Objective:To evaluate the radiological features of IgG4-related disease(IgG4-RD)in the head and neck region.Methods:In this radiology-based study,radiological features,clinical,laboratory,pathological findings,and pro...Objective:To evaluate the radiological features of IgG4-related disease(IgG4-RD)in the head and neck region.Methods:In this radiology-based study,radiological features,clinical,laboratory,pathological findings,and prognosis of nine patients with head and neck involvement diagnosed with IgG4-RD were investigated retrospectively.Results:The median age of the patients was 38 years(range:2.5-79 years),and there were six males and three females.The most common symptoms and clinical findings of the patients were eyelid and lacrimal gland swelling,painless exophthalmos,and ophthalmoplegia.The most common site of involvement on MRI was the orbit.Orbital involvement was followed by branches of the trigeminal nerve,sinonasal cavity,cervical lymph nodes,and dural involvement.The most common and remarkable imaging features were T2 hypointensity and diffuse homogeneous contrast enhancement.Conclusions:Head and neck involvement of the IgG4-RD,has specific imaging features that can help with diagnosis.Thus,early diagnosis and better outcomes can be achieved with increasing awareness of these features of this relatively new pathology.展开更多
The pathogenesis of rheumatoid arthritis(RA) remains to be completely elucidated so far; however, it is known that proinflammatory cytokines play a pivotal role in the induction of RA. Tumor necrosis factor(TNF-α), i...The pathogenesis of rheumatoid arthritis(RA) remains to be completely elucidated so far; however, it is known that proinflammatory cytokines play a pivotal role in the induction of RA. Tumor necrosis factor(TNF-α), in particular, is considered to play a central role in bone destruction by mediating the abnormal activation of osteoclasts or the production of proteolytic enzymes through direct or indirect mechanisms. The use of TNF-α blocking agents has a significant impact on RA therapy. Anti-TNF-α blocking agents such as infliximab are very effective for treatment of RA, especially for the prevention of articular destruction. We have previously shown that several proteins exhibited extensive changes in their expression after amelioration of RA with infliximab treatment. Among the proteins, connective tissue growth factor(CTGF) has a significantrole for the development of RA. Herein, we review the function of CTGF in the pathogenesis of RA and discuss the possibility of a novel treatment for RA. We propose that CTGF is a potentially novel effector molecule in the pathogenesis of RA. Blocking the CTGF pathways by biological agents may have great beneficial effect in patients with RA.展开更多
A lupus patient with a clinically quiescent disease stage will be described who had severely depressed C4 levels while levels of C3 en CH50 were normal. Additional testing revealed a homozygous C4Aisotype deficiency a...A lupus patient with a clinically quiescent disease stage will be described who had severely depressed C4 levels while levels of C3 en CH50 were normal. Additional testing revealed a homozygous C4Aisotype deficiency as the cause of the very low C4 levels. It should be emphasized that in SLE, a (very) low C4 level does not always means (subclinical) disease activity.展开更多
Thyroid associated ophthalmopathy is an autoimmune disorder which involves orbital and periorbital tissue. The immune-mediated inflammation of the orbital tissues can involve extraocular muscles, orbital connective ti...Thyroid associated ophthalmopathy is an autoimmune disorder which involves orbital and periorbital tissue. The immune-mediated inflammation of the orbital tissues can involve extraocular muscles, orbital connective tissue or orbital fat and periocular soft tissues. Bilateral involvement of thyroid associated orbitopathy is usually asymmetric, but unilateral thyroid associated orbitopathy has been less reported. Periorbital oedema as the only sign with hypothyroidism is uncommon and if present, it is more frequent bilaterally present and no cases are evidenced as unilateral. Pitting oedema in hypothyroidism is rare and can be due to increased capillary permeability, decreased adrenergic tone and increase in serotonin metabolism. Unilateral periorbital and eyelid oedema can associate with various clinical entities, multidisciplinary team is necessary to exclude the concomitant disease, so the patient can immediately be treated with proper therapy. We represent the case of unusually unilateral recurrent periorbital oedema in the period of time for 3 years with stabilized primary hypothyroidism and multinodular goitre.展开更多
Sjogren Syndrome (SS) is an autoimmune disease characterized by a lymphocytic infiltration of exocrine glands. This abnormality leads to the destruction of these glands with the clinical expression of the sicca syndro...Sjogren Syndrome (SS) is an autoimmune disease characterized by a lymphocytic infiltration of exocrine glands. This abnormality leads to the destruction of these glands with the clinical expression of the sicca syndrome (dry eyes and dry mouth). Usually Sjogren Syndrome follows a non-aggressive course, but patients suffering from this disease have a very high risk of developing malignant lymphoma (MALT). The evolution from benign lymphocytic infiltration characteristic of Sjogren Syndrome to malignant lymphoma (usually MALT subtype) is a multistep process [1-3]. We present the case of 35 years old woman admitted to “Sf. Maria” Clinical Hospital—Bucharest, Romania, in the Rheumatology Department in May 2011, representative for the diagnosis difficulties as well as for the unfortunate evolution. She was diagnosed with primary Sjogren Syndrome in 2004. The first symptoms of the disease, dry eyes and dry mouth, appeared in 2000 but they were undermined by some hematological disorders, as the patient had a low white blood cell count at the biological evaluation. Four years later, when the diagnosis of Sjogren Syndrome was set, she already had some negative prognosis factors. In the end, the disease had an unfavorable outcome, as the patient developed non-Hodgkin malignant lymphoma for an unfortunate evolution.展开更多
Parasellar extra-axial cavemomas are rare lesions.The authors report a case of extra-axial cavemoma in a 50-year-old male patient,who presented with occipital headache and double vision.The magnetic resonance imaging ...Parasellar extra-axial cavemomas are rare lesions.The authors report a case of extra-axial cavemoma in a 50-year-old male patient,who presented with occipital headache and double vision.The magnetic resonance imaging showed an enhancing extra-axial dural-based mass in the left parasellar region invading cavernous sinus,hyper-intense on T2-weighted images,iso-intense on Tl-weighted images and high relative cerebral blood velocity on magnetic resonance perfusion.The patient underwent a left pterional craniotomy and parasellar space occupying lesion was excised.Histopathology was suggestive of cavernous hemangioma.展开更多
文摘Objective:To evaluate the radiological features of IgG4-related disease(IgG4-RD)in the head and neck region.Methods:In this radiology-based study,radiological features,clinical,laboratory,pathological findings,and prognosis of nine patients with head and neck involvement diagnosed with IgG4-RD were investigated retrospectively.Results:The median age of the patients was 38 years(range:2.5-79 years),and there were six males and three females.The most common symptoms and clinical findings of the patients were eyelid and lacrimal gland swelling,painless exophthalmos,and ophthalmoplegia.The most common site of involvement on MRI was the orbit.Orbital involvement was followed by branches of the trigeminal nerve,sinonasal cavity,cervical lymph nodes,and dural involvement.The most common and remarkable imaging features were T2 hypointensity and diffuse homogeneous contrast enhancement.Conclusions:Head and neck involvement of the IgG4-RD,has specific imaging features that can help with diagnosis.Thus,early diagnosis and better outcomes can be achieved with increasing awareness of these features of this relatively new pathology.
基金Supported by Grant-in-Aid for Scientific Research(C),The Ministry of Education,Culuture,Sports,Science and Technology and The Institute for Environment and Gender-specific Medicine,Juntendo University Graduate School of Medicine
文摘The pathogenesis of rheumatoid arthritis(RA) remains to be completely elucidated so far; however, it is known that proinflammatory cytokines play a pivotal role in the induction of RA. Tumor necrosis factor(TNF-α), in particular, is considered to play a central role in bone destruction by mediating the abnormal activation of osteoclasts or the production of proteolytic enzymes through direct or indirect mechanisms. The use of TNF-α blocking agents has a significant impact on RA therapy. Anti-TNF-α blocking agents such as infliximab are very effective for treatment of RA, especially for the prevention of articular destruction. We have previously shown that several proteins exhibited extensive changes in their expression after amelioration of RA with infliximab treatment. Among the proteins, connective tissue growth factor(CTGF) has a significantrole for the development of RA. Herein, we review the function of CTGF in the pathogenesis of RA and discuss the possibility of a novel treatment for RA. We propose that CTGF is a potentially novel effector molecule in the pathogenesis of RA. Blocking the CTGF pathways by biological agents may have great beneficial effect in patients with RA.
文摘A lupus patient with a clinically quiescent disease stage will be described who had severely depressed C4 levels while levels of C3 en CH50 were normal. Additional testing revealed a homozygous C4Aisotype deficiency as the cause of the very low C4 levels. It should be emphasized that in SLE, a (very) low C4 level does not always means (subclinical) disease activity.
文摘Thyroid associated ophthalmopathy is an autoimmune disorder which involves orbital and periorbital tissue. The immune-mediated inflammation of the orbital tissues can involve extraocular muscles, orbital connective tissue or orbital fat and periocular soft tissues. Bilateral involvement of thyroid associated orbitopathy is usually asymmetric, but unilateral thyroid associated orbitopathy has been less reported. Periorbital oedema as the only sign with hypothyroidism is uncommon and if present, it is more frequent bilaterally present and no cases are evidenced as unilateral. Pitting oedema in hypothyroidism is rare and can be due to increased capillary permeability, decreased adrenergic tone and increase in serotonin metabolism. Unilateral periorbital and eyelid oedema can associate with various clinical entities, multidisciplinary team is necessary to exclude the concomitant disease, so the patient can immediately be treated with proper therapy. We represent the case of unusually unilateral recurrent periorbital oedema in the period of time for 3 years with stabilized primary hypothyroidism and multinodular goitre.
文摘Sjogren Syndrome (SS) is an autoimmune disease characterized by a lymphocytic infiltration of exocrine glands. This abnormality leads to the destruction of these glands with the clinical expression of the sicca syndrome (dry eyes and dry mouth). Usually Sjogren Syndrome follows a non-aggressive course, but patients suffering from this disease have a very high risk of developing malignant lymphoma (MALT). The evolution from benign lymphocytic infiltration characteristic of Sjogren Syndrome to malignant lymphoma (usually MALT subtype) is a multistep process [1-3]. We present the case of 35 years old woman admitted to “Sf. Maria” Clinical Hospital—Bucharest, Romania, in the Rheumatology Department in May 2011, representative for the diagnosis difficulties as well as for the unfortunate evolution. She was diagnosed with primary Sjogren Syndrome in 2004. The first symptoms of the disease, dry eyes and dry mouth, appeared in 2000 but they were undermined by some hematological disorders, as the patient had a low white blood cell count at the biological evaluation. Four years later, when the diagnosis of Sjogren Syndrome was set, she already had some negative prognosis factors. In the end, the disease had an unfavorable outcome, as the patient developed non-Hodgkin malignant lymphoma for an unfortunate evolution.
文摘Parasellar extra-axial cavemomas are rare lesions.The authors report a case of extra-axial cavemoma in a 50-year-old male patient,who presented with occipital headache and double vision.The magnetic resonance imaging showed an enhancing extra-axial dural-based mass in the left parasellar region invading cavernous sinus,hyper-intense on T2-weighted images,iso-intense on Tl-weighted images and high relative cerebral blood velocity on magnetic resonance perfusion.The patient underwent a left pterional craniotomy and parasellar space occupying lesion was excised.Histopathology was suggestive of cavernous hemangioma.
