P Aparna, Ingle Vaibhav, Singhai Abhishek, Khadanga Sagar, Joshi Rajnish, Saigal Saurabh, Kotnis Ashwin
Internal Medicine, All India Institute of Medical Sciences, Bhopal, Bhopal, IND.
Anaesthesiology, All India Institute of Medical Sciences, Bhopal, Bhopal, IND.
Cureus. 2023 Mar 8;15(3):e35916. doi: 10.7759/cureus.35916. eCollection 2023 Mar.
Autoimmune rheumatic diseases (ARD) present unique challenges in clinical practice. Many of them present in medical emergencies in an unstable state and need immediate evaluation for further plans of action. The clinical conundrum is to distinguish between sepsis, disease flare, or Addisonian crisis (AC) (secondary to steroid withdrawal). This may be further complicated by overlapping clinical features like shock/fever and the coexistence of a combination of the above pathophysiologic mechanisms (e.g. AC with sepsis or AC with disease flare). The known biomarkers may not perform optimally to distinguish them and additional supportive investigations like imaging, cultures, autoimmune serological markers, etc. are needed. Ultimately the boundaries between "the art of medicine" and "the science of medicine" may get blurred, as the established literature evidence falls short and the expert opinion is needed in a time-sensitive manner. In this pragmatic study, researchers have attempted to explore the presentation of rheumatologic emergencies on the above three differentials (sepsis, disease flare, and AC).
In this hospital-based cross-sectional study, adult patients (age >18 years) with ARD who had unplanned hospital admission due to acute worsening were enrolled. This study was conducted over one year, after getting the Institutional Human Ethics Committee's approval. All relevant hematological, immunological, and hormonal parameters (specifically morning cortisol) were collected and analyzed. The aim was to find the individual and combined prevalence of sepsis, disease flare, or AC in this study group.
Forty-one patients were analyzed, with females in the majority (95%) and the dominant age group being 26-49 years (56.1%). A majority had a diagnosis of rheumatoid arthritis (RA) (56.1%) or systemic lupus erythematosus (SLE) (31.7%); the rest were other connective tissue diseases (12.2%). High-risk Quick Sequential Organ Failure Assessment score (qSOFA) score 2-3 was present in 29.3% while the rest had low-risk scores (qSOFA score 0-1). Thirty-two percent had severe disease activity, 46% had mild to moderate disease activity, and 22% of patients had no disease activity. While 78% of patients had low procalcitonin (PCT) values <0.5 microgm/L (low risk of sepsis), 15% had <20 microgm/L, and 7% percentage of patients had serum levels >20 microgm/L (high risk of sepsis). A total of 73.2% of patients had no evidence of infection while 26.8% had either microbiological/radiological evidence of infection. Only 7% of all patients had the presence of an AC. qSOFA scores didn't statistically correlate with a diagnosis of infection or AC but positively correlated with PCT and C-reactive protein (CRP) values. Serum PCT didn't correlate with the presence of infection with statistically significance (p-value 0.217).
Infections and sepsis are the most important considerations in the emergency presentations of ARDs. Disease flare and AC are also important differentials. Current inflammatory biomarkers like serum CRP and PCT may be less valuable for discriminating between infectious and non-infectious sepsis, especially in chronic inflammatory diseases like ARDs. qSOFA scores may have a prognostic role with less discriminant value. Management of ARD emergencies needs better biomarkers and more research is warranted.
自身免疫性风湿性疾病（ARD）在临床实践中带来了独特的挑战。其中许多疾病在医疗紧急情况下以不稳定状态出现，需要立即进行评估以制定进一步的行动计划。临床难题在于区分脓毒症、疾病发作或艾迪生病危象（AC）（继发于类固醇撤药）。休克/发热等重叠的临床特征以及上述病理生理机制的组合并存（如AC合并脓毒症或AC合并疾病发作）可能会使情况更加复杂。已知的生物标志物可能无法很好地区分它们，因此需要额外的支持性检查，如图像检查、培养、自身免疫血清学标志物等。最终，由于现有文献证据不足且需要在时间紧迫的情况下获取专家意见，“医学艺术”与“医学科学”之间的界限可能会变得模糊。在这项务实的研究中，研究人员试图探讨上述三种不同情况（脓毒症、疾病发作和AC）下风湿性急症的表现。
在这项基于医院的横断面研究中，纳入了因急性病情恶化而意外入院的成年ARD患者（年龄>18岁）。在获得机构人类伦理委员会批准后，这项研究进行了一年。收集并分析了所有相关的血液学、免疫学和激素参数（特别是清晨皮质醇）。目的是找出该研究组中脓毒症、疾病发作或AC的个体及合并患病率。
共分析了41例患者，其中女性占大多数（95%），主要年龄组为26 - 49岁（56.1%）。大多数患者诊断为类风湿关节炎（RA）（56.1%）或系统性红斑狼疮（SLE）（31.7%）；其余为其他结缔组织疾病（12.2%）。29.3%的患者快速序贯器官衰竭评估（qSOFA）高危评分2 - 3分，其余患者为低危评分（qSOFA评分0 - 1分）。32%的患者疾病活动严重，46%的患者疾病活动为轻度至中度，22%的患者无疾病活动。78%的患者降钙素原（PCT）值<0.5μg/L（脓毒症低风险），15%的患者<20μg/L，7%的患者血清水平>20μg/L（脓毒症高风险）。73.2%的患者没有感染证据，26.8%的患者有微生物学/影像学感染证据。所有患者中只有7%存在AC。qSOFA评分与感染或AC的诊断无统计学相关性，但与PCT和C反应蛋白（CRP）值呈正相关。血清PCT与感染的存在无统计学相关性（p值0.217）。
感染和脓毒症是ARD急症表现中最重要的考虑因素。疾病发作和AC也是重要的鉴别诊断。目前的炎症生物标志物如血清CRP和PCT在区分感染性和非感染性脓毒症方面可能价值较小，尤其是在ARD等慢性炎症性疾病中。qSOFA评分可能具有预后作用，但鉴别价值较小。ARD急症的管理需要更好的生物标志物，有必要进行更多研究。
