Clinical and serological characteristics of anti-Ro/SS-A and anti-La/SS-B negative primary Sjögren's syndrome: a comparative study.

OBJECTIVE: This study aimed to describe the clinical spectrum of primary Sjögren's syndrome (pSS) patients with anti-Ro/SS-A and anti-La/SS-B negativity. PATIENTS AND METHODS: From a single-center study population of consecutive SS patients fulfilling the 2016 ACR-EULAR classification criteria, those with triple seronegativity anti-Ro/SS-A (anti-Sjögren's-syndrome-related antigen A autoantibody), anti-La/SS-B (anti-Sjögren's-syndrome-related antigen B autoantibody), rheumatoid factor (RF) (-) and antinuclear antibody (ANA)(+)] or [anti-Ro/SS-A(-), anti-La/ SS-B(-), RF(+) and ANA(-)] and quad¬ruple seronegativity [anti-Ro/SS-A(-), anti-La/SS-B(-), RF(-) and ANA(-)] were identified retrospectively. Clinical, serological, and laboratory features were compared. A comparison between triple and quadruple seronegative pSS patients was also performed. RESULTS: We included 184 patients (168 women, 16 men) with a mean age at diagnosis of 50.1±13.1 years. The most common subjective presenting features at the time of the diagnosis were dry mouth (94.5%) and dry eye (91.3 %). ANA positivity was 57.0%, and RF positivity was 30.4%. Salivary gland enlargement, arthritis, Raynaud's phenomenon, vasculitis, interstitial lung disease (ILD), neurological involvement, primary biliary cholangitis (PBC), lymphopenia, and thrombocytopenia were observed in ANA+ and RF+ patients but not in seronegative patients (p<0.0001). Arthritis was observed most frequently in RF-positive patients and secondly in ANA-positive patients, whereas arthritis was not observed in seronegative patients (p<0.0001). Autoimmune thyroiditis was present in 65 patients (35.0%), 84.6% of these patients were ANA positive while 12.3% were ANA negative (p=0.0014), RF positivity was 30.7% while RF negativity was 6.15% (p=0.001), 23.0% were both ANA and RF positive while 12.3% were seronegative (p<0.002). Cryoglobulinemia, renal disease, and lymphoma were not observed in any of the patients. CONCLUSIONS: We confirm the strong influence of immunological markers on the phenotype of primary SS at diagnosis.

Clinical study on single-organ cutaneous small vessels vasculitis: a retrospective observational study.

OBJECTIVE: Single-organ cutaneous small-vessel vasculitis (SoCSVV) is an inflammatory skin-limited vascular disease affecting the dermal and/or hypodermal vessel wall. Pathogenetically, idiopathic forms are described, as well as the induction from different triggers, such as infections, drugs, and vaccines. Following the Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) pandemic outbreak, cases of cutaneous vasculitis induced by both COVID-19 and COVID-19 vaccinations have been reported in literature. The aim of this study is to provide the most recent evidence on new etiological factors, clinical features, and management of the SoCSVV. PATIENTS AND METHODS: We included 42 patients (22 women, 20 men) with SoCSVV and no systemic involvement in the study. The mean age of the patients was 57.3 years. Palpable purpura was the most frequent clinical manifestation (38 cases-90.4%). All patients were diagnosed with leukocytoclastic vasculitis by skin biopsy. RESULTS: The etiological factors were as follows: idiopathic in 9 (21%) patients, drug-related in 19 (45%) patients, COVID-19 infection-related in 5 (12%) patients, post-COVID-19 vaccination in 5 (12%) patients, paraneoplastic in 2 (5%) patients, and drug and infection and sepsis in 1 patient each. Among the drug-related cases, 16 (84%) were antibiotic-related, and most of them were beta-lactam antibiotics. Eosinophilia was present in skin biopsy in the cases related to vaccination and drugs, while intense necrosis and vascular damage in the skin were observed in the cases related to COVID-19 infection, unlike the others. A rapid resolution was observed with the cessation of drugs and short-term steroid treatment for the precipitating factors. CONCLUSIONS: SoCSVV is usually associated with drugs, preceding infections, and vaccines. COVID-19 infection and COVID-19 vaccinations have been reported as new etiological factors. SoCSVV indicates that the disease seems to be a mild, self-limiting illness with a good clinical result.