与IgG4相关的疾病涉及骨底部的独特区域:我们的经验
Sharmistha Chakravarty1, Kritika Bhardwaj2
1Department of ENT & HNS , All India Institute of Medical Sciences (AIIMS) Raipurs, Gate No. 1, AIIMS Raipur, Great Eastern Road, Tatibandh CG, Raipur, Chhattisgarh, 492099, India.
概括
与免疫球蛋白G4相关的疾病 (IgG4-RD) 可以以各种方式影响头骨底部. 早期诊断和皮质类固醇治疗是关键,尽管一些耐药病例可能需要免疫抑制剂.
科学领域:
- 神经学 神经学
- 免疫学 免疫学 免疫学
- 耳鼻喉科 耳鼻喉科 耳鼻喉科
背景情况:
- 免疫球蛋白G4相关疾病 (IgG4-RD) 是一种罕见的全身纤维炎症疾病.
- 骨基参与IgG4-RD并不常见,并带来诊断和治疗方面的挑战.
- IgG4-RD的特点是特定的组织病理学发现和血清IgG4水平升高.
研究的目的:
- 介绍三种不同模式的骨基参与IgG4-RD.
- 要突出与骨底部IgG4-RD相关的诊断和治疗挑战.
- 为了说明影响头骨底部的IgG4-RD的不同临床表现和结果.
主要方法:
- 一系列病例描述了三名患有IgG4-RD的患者,影响了不同的头骨底部区域.
- 通过对受影响部位的活检进行组织病理学检查来确认诊断.
- 用皮质类固醇治疗和监测临床反应的治疗.
主要成果:
- 案例1: 轨道顶部和鼻鼻卷入视力损失,用皮质类固醇成功治疗.
- 案例2:耳,骨和喉空间的卷入,表现为耳痛和面部神经麻,用皮质类固醇改善.
- 案例3:鼻和关节的参与导致疾病进展和多器官衰竭,尽管最初的皮质类固醇反应.
结论:
- 头骨底部IgG4-RD呈现出不同的临床表现和结果.
- 通过组织病理学的早期诊断和及时的皮质类固醇治疗对于有利的结果至关重要.
- 耐火性IgG4-RD病例可能需要使用免疫抑制剂.
相关概念视频
01:22Sutures of the Skull
10.2K
The human skull is composed of several bones that come together to protect the brain and support the structures of the face. The junctions where these bones meet are called sutures.
Sutures are immobile joints between adjacent bones of the skull. The narrow gap between the bones is filled with dense, fibrous connective tissue that unites the bones. The long sutures located between the skull bones are not straight but instead follow irregular, tightly twisting paths. These twisting lines tightly...
Sutures are immobile joints between adjacent bones of the skull. The narrow gap between the bones is filled with dense, fibrous connective tissue that unites the bones. The long sutures located between the skull bones are not straight but instead follow irregular, tightly twisting paths. These twisting lines tightly...
10.2K
01:19Cranial and Spinal Meninges
3.6K
The cranial and spinal meninges are complex protective structures surrounding the central nervous system (CNS), consisting of the brain and spinal cord. These meninges consist of the dura mater, the arachnoid mater, and the pia mater. They protect the CNS, provide structural support, and aid in circulating cerebrospinal fluid (CSF).
Cranial Meninges
These meningeal layers cover the cranium. The dura mater is the outermost layer of cranial meninges. It is a thick and durable membrane of dense...
Cranial Meninges
These meningeal layers cover the cranium. The dura mater is the outermost layer of cranial meninges. It is a thick and durable membrane of dense...
3.6K
01:08Overview of the Skull
7.5K
The cranium (skull) is the skeletal structure of the head that supports the face and protects the brain. It is subdivided into the facial bones and the brain case, or cranial vault. The facial bones underlie the facial structures, form the nasal cavity, enclose the eyeballs, and support the teeth of the upper and lower jaws.
The cranial vault surrounds and protects the brain and houses the middle and inner ear structures. This cavity is bounded superiorly by the rounded top of the skull, which...
The cranial vault surrounds and protects the brain and houses the middle and inner ear structures. This cavity is bounded superiorly by the rounded top of the skull, which...
7.5K
01:14Cranial Bones: Superior and Posterior View
4.9K
The superior view of the cranium shows the frontal and paired parietal bones.
The frontal bone is the single bone that forms the forehead. At its anterior midline, between the eyebrows, there is a slight depression called the glabella. The frontal bone also forms the supraorbital margin of the orbit. Near the middle of this margin is the supraorbital foramen, the opening that provides passage for a sensory nerve to the forehead. The frontal bone is thickened just above each supraorbital margin,...
The frontal bone is the single bone that forms the forehead. At its anterior midline, between the eyebrows, there is a slight depression called the glabella. The frontal bone also forms the supraorbital margin of the orbit. Near the middle of this margin is the supraorbital foramen, the opening that provides passage for a sensory nerve to the forehead. The frontal bone is thickened just above each supraorbital margin,...
4.9K
01:27Cranial Bones: Lateral View
4.4K
The lateral view of the cranium is dominated by temporal, sphenoid, and ethmoid bones.
The temporal bone forms the lower lateral side of the skull. The temporal bone is subdivided into several regions. The flattened upper portion is the squamous portion of the temporal bone. Below this area and projecting anteriorly is the zygomatic process of the temporal bone, which forms the posterior portion of the zygomatic arch. Posteriorly is the mastoid portion of the temporal bone. Projecting...
The temporal bone forms the lower lateral side of the skull. The temporal bone is subdivided into several regions. The flattened upper portion is the squamous portion of the temporal bone. Below this area and projecting anteriorly is the zygomatic process of the temporal bone, which forms the posterior portion of the zygomatic arch. Posteriorly is the mastoid portion of the temporal bone. Projecting...
4.4K