Kimura Disease or eosinophilic lymphogranuloma is a benign and rare chronic inflammatory disorder. It mainly affects soft tissues specifically lymph nodes and subcutaneous tissue. It accumulates eosinophils and leads to painless and enlarged mass in the head and neck area. It affects cervical lymph nodes and submandibular. It leads o swelling and cosmetic abnormalities which affect the overall quality of life.
Epidemiology
Kimura disease is a rare disease. The higher rate is seen in areas of Asia like China, Japan, and Southeast Asian country. The prevalence rate is low other than these areas. It occurs in young adults whose age is late teens and early 30s. It is more common in men. The men to women ratio are about 3:1. It is seen in Asian ethnicity specifically in China and Japan.
Anatomy
Pathophysiology
Kimura disease is an abnormal response of immune system. It increases the production of different immune cells like T-helper 2 (Th2) cells, eosinophils, and immunoglobulin E (IgE). The overactive response of Th2 can lead to release of cytokines specifically IL-4 and IL-5. Kimura disease is linked with allergic and atopic conditions. The exact immune response is not known.
There is a proliferation of lymphoid tissue specifically in the lymph nodes and subcutaneous tissue in kimura disease. This can lead to accumulation of the immune cells like eosinophils and lymphocytes with the formation of the lymphoid follicles.
Eosinophils contribute to the immune system response of the body when it comes to the contact with allergens and parasites. These is infiltration of eosinophils in the affected tissue and lead to damage and inflammation to tissue in kimura disease.
Angiogenesis forms a new blood vessels. Kimura disease is caused by an increased synthesis of VEFG. It leads to the formation of vascularized lesion in the affected tissue. Genetic factors can also contribute to Kimura disease. Specific genes which are included in the regulation of immune system and allergic response may have a role in elevating the susceptibility to disease.
Kimura disease is a chronic and relapsing disease with the recurrence of inflammation and symptoms intermediately. The mechanism of he chronicity is not known but it is linked with the dysregulation of immune system and allergic responses.
Etiology
Kimura disease occurs because of the abnormal response of immune system. It leads to chronic inflammation in lymph nodes and soft tissues.
As per some studies, Kimura disease can decrease the allergic response to specific allergens or environmental factor. Viral infections can trigger for Kimura disease. No particular virus is linked with this disease. Genetic predisposition may play role in the development of the Kimura disease. Specific genetic factors can contribute to the susceptibility of the disease. Kimura disease is not an autoimmune disease but some aspects are linked with autoimmune response.
Genetics
Prognostic Factors
Kimura disease can be caused by persistent or recurrent lesions after having the treatment. It can cause disfigurement specifically when the lesion is not treated specifically in neck and head area. Poor diagnosis of kimura disease is associated with the habits of smoking and any history of systemic disease.
Clinical History
Kimura disease can affect the young adult men of Asia specifically from China, Japan and Southeast Asia. The age group is between 20 years to 40 years. It also occurs in children and old adults.
Associated Comorbidity or Activity:
Kimura disease is linked with other allergic diseases like asthma, hay fever, and eczema. It is also linked with nephrotic syndrome caused by the presence of protein in urine, low levels of protein in blood. High levels of cholesterol, and edema.
Kimura disease can occur along with autoimmune diseases like SLE, and rheumatoid arthritis. Patients who have Kimura disease may have changes in the immune system and lead to abnormal immune response.
Physical Examination
Blood Tests:
Complete Blood Count (CBC): It identifies the elevates count of eosinophils which is a main effect of Kimura disease.
Erythrocyte Sedimentation Rate (ESR): An increased ESR can suggest ongoing process of inflammatory response.
IgE levels: Levels of IgE increase in the Kimura disease.
Imaging Studies:
Ultrasonography: It is a non-invasive method which can see the subcutaneous nodules and affected lymph nodes.
MRI or CT scans: It is used to assess the involvement of any complications.
Biopsy: Biopsy is needed to confirm the diagnosis of the disease. It analyses the sample under a microscope to identify the different histopathological factors which can confirm the diagnosis.
Systemic Examination: Kimura disease may include the head and neck. The physician may perform a systemic evaluation of the other pars of the body which are involved.
Age group
Associated comorbidity
Associated activity
Acuity of presentation
Insidious Onset: Kimura disease occurs gradually over the weeks to months. Patient may not observe the symptoms early and swelling can be taken as a benign.
Painless Swelling: Kimura disease is painless swelling of soft tissue. It can occur at different locations like lymph nodes, salivary glands, and other subcutaneous tissues.
Tender Lymph Nodes: It affects the lymph nodes, and it can be tender or mild painful on palpation.
Recurrent Episodes: Kimura disease may have a chronic or recurrent course. Swelling can be entirely or partially gone. The recurrence of disease can stay for month to years.
Lymphadenopathy: Rubbery and enlarged lymph nodes is a common symptoms of Kimura disease.
Differential Diagnoses
Mikulicz’s Disease: Mikulicz’s disease is a rare medical disease and caused by a symmetrical enlargement of tear glands and salivary glands. It is part of IgG4-related disease (IgG4-RD). Patients who have Mikulicz’s disease can have a xerophthalmia and xerostomia because of the enlargement and inflammation of lacrimal and salivary gland.
Infective Parotitis: Infective parotitis occurs when salivary gland infected because of the bacterial pathogens like Staphylococcus aureus. It is linked with the pain, swelling and tenderness of the affected gland.
Salivary Gland Tumours: Salivary gland tumors are caused by the abnormal growth of the salivary gland. Different types of salivary gland tumor like pleomorphic adenoma, mucoepidermoid carcinoma, adenoid cystic carcinoma, and acinic cell carcinoma are linked with this disease.
Sjogren’s Syndrome: Sjogren’s syndrome is immune system related disease. It causes persistent inflammation of exocrine glands. Patients who have Sjogren’s Syndrome can have a xerophthalmia and xerostomia because of the enlargement and inflammation of lacrimal and salivary gland. It also affects other parts of body and causes the symptoms like fatigue, joint pain and rashes.
Other diseases may includes:
Castleman disease
Angioimmunoblastic T-cell lymphoma
Cylindrome
Angiolymphoid Hyperplasia alongwith Eosinophilia
Dermatofibrosarcoma Protuberans
Drug-related lymphadenopathy
Dermatologic Manifestations of the Kaposi Sarcoma
Hodgkin Lymphoma
Pyogenic Granuloma
IgG4-related disease
Lobular Capillary Hemangioma
Inflammatory disease of the orbit
Parasitic lymphadenitis
Malignant Lymphoma of Bone
Malignant tumors of the head and neck
Langerhans Cell Histiocytosis
Laboratory Studies
Imaging Studies
Procedures
Histologic Findings
Staging
Treatment Paradigm
Corticosteroids: Corticosteroid medication like prednisone is used to decrease the inflammation and control symptoms of kimura disease.
Immunosuppressant drugs: Immunosuppressive drugs is suggested when the corticosteroids is not effective. Cyclosporine and methotrexate is used to reduce the inflammation.
Surgical excision: Surgery is needed when the masses is not respond to the treatment. Surgery removes the masses of lymph nodes.
Radiation therapy: Radiation therapy is used when disease is not response to the treatment or recurrent of the disease.
Allergy testing: It is used to detect the allergens which are responsible to cause the inflammation and make the worse condition.
Supportive care: Supportive care like pain management and wound care is needed specifically if there is some complications or open sores in affected area.
by Stage
by Modality
Chemotherapy
Radiation Therapy
Surgical Interventions
Hormone Therapy
Immunotherapy
Hyperthermia
Photodynamic Therapy
Stem Cell Transplant
Targeted Therapy
Palliative Care
Effectiveness of Oral corticosteroids in combination with cetrizine to treat kimura disease
Oral corticosteroids along with cetirizine is used in cases where symptoms are not properly controlled by the corticosteroid alone. They can target both the immune response and allergy which is included in the disease and gives the effective relief.
Use of immunosuppressants in Kimura disease
leflunomide
Leflunomide is used to suppress the immune system and reduce the inflammation. There is limited evidence based on this treatment.
leflunomide in combination with oral prednisone
It includes glucocorticosteroids like oral prednisone to decrease the inflammation and control the symptoms.
cyclosporine
It is a treatment option for resistant to conventional treatment. It helps to control the symptoms and reduce the inflammation.
Use of Oral pentoxifylline in kimura disease
Oral pentoxifylline is effective to treat kimura disease as per the study.
Effectiveness of monoclonal antibody in kimura disease
dupilumab:
Dupilumab is effective to treat kimura disease as per the study on 1 patient who went for surgical excision. He was administered the dosage of 600 mg in starting and then 300 mg for every 3 weeks for 8 months. After the 1 year of follow up, there is no recurrence of disease.
Tyrosine kinase inhibitors as targeted therapy in kimura disease
Imatinib:
Imatinib is effective to manage Kimura disease in cases of refractory or not response to treatment. It inhibits the certain tyrosine kinase which are included in growth and proliferation of the cells.
medical-treatment
Corticosteroids: The main treatment is corticosteroids to treat Kimura disease. It decreases the inflammation and suppress immune response. It is administered by orally or injected directly in the affected area.
Immunosuppressive drugs: Immunosuppressive drugs is prescribed along with the corticosteroid to control the immune response.
Surgical Intervention:
Excisional Biopsy: Surgical excision and biopsy is needed when affected lymph nodes can cause discomfort, enlarge or have malignancy. It removes the affected tissue for examination under the microscope to exclude the cancer.
phases-of-management
Diagnosis and Evaluation: Proper diagnosis is necessary to start the proper treatment. There is many examinations and imaging procedures like ultrasound, MRI, or CT scan which is used. A biopsy of affected tissue is needed to conform the diagnosis.
Conservative Management: The 1st method to manage Kimura disease is to control the symptoms and inflammation. Corticosteroids like prednisone is used to reduce the swelling and immune response.
Immunosuppressive Therapy: Cyclosporine, methotrexate, and mycophenolate mofetil is used along with corticosteroids to inhibit the immune system and reduce the inflammation.
Monitoring and Follow-up: Regular monitor the progression of disease and treatment response is necessary. follow-up is needed for any complication which is occur during the treatment.
Surgical Intervention: If disease is not treated with medical treatment, surgery is needed to remove the affected lymph node and mass to reduce the symptoms and for further evaluation.
Radiation Therapy: Radiation treatment is needed in recurrent lesions. It is used because of the potential risk and long term side effects.
Supportive Care: Supportive care like pain management, wound care and psychological or emotional factors may be necessary to manage the kimura disease.
Kimura Disease or eosinophilic lymphogranuloma is a benign and rare chronic inflammatory disorder. It mainly affects soft tissues specifically lymph nodes and subcutaneous tissue. It accumulates eosinophils and leads to painless and enlarged mass in the head and neck area. It affects cervical lymph nodes and submandibular. It leads o swelling and cosmetic abnormalities which affect the overall quality of life.
Kimura disease is a rare disease. The higher rate is seen in areas of Asia like China, Japan, and Southeast Asian country. The prevalence rate is low other than these areas. It occurs in young adults whose age is late teens and early 30s. It is more common in men. The men to women ratio are about 3:1. It is seen in Asian ethnicity specifically in China and Japan.
Kimura disease is an abnormal response of immune system. It increases the production of different immune cells like T-helper 2 (Th2) cells, eosinophils, and immunoglobulin E (IgE). The overactive response of Th2 can lead to release of cytokines specifically IL-4 and IL-5. Kimura disease is linked with allergic and atopic conditions. The exact immune response is not known.
There is a proliferation of lymphoid tissue specifically in the lymph nodes and subcutaneous tissue in kimura disease. This can lead to accumulation of the immune cells like eosinophils and lymphocytes with the formation of the lymphoid follicles.
Eosinophils contribute to the immune system response of the body when it comes to the contact with allergens and parasites. These is infiltration of eosinophils in the affected tissue and lead to damage and inflammation to tissue in kimura disease.
Angiogenesis forms a new blood vessels. Kimura disease is caused by an increased synthesis of VEFG. It leads to the formation of vascularized lesion in the affected tissue. Genetic factors can also contribute to Kimura disease. Specific genes which are included in the regulation of immune system and allergic response may have a role in elevating the susceptibility to disease.
Kimura disease is a chronic and relapsing disease with the recurrence of inflammation and symptoms intermediately. The mechanism of he chronicity is not known but it is linked with the dysregulation of immune system and allergic responses.
Kimura disease occurs because of the abnormal response of immune system. It leads to chronic inflammation in lymph nodes and soft tissues.
As per some studies, Kimura disease can decrease the allergic response to specific allergens or environmental factor. Viral infections can trigger for Kimura disease. No particular virus is linked with this disease. Genetic predisposition may play role in the development of the Kimura disease. Specific genetic factors can contribute to the susceptibility of the disease. Kimura disease is not an autoimmune disease but some aspects are linked with autoimmune response.
Kimura disease can be caused by persistent or recurrent lesions after having the treatment. It can cause disfigurement specifically when the lesion is not treated specifically in neck and head area. Poor diagnosis of kimura disease is associated with the habits of smoking and any history of systemic disease.
Kimura disease can affect the young adult men of Asia specifically from China, Japan and Southeast Asia. The age group is between 20 years to 40 years. It also occurs in children and old adults.
Associated Comorbidity or Activity:
Kimura disease is linked with other allergic diseases like asthma, hay fever, and eczema. It is also linked with nephrotic syndrome caused by the presence of protein in urine, low levels of protein in blood. High levels of cholesterol, and edema.
Kimura disease can occur along with autoimmune diseases like SLE, and rheumatoid arthritis. Patients who have Kimura disease may have changes in the immune system and lead to abnormal immune response.
Blood Tests:
Complete Blood Count (CBC): It identifies the elevates count of eosinophils which is a main effect of Kimura disease.
Erythrocyte Sedimentation Rate (ESR): An increased ESR can suggest ongoing process of inflammatory response.
IgE levels: Levels of IgE increase in the Kimura disease.
Imaging Studies:
Ultrasonography: It is a non-invasive method which can see the subcutaneous nodules and affected lymph nodes.
MRI or CT scans: It is used to assess the involvement of any complications.
Biopsy: Biopsy is needed to confirm the diagnosis of the disease. It analyses the sample under a microscope to identify the different histopathological factors which can confirm the diagnosis.
Systemic Examination: Kimura disease may include the head and neck. The physician may perform a systemic evaluation of the other pars of the body which are involved.
Insidious Onset: Kimura disease occurs gradually over the weeks to months. Patient may not observe the symptoms early and swelling can be taken as a benign.
Painless Swelling: Kimura disease is painless swelling of soft tissue. It can occur at different locations like lymph nodes, salivary glands, and other subcutaneous tissues.
Tender Lymph Nodes: It affects the lymph nodes, and it can be tender or mild painful on palpation.
Recurrent Episodes: Kimura disease may have a chronic or recurrent course. Swelling can be entirely or partially gone. The recurrence of disease can stay for month to years.
Lymphadenopathy: Rubbery and enlarged lymph nodes is a common symptoms of Kimura disease.
Mikulicz’s Disease: Mikulicz’s disease is a rare medical disease and caused by a symmetrical enlargement of tear glands and salivary glands. It is part of IgG4-related disease (IgG4-RD). Patients who have Mikulicz’s disease can have a xerophthalmia and xerostomia because of the enlargement and inflammation of lacrimal and salivary gland.
Infective Parotitis: Infective parotitis occurs when salivary gland infected because of the bacterial pathogens like Staphylococcus aureus. It is linked with the pain, swelling and tenderness of the affected gland.
Salivary Gland Tumours: Salivary gland tumors are caused by the abnormal growth of the salivary gland. Different types of salivary gland tumor like pleomorphic adenoma, mucoepidermoid carcinoma, adenoid cystic carcinoma, and acinic cell carcinoma are linked with this disease.
Sjogren’s Syndrome: Sjogren’s syndrome is immune system related disease. It causes persistent inflammation of exocrine glands. Patients who have Sjogren’s Syndrome can have a xerophthalmia and xerostomia because of the enlargement and inflammation of lacrimal and salivary gland. It also affects other parts of body and causes the symptoms like fatigue, joint pain and rashes.
Other diseases may includes:
Castleman disease
Angioimmunoblastic T-cell lymphoma
Cylindrome
Angiolymphoid Hyperplasia alongwith Eosinophilia
Dermatofibrosarcoma Protuberans
Drug-related lymphadenopathy
Dermatologic Manifestations of the Kaposi Sarcoma
Hodgkin Lymphoma
Pyogenic Granuloma
IgG4-related disease
Lobular Capillary Hemangioma
Inflammatory disease of the orbit
Parasitic lymphadenitis
Malignant Lymphoma of Bone
Malignant tumors of the head and neck
Langerhans Cell Histiocytosis
Corticosteroids: Corticosteroid medication like prednisone is used to decrease the inflammation and control symptoms of kimura disease.
Immunosuppressant drugs: Immunosuppressive drugs is suggested when the corticosteroids is not effective. Cyclosporine and methotrexate is used to reduce the inflammation.
Surgical excision: Surgery is needed when the masses is not respond to the treatment. Surgery removes the masses of lymph nodes.
Radiation therapy: Radiation therapy is used when disease is not response to the treatment or recurrent of the disease.
Allergy testing: It is used to detect the allergens which are responsible to cause the inflammation and make the worse condition.
Supportive care: Supportive care like pain management and wound care is needed specifically if there is some complications or open sores in affected area.
Dermatology, General
Rheumatology
Oral corticosteroids along with cetirizine is used in cases where symptoms are not properly controlled by the corticosteroid alone. They can target both the immune response and allergy which is included in the disease and gives the effective relief.
Dermatology, General
Rheumatology
leflunomide
Leflunomide is used to suppress the immune system and reduce the inflammation. There is limited evidence based on this treatment.
leflunomide in combination with oral prednisone
It includes glucocorticosteroids like oral prednisone to decrease the inflammation and control the symptoms.
cyclosporine
It is a treatment option for resistant to conventional treatment. It helps to control the symptoms and reduce the inflammation.
Dermatology, General
Rheumatology
Oral pentoxifylline is effective to treat kimura disease as per the study.
Dermatology, General
Rheumatology
dupilumab:
Dupilumab is effective to treat kimura disease as per the study on 1 patient who went for surgical excision. He was administered the dosage of 600 mg in starting and then 300 mg for every 3 weeks for 8 months. After the 1 year of follow up, there is no recurrence of disease.
Dermatology, General
Rheumatology
Imatinib:
Imatinib is effective to manage Kimura disease in cases of refractory or not response to treatment. It inhibits the certain tyrosine kinase which are included in growth and proliferation of the cells.
Corticosteroids: The main treatment is corticosteroids to treat Kimura disease. It decreases the inflammation and suppress immune response. It is administered by orally or injected directly in the affected area.
Immunosuppressive drugs: Immunosuppressive drugs is prescribed along with the corticosteroid to control the immune response.
Surgical Intervention:
Excisional Biopsy: Surgical excision and biopsy is needed when affected lymph nodes can cause discomfort, enlarge or have malignancy. It removes the affected tissue for examination under the microscope to exclude the cancer.
Diagnosis and Evaluation: Proper diagnosis is necessary to start the proper treatment. There is many examinations and imaging procedures like ultrasound, MRI, or CT scan which is used. A biopsy of affected tissue is needed to conform the diagnosis.
Conservative Management: The 1st method to manage Kimura disease is to control the symptoms and inflammation. Corticosteroids like prednisone is used to reduce the swelling and immune response.
Immunosuppressive Therapy: Cyclosporine, methotrexate, and mycophenolate mofetil is used along with corticosteroids to inhibit the immune system and reduce the inflammation.
Monitoring and Follow-up: Regular monitor the progression of disease and treatment response is necessary. follow-up is needed for any complication which is occur during the treatment.
Surgical Intervention: If disease is not treated with medical treatment, surgery is needed to remove the affected lymph node and mass to reduce the symptoms and for further evaluation.
Radiation Therapy: Radiation treatment is needed in recurrent lesions. It is used because of the potential risk and long term side effects.
Supportive Care: Supportive care like pain management, wound care and psychological or emotional factors may be necessary to manage the kimura disease.
Kimura Disease or eosinophilic lymphogranuloma is a benign and rare chronic inflammatory disorder. It mainly affects soft tissues specifically lymph nodes and subcutaneous tissue. It accumulates eosinophils and leads to painless and enlarged mass in the head and neck area. It affects cervical lymph nodes and submandibular. It leads o swelling and cosmetic abnormalities which affect the overall quality of life.
Kimura disease is a rare disease. The higher rate is seen in areas of Asia like China, Japan, and Southeast Asian country. The prevalence rate is low other than these areas. It occurs in young adults whose age is late teens and early 30s. It is more common in men. The men to women ratio are about 3:1. It is seen in Asian ethnicity specifically in China and Japan.
Kimura disease is an abnormal response of immune system. It increases the production of different immune cells like T-helper 2 (Th2) cells, eosinophils, and immunoglobulin E (IgE). The overactive response of Th2 can lead to release of cytokines specifically IL-4 and IL-5. Kimura disease is linked with allergic and atopic conditions. The exact immune response is not known.
There is a proliferation of lymphoid tissue specifically in the lymph nodes and subcutaneous tissue in kimura disease. This can lead to accumulation of the immune cells like eosinophils and lymphocytes with the formation of the lymphoid follicles.
Eosinophils contribute to the immune system response of the body when it comes to the contact with allergens and parasites. These is infiltration of eosinophils in the affected tissue and lead to damage and inflammation to tissue in kimura disease.
Angiogenesis forms a new blood vessels. Kimura disease is caused by an increased synthesis of VEFG. It leads to the formation of vascularized lesion in the affected tissue. Genetic factors can also contribute to Kimura disease. Specific genes which are included in the regulation of immune system and allergic response may have a role in elevating the susceptibility to disease.
Kimura disease is a chronic and relapsing disease with the recurrence of inflammation and symptoms intermediately. The mechanism of he chronicity is not known but it is linked with the dysregulation of immune system and allergic responses.
Kimura disease occurs because of the abnormal response of immune system. It leads to chronic inflammation in lymph nodes and soft tissues.
As per some studies, Kimura disease can decrease the allergic response to specific allergens or environmental factor. Viral infections can trigger for Kimura disease. No particular virus is linked with this disease. Genetic predisposition may play role in the development of the Kimura disease. Specific genetic factors can contribute to the susceptibility of the disease. Kimura disease is not an autoimmune disease but some aspects are linked with autoimmune response.
Kimura disease can be caused by persistent or recurrent lesions after having the treatment. It can cause disfigurement specifically when the lesion is not treated specifically in neck and head area. Poor diagnosis of kimura disease is associated with the habits of smoking and any history of systemic disease.
Kimura disease can affect the young adult men of Asia specifically from China, Japan and Southeast Asia. The age group is between 20 years to 40 years. It also occurs in children and old adults.
Associated Comorbidity or Activity:
Kimura disease is linked with other allergic diseases like asthma, hay fever, and eczema. It is also linked with nephrotic syndrome caused by the presence of protein in urine, low levels of protein in blood. High levels of cholesterol, and edema.
Kimura disease can occur along with autoimmune diseases like SLE, and rheumatoid arthritis. Patients who have Kimura disease may have changes in the immune system and lead to abnormal immune response.
Blood Tests:
Complete Blood Count (CBC): It identifies the elevates count of eosinophils which is a main effect of Kimura disease.
Erythrocyte Sedimentation Rate (ESR): An increased ESR can suggest ongoing process of inflammatory response.
IgE levels: Levels of IgE increase in the Kimura disease.
Imaging Studies:
Ultrasonography: It is a non-invasive method which can see the subcutaneous nodules and affected lymph nodes.
MRI or CT scans: It is used to assess the involvement of any complications.
Biopsy: Biopsy is needed to confirm the diagnosis of the disease. It analyses the sample under a microscope to identify the different histopathological factors which can confirm the diagnosis.
Systemic Examination: Kimura disease may include the head and neck. The physician may perform a systemic evaluation of the other pars of the body which are involved.
Insidious Onset: Kimura disease occurs gradually over the weeks to months. Patient may not observe the symptoms early and swelling can be taken as a benign.
Painless Swelling: Kimura disease is painless swelling of soft tissue. It can occur at different locations like lymph nodes, salivary glands, and other subcutaneous tissues.
Tender Lymph Nodes: It affects the lymph nodes, and it can be tender or mild painful on palpation.
Recurrent Episodes: Kimura disease may have a chronic or recurrent course. Swelling can be entirely or partially gone. The recurrence of disease can stay for month to years.
Lymphadenopathy: Rubbery and enlarged lymph nodes is a common symptoms of Kimura disease.
Mikulicz’s Disease: Mikulicz’s disease is a rare medical disease and caused by a symmetrical enlargement of tear glands and salivary glands. It is part of IgG4-related disease (IgG4-RD). Patients who have Mikulicz’s disease can have a xerophthalmia and xerostomia because of the enlargement and inflammation of lacrimal and salivary gland.
Infective Parotitis: Infective parotitis occurs when salivary gland infected because of the bacterial pathogens like Staphylococcus aureus. It is linked with the pain, swelling and tenderness of the affected gland.
Salivary Gland Tumours: Salivary gland tumors are caused by the abnormal growth of the salivary gland. Different types of salivary gland tumor like pleomorphic adenoma, mucoepidermoid carcinoma, adenoid cystic carcinoma, and acinic cell carcinoma are linked with this disease.
Sjogren’s Syndrome: Sjogren’s syndrome is immune system related disease. It causes persistent inflammation of exocrine glands. Patients who have Sjogren’s Syndrome can have a xerophthalmia and xerostomia because of the enlargement and inflammation of lacrimal and salivary gland. It also affects other parts of body and causes the symptoms like fatigue, joint pain and rashes.
Other diseases may includes:
Castleman disease
Angioimmunoblastic T-cell lymphoma
Cylindrome
Angiolymphoid Hyperplasia alongwith Eosinophilia
Dermatofibrosarcoma Protuberans
Drug-related lymphadenopathy
Dermatologic Manifestations of the Kaposi Sarcoma
Hodgkin Lymphoma
Pyogenic Granuloma
IgG4-related disease
Lobular Capillary Hemangioma
Inflammatory disease of the orbit
Parasitic lymphadenitis
Malignant Lymphoma of Bone
Malignant tumors of the head and neck
Langerhans Cell Histiocytosis
Corticosteroids: Corticosteroid medication like prednisone is used to decrease the inflammation and control symptoms of kimura disease.
Immunosuppressant drugs: Immunosuppressive drugs is suggested when the corticosteroids is not effective. Cyclosporine and methotrexate is used to reduce the inflammation.
Surgical excision: Surgery is needed when the masses is not respond to the treatment. Surgery removes the masses of lymph nodes.
Radiation therapy: Radiation therapy is used when disease is not response to the treatment or recurrent of the disease.
Allergy testing: It is used to detect the allergens which are responsible to cause the inflammation and make the worse condition.
Supportive care: Supportive care like pain management and wound care is needed specifically if there is some complications or open sores in affected area.
Dermatology, General
Rheumatology
Oral corticosteroids along with cetirizine is used in cases where symptoms are not properly controlled by the corticosteroid alone. They can target both the immune response and allergy which is included in the disease and gives the effective relief.
Dermatology, General
Rheumatology
leflunomide
Leflunomide is used to suppress the immune system and reduce the inflammation. There is limited evidence based on this treatment.
leflunomide in combination with oral prednisone
It includes glucocorticosteroids like oral prednisone to decrease the inflammation and control the symptoms.
cyclosporine
It is a treatment option for resistant to conventional treatment. It helps to control the symptoms and reduce the inflammation.
Dermatology, General
Rheumatology
Oral pentoxifylline is effective to treat kimura disease as per the study.
Dermatology, General
Rheumatology
dupilumab:
Dupilumab is effective to treat kimura disease as per the study on 1 patient who went for surgical excision. He was administered the dosage of 600 mg in starting and then 300 mg for every 3 weeks for 8 months. After the 1 year of follow up, there is no recurrence of disease.
Dermatology, General
Rheumatology
Imatinib:
Imatinib is effective to manage Kimura disease in cases of refractory or not response to treatment. It inhibits the certain tyrosine kinase which are included in growth and proliferation of the cells.
Corticosteroids: The main treatment is corticosteroids to treat Kimura disease. It decreases the inflammation and suppress immune response. It is administered by orally or injected directly in the affected area.
Immunosuppressive drugs: Immunosuppressive drugs is prescribed along with the corticosteroid to control the immune response.
Surgical Intervention:
Excisional Biopsy: Surgical excision and biopsy is needed when affected lymph nodes can cause discomfort, enlarge or have malignancy. It removes the affected tissue for examination under the microscope to exclude the cancer.
Diagnosis and Evaluation: Proper diagnosis is necessary to start the proper treatment. There is many examinations and imaging procedures like ultrasound, MRI, or CT scan which is used. A biopsy of affected tissue is needed to conform the diagnosis.
Conservative Management: The 1st method to manage Kimura disease is to control the symptoms and inflammation. Corticosteroids like prednisone is used to reduce the swelling and immune response.
Immunosuppressive Therapy: Cyclosporine, methotrexate, and mycophenolate mofetil is used along with corticosteroids to inhibit the immune system and reduce the inflammation.
Monitoring and Follow-up: Regular monitor the progression of disease and treatment response is necessary. follow-up is needed for any complication which is occur during the treatment.
Surgical Intervention: If disease is not treated with medical treatment, surgery is needed to remove the affected lymph node and mass to reduce the symptoms and for further evaluation.
Radiation Therapy: Radiation treatment is needed in recurrent lesions. It is used because of the potential risk and long term side effects.
Supportive Care: Supportive care like pain management, wound care and psychological or emotional factors may be necessary to manage the kimura disease.
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