Undifferentiated Connective Tissue Diseases

COPENHAGEN, Denmark, June 2, 2022 /PRNewswire/ -- A new study by researchers at Hospital for Special Surgery (HSS) has further clarified the clinical manifestations of undifferentiated connective tissue disease (UCTD). The findings were published as an abstract in the meeting journal of the European Alliance of Associations for Rheumatology (EULAR) 2022 Congress. "We have been interested in studying UCTD because it's commonly encountered in clinical practice but not well characterized," said lead study author Caroline Siegel, MD, a rheumatology fellow at HSS. "UCTD is inherently challenging to define, but it's very important to understand what features differentiate patients with UCTD from those with defined connective tissue diseases (CTD) such as lupus so that we can better manage patients with this condition and ultimately predict who may go on to develop more severe clinical manifestations." The study included 89 patients aged 18 years and older treated at HSS between 2018 and 2022 who had rheumatologist-diagnosed UCTD with positive antinuclear antibody and at least one sign or symptom of CTD. The researchers reviewed medical records to identify those who fulfilled established classification criteria for systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), systemic sclerosis, primary Sjögren's, idiopathic inflammatory myopathy, or antiphospholipid syndrome (APS). The researchers compared sociodemographic, clinical, serologic, and treatment variables between 59 patients with UCTD and 30 patients who fulfilled criteria for CTD. Patients in both groups had similar non-criteria manifestations, most commonly joint pain and fatigue. However, patients with UCTD were less likely to have arthritis, criteria-based hematologic manifestations, anti-double stranded DNA or anti-Smith antibodies, and hypocomplementemia. The frequency of RA, Sjögren's, and APS-related serologies did not differ between groups. Compared to those with CTD, UCTD patients were less likely to have ever received systemic corticosteroids. "Our main findings were that patients who met a strict definition of UCTD versus those who met classification criteria for other CTDs were less likely to have clinical and laboratory features such as arthritis and hematologic abnormalities, as well as certain autoantibodies that are commonly seen in lupus," said Dr. Siegel. "They were also less likely to have received treatment with steroids," she added. The results reveal that even among patients diagnosed by their treating physician with UCTD, some may meet classification criteria for other rheumatic diseases. Within this group, patients with strictly defined UCTD may differ in meaningful ways. "We are starting to recognize features of strictly defined UCTD that will allow us to better identify and manage this condition," said senior study author Medha Barbhaiya MD, MPH, rheumatologist at HSS. "Patients struggle with the uncertainty of a UCTD diagnosis and our study aimed to demonstrate that UCTD may be a distinct entity from other rheumatic conditions. These findings lend support to the claim that patients who have UCTD require separate study, and their disease manifestations warrant further understanding." About HSS HSS is the world's leading academic medical center focused on musculoskeletal health. At its core is Hospital for Special Surgery, nationally ranked No. 1 in orthopedics (for the 12th consecutive year), No. 4 in rheumatology by U.S. News & World Report (2021-2022), and the best pediatric orthopedic hospital in NY, NJ and CT by U.S. News & World Report "Best Children's Hospitals" list (2021-2022). In a survey of medical professionals in more than 20 countries by Newsweek, HSS is ranked world #1 in orthopedics for a second consecutive year (2022). Founded in 1863, the Hospital has the lowest complication and readmission rates in the nation for orthopedics, and among the lowest infection rates. HSS was the first in New York State to receive Magnet Recognition for Excellence in Nursing Service from the American Nurses Credentialing Center five consecutive times. An affiliate of Weill Cornell Medical College, HSS has a main campus in New York City and facilities in New Jersey, Connecticut and in the Long Island and Westchester County regions of New York State, as well as in Florida. In addition to patient care, HSS leads the field in research, innovation and education. The HSS Research Institute comprises 20 laboratories and 300 staff members focused on leading the advancement of musculoskeletal health through prevention of degeneration, tissue repair and tissue regeneration. The HSS Innovation Institute works to realize the potential of new drugs, therapeutics and devices. The HSS Education Institute is a trusted leader in advancing musculoskeletal knowledge and research for physicians, nurses, allied health professionals, academic trainees, and consumers in more than 145 countries. The institution is collaborating with medical centers and other organizations to advance the quality and value of musculoskeletal care and to make world-class HSS care more widely accessible nationally and internationally. .

A small study by researchers in Israel published in the journal Rheumatology identified six cases of patients developing herpes zoster rashes (shingles) after receiving the Pfizer-BioNTech vaccine. A small study by researchers in Israel published in the journal Rheumatology identified six cases of patients developing herpes zoster rashes (shingles) after receiving the Pfizer-BioNTech vaccine. The study found six cases out of 491 people, or 1.2%. A total of six patients had mild cases of autoimmune inflammatory rheumatic diseases. All were under the age of 50. “That is why we reported on it,” Victoria Furer, lead author, from Tel Aviv Sourasky Medical Center and Carmel Medical Center in Haifa, Israel, told the Jerusalem Post . Several media outlets, including Fox News, have inferred with their headlines that herpes infections and shingles infections may be linked to the Pfizer-BioNTech COVID-19 vaccine. This is a gross and misleading description of the study. Shingles is a severe illness caused by the herpes zoster virus that affects a nerve in the dermatome, under the skin, accompanied by painful blisters and sores. It can last anywhere from days to months. Although some pain drugs and antivirals can help, it usually just runs its course. It can also return. The study evaluated the safety of the mRNA-based COVID-19 vaccines in patients with autoimmune inflammatory rheumatic diseases (AIIRD). All of the six cases were women with stable AIIRD, either rheumatoid arthritis, Sjogren’s syndrome, and in one, an undifferentiated connective disease. All developed the herpes zoster rash for the first time after the first vaccine in five cases and after the second shot in one case. Five of the six cases were mild except one case of HZ ophthalmicus, without corneal involvement, in an RA patient treated with tofacitinib. There were no cases of more serious disseminated HZ disease or postherpetic neuralgia. It’s important to note that this herpes is not associated with oral and genital herpes infections observed in sexually transmitted diseases. Herpesvirus is a DNA virus that includes herpes simplex virus 1 (HSV1, common cold sores) and herpes simplex virus 2 (HSV2, which tend to flare up around the genital in times of stress). Chickenpox is also caused by a herpesvirus, varicella zoster virus. This infection is quite rare now because of a very effective varicella vaccine, typically given in combination with the measles, mumps, and rubella (MMR) vaccine as MMRV. One of the oddities of the chickenpox herpesvirus is that if you had chickenpox, the virus can stay dormant in your nerves and then become a full-blown shingles outbreak during adulthood, typically during or after periods of stress. There is an effective vaccine against shingles, but it generally is not given until you are in your 50s. In immune suppressed individuals who have the herpes zoster virus dormant in their nerves, it can re-emerge if they have a weakened immune system from illness, immunosuppressant mediations like high-dose steroids or chemotherapy. It can also be triggered by severe illness, emotional stress, and even the stress caused by a vaccine of any kind , resulting in shingles. In the case of these patients, some people can’t receive the shingles vaccine because of their immune status, or, if they have received the shingles vaccine, have weakened immune systems, which makes them susceptible to shingles even with the vaccine. Vaccines are designed to train the immune system to respond to a virus. However, if the immune system is weak—such as is the case with the women in the study—it may not respond well. In the study, all six women were on immunosuppressant drugs because of their autoimmune disorders and had not been vaccinated for chickenpox or shingles. Nina Shapiro , Director of Pediatric Otolaryngology and Professor of Head and Neck Surgery at the David Geffen School of Medicine at UCLA, wrote her response to an article made by the New York Post, which may have created some undue fear that the COVID-19 vaccine can cause herpes infections. This was not the study authors’ intent, nor was it the result of the study. The authors state: ‘The study design is not structured to determine a causal relationship between [COVID-19] vaccination and HZ [herpes zoster].”