BackgroundEosinophilic fasciitis (EF), a fibrotic disease that causes inflammatory infiltration in the subcutaneous fascia is clinically characterized by edema and subsequent induration and tightening of the skin and subcutaneous tissue. EF is a rare immune-related adverse event of immune checkpoint inhibitors (ICI).ObjectivesThis article aims to investigate the clinical features of ICI-related EF and to improve the understanding of the disease among rheumatologists.MethodsSearching relevant articles in the electronic databases Medline, PubMed, Science Citation Index, China Biomedical Literature Database (CBM), China Journal Full Text Database (CNKI), and WANFANG Data with the key words of “eosinophilic fasciitis” or “Shulman syndrome” and “checkpoint inhibitor”, “CTLA-4”, “PD-1” or “PD-L1”. Only articles or case reports with detailed medical records of ICI-related EF patients were included. ICI-related EF patients in our department were also included.ResultsA 58-year-old male patient with angioimmunoblastic T cell lymphoma presented with skin edema, subsequent induration, tightening of the skin, and subcutaneous tissue (Figure 1 A&B). The eosinophils elevated in peripheral blood after 6-month treatment of PD-1 inhibitor (Camrelizumab). The patient was diagnosed as ICI-related EF and then was referred to our rheumatology department. Other seventeen EF patients from the above databases were also included for analysis. Among these 18 patients, 50% (9/18) were male and the mean age was 57±14 years. The most commonly used ICI was PD-1 inhibitor such as nivolumab and pembrolizumab, accounting for 72% (13/18), next was PD-L1 inhibitor (17%, 3/18), and 3 patients used PD-L1 inhibitor combined with CTLA-4 inhibitor. Of all cases, 50% (9/18) was metastatic melanoma and 17% (3/18) was lung cancer. After ICI treatment, the median onset time of EF was 12 months. The most common involved organ was skin and 94% (17/18) of patients presenting with symmetrical skin edema and sclerosis. For other skin manifestations, 44% (8/18) of patients had typical “groove sign”, and one case showed unilateral skin involvement of skin tension and erythema from pubis to left anterior iliac crest. When the affected limb raised, there is a visible sulcus due to the decrease of venous dilation pressure, namely “sulcus sign”. The second common involved organ was the joints which were presented with limited function in 56% (10/18) of patients. Additionally, 39% (7/18) showed muscle involvement such as myalgia and myasthenia. Increased eosinophils in peripheral blood was observed in 72% (13/18) of patients. Twelve patients (63%) received MRI examinations which showed the signal in the subcutaneous and deep fascia increased in both fat-suppressed T2 images(Figure 1 C&E) and post-enhancement T1 images(Figure 1 D&F). Fifteen patients underwent full-thickness skin biopsy at the lesion site, the pathological changes in all patients supported the diagnosis of EF. The ICI was discontinued in 94% of patients. Meanwhile, 83% received glucocorticoids and 56% of patients were treated with methotrexate. After these treatment, the clinical symptoms of EF improved in 89% of patients (n=16), while eosinophils returned to normal after a median treatment time of 2.5 months. EF progressed even through the combined treatment of prednisone, MTX, and abatacept in one patient. Another one case died after 6 months due to metastasis of bladder cancer (stageIV).ConclusionAlthough EF is a rare adverse effect of ICI treatment, individuals receiving these drugs should be monitored closely for symptoms of EF. Discontinuation of ICI and administration of immunosuppressants may prevent the progression of EF.Disclosure of InterestsNone declared
BackgroundSalivary gland ultrasonography (SGUS) is promising on diagnosis and disease monitoring of primary Sjögren’s syndrome (pSS). However, there is neither study concerning the association between SGUS and salivary function in pSS.ObjectivesThis study aimed to explore how SGUS features reflected the secretory function of salivary glands in pSS.MethodsThe subjects who presented with ocular dryness and/or oral dryness were recruited from rom July 2021 to July 2022 at our department. The subjects were classified as pSS and non-SS according to the 2002 the American-European Consensus Group (AECG) criteria for pSS. Unstimulated whole salivary flow (UWSF) and stimulated whole salivary flow (SWSF) were assessed to evaluate salivary function. SGUS was performed to bilateral parotid and submandibular glands. Each gland was assessed by a well-accepted 0–4 scoring system (gland score) and a 0-3 scoring system from the Outcome Measures in Rheumatology Clinical Trials working group (OMERACT score).Results① Among 309 enrolled subjects with suspected pSS, 95 subjects were excluded due to being classified as secondary SS, or failing to receive the designated SGUS examination or labial salivary glands biopsy. Therefore, 214 subjects were qualified for statistical analysis, including 116 pSS patients. The mean age of pSS patients was 47.1±13.3 years old and 97% of them were female, age-match and sex-match to the non-SS controls.② Compared to the pSS patients without hypofunction, pSS patients with salivary hypofunction (n=71, SWSF ≤0.7mL/min) were significantly older, higher positive rate of the Van Bijsterveld score, higher positive rate of anti-SSA/Ro, higher incidence of UWSF≤0.1mL/min and lower incidence of thrombocytopenia (allp<0.05).③ The pSS patients with salivary hypofunction had significantly higher total OMERACT score (10 [interquartile range (IQR), 8-12] vs. 8 [IQR,6-8],p<0.001), and total gland score (12 [IQR,10-12] vs. 8 [IQR,6-11],p<0.001) than the patients without salivary hypofunction (Figure 1A). ROC curve confirmed total OMERACT score (AUC: 0.804, 95%CI: 0.727-0.881,p<0.001) and total gland score (AUC: 0.792, 95%CI: 0.704-0.881,p<0.001) could significantly distinguish pSS patients with salivary hypofunction from those without salivary hypofunction. The cut-off values of total OMERACT score were 7 and 9, and of total gland score were 9 and 11, according to the Youden index. The absolute value of SWSF in the pSS patients with total gland score >11 was significantly lower than those with total gland score of 9~11 or total gland score <9 (bothp<0.05). The absolute value of SWSF in the pSS patients with total OMERACT score >9 was significantly lower than those with total gland score of 7~9 or total gland score <7 (both p<0.001, Figure 1B).④ Total OMERACT score and total gland score were combined to generate a 3×3 matrix model and then the estimated risk probability for salivary hypofunction of pSS was calculated in each grid (Figure 1C). Accordingly, pSS patients could be stratified into three risk subgroups: (i) High-risk (n=39): pSS patients whose total OMERACT score was >9 and total gland score ≥9 had estimated risk probability greater than 80%, and the actual incidence of SWSF≤0.7mL/min was 95% (37/39). (ii) Low-risk (n=28): pSS patients whose total OMERACT score was <7; or whose total OMERACT score was 7~9 and total gland score <9 had estimated risk probability less than 35%, and the actual incidence of SWSF≤0.7mL/min was 14% (4/28). (iii) Moderate-risk (n=49): pSS patients who fulfilled neither high-risk nor low-risk had an actual incidence of SWSF≤0.7mL/min in a moderate level (61%, 30/49).ConclusionThis study first reported B-mode ultrasonography examination on four major salivary glands could reflect salivary function of pSS. We also built a matrix risk model composed of SGSU gland score and OMERACT score associated with salivary hypofunction in pSS which was conviently used for rheumatologists, especially in specific clinical situations that SWSF was not available.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of InterestsNone Declared.
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