tenofovir-induced DRESS, Raynaud’s phenomena, Raynaud’s of the tongue

Raynaud's phenomenon is a condition characterized by intermittent vasoconstriction of arteries in the fingers and skin, triggered by cold temperatures or emotional stress, aimed at conserving body heat. This condition is classified into primary and secondary forms, with secondary Raynaud's often linked to connective tissue diseases, medications, infections, and occupational exposures. A notable clinical case involves a 51-year-old male experiencing episodes of painful, white discoloration of the tongue, which were managed through a comprehensive diagnostic process, including rheumatological and cardiological evaluations, to rule out connective tissue diseases and cardiac dysfunction. We highlight the complex pathophysiology of Raynaud's, involving vascular, neurogenic, and immune mechanisms. Management strategies focus on lifestyle modifications and pharmacologic treatments, such as calcium channel blockers, to reduce attack frequency and severity. For refractory cases, advanced therapies, including phosphodiesterase inhibitors, intravenous prostaglandins, and surgical sympathectomy, may be considered. Effective diagnosis and individualized treatment are crucial for preventing complications and improving patient outcomes., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Basharat et al.)

Raynaud's syndrome is a common finding in many autoimmune conditions. Accurately diagnosing Raynaud's, and differentiating it from mimicking conditions, is imperative in rheumatologic diseases. Raynaud's syndrome and Raynaud's mimickers, especially painful Raynaud's mimickers, can prove a diagnostic challenge for the practicing rheumatologist. Painful Raynaud's mimickers can lead to increased patient stress and unnecessary medical work up; Healthcare providers need to be aware of Raynaud's mimickers when evaluating patient concerns of skin color changes and pain. The present narrative review aims to highlight Raynaud's syndrome, important painful mimickers that may be seen, diagnosis, and updated management recommendations., Competing Interests: Declaration of competing interest None of the authors have relevant declarations related to this narrative review., (Copyright © 2024 Elsevier Ltd. All rights reserved.)

Limited cutaneous systemic sclerosis (lcSSc) is a sub-type of systemic sclerosis (SSc) characterized by association of Raynaud's phenomena with skin fibrosis limited to hand, face, feet and forearm. Manifests in females of 40-50 years (female: male - 4:1) with prevalence of 1/12500 adults. Disease originates from idiopathic auto-immune reaction leading to over production of collagen or with the chemical exposure. Prognosis of disease is relatively good with survival of 10 years in 80-90% patients. Complications of Pulmonary arterial hypertension and lung fibrosis have poor prognosis. [ABSTRACT FROM AUTHOR]

Erasmus syndrome is an uncommon disease brought on by exposure to silica and later manifests as systemic sclerosis (SSc) with or without silicosis. The body of literature on Erasmus syndrome is scarce. Here, we report two cases of male patients presenting with SSc after silica exposure. One of the patients had worked in the steel industry, and another had worked in the sculpture manufacturing for a decade before the presentation. It is imperative to raise awareness of this uncommon illness because avoiding further exposure remains the mainstay of management. Our case reports reemphasize the importance of occupational history in all patients of SSc., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Patro et al.)

Objectives: This systematic review seeks to understand whether cold intolerance (CI) improves with time and if there is any role for management strategies such as behavioural therapy, surgery, or pharmacotherapy., Methods: Two independent reviewers used a predefined search strategy to query MEDLINE, PubMed, Embase, CINAHL, Cochrane Library, Web of Science and Google Scholar databases. Articles written in English, Studies of interventions (such as pharmacotherapy or behavioural therapy) for cold intolerance in adult patients with a history of hand injury along with prevalence over time were included for review., Results: Seventeen studies were included, with twelve prognostic studies of the effect of time on CI, four studies of self management/behavioural therapies, and a single study of surgical treatment of neuromas. No studies of pharmacotherapies were identified for inclusion in the hand injury literature. Most studies (76.4%) were either prevalence or prospective cohort studies; no level I or II evidence studies were included., Conclusions: Cold intolerance does not resolve over time for the vast majority of patients. Behavioral and self-management studies have low efficacy and studies presented had a high risk of bias. There is a lack of evidence for the use of pharmacotherapy in CI and this could be considered for future studies., (Copyright © 2023 SFCM. Published by Elsevier Masson SAS. All rights reserved.)

Phacomatosis pigmentovascularis is a rare dermal disorder attributed to the presence of various nevi. These lesions exist since birth, so the patient remains well aware of them. Various systemic involvements may be associated with the nevus, but the association of Raynaud's phenomenon is seldom reported. Our patient came with similar features and, on workup, no neurovascular compression was present, such as cervical rib or thoracic outlet syndrome. Therefore, he was managed conservatively and experienced improvement following the treatment. The objective of reporting this case is to highlight the association of Raynaud's phenomenon with such nervous lesions., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2022, Pipal et al.)