Greetings
Intractable Disease Research Center was established in 1988 under the designation of Kanagawa Prefecture.
Intractable diseases are diseases with unknown causes and no established treatments. They can develop chronically and can cause aftereffects, placing a significant economic and psychological burden on patients. Kanagawa Prefecture has designated four base hospitals within the prefecture, established the Intractable Disease Treatment Research Center, and subsidized its operation to support patients with intractable diseases by promoting research into the treatment and Intractable Disease Research Center intractable diseases, providing medical consultations for patients and their families, and training medical professionals. Currently, the Center is operated as a commissioned project by Kanagawa Prefecture, but in the future, intractable disease medical system reforms will see the establishment of base hospitals for intractable disease medical care within the prefecture. Under this new system related to the reform of intractable disease countermeasures, we will provide the latest information to patients, their families, and medical professionals.
Director of Intractable Disease Research Center
Takeshi Oku
Features
Intractable diseases are those with unknown causes and no established treatments. They are chronic and can cause long-term aftereffects, placing a significant economic and psychological burden on people. These include a wide range of diseases, including collagen diseases such as systemic lupus erythematosus, scleroderma, and dermatomyositis, blood diseases such as aplastic anemia, neurological diseases such as amyotrophic lateral sclerosis and myasthenia gravis, digestive diseases such as ulcerative colitis and Crohn's disease, as well as liver disease, heart disease, lung disease, skin disease, kidney disease, endocrine disorders, bone and joint diseases, and genetic disorders. Many of these diseases are designated as nationally designated diseases eligible for medical assistance. As of March 2024, the number of diseases eligible for medical assistance has increased to 341.
Kanagawa Prefecture has designated four base hospitals within the prefecture, established intractable disease treatment research centers, and subsidized their operation, with the aim of supporting patients with intractable diseases by promoting research into the treatment and Intractable Disease Research Center, providing medical consultations for patients and their families, and training for medical professionals. Our center is one of these centers.
The center also provides treatment for intractable diseases, conducts treatment research, provides medical consultations, and holds lectures. Medical treatment for each disease is provided by specialized departments such as Rheumatology, Hematology-oncology, Department of Neurology, Gastroenterology, Cardiology, Respiratory Medicine, Department of Renal Endocrinology and Metabolism, and metabolism, plastic surgery, and dermatology. Medical consultations are handled through the hospital's General Consulting Office, where social workers work with doctors, pharmacists, and registered dietitians to answer questions from patients and their families.
We hold lectures twice a year called "Incurable Disease Lectures" aimed at patients, their families, and medical professionals, providing the latest information on incurable diseases that will be useful for treatment and convalescence.
Rheumatology
Rheumatology diagnoses and treats many intractable diseases that have been designated as specific diseases by the government and are eligible for medical assistance. Representative diseases include systemic lupus erythematosus, systemic sclerosis, polymyositis/dermatomyositis, and vasculitis syndrome. These diseases are characterized by varying degrees of damage to organs throughout the body, making advances in diagnostic and therapeutic methods essential to alleviating symptoms. Furthermore, because treatment is long-term, continuous support is also necessary for patients and their families.
Department of Neurology
Many of the intractable diseases treated by Department of Neurology have been designated as specific diseases by the government and are eligible for medical assistance. Representative diseases include Parkinson's disease, amyotrophic lateral sclerosis, myasthenia gravis, multiple sclerosis/neuromyelitis optica, chronic inflammatory demyelinating polyneuropathy/multifocal motor neuropathy, spinocerebellar degeneration, and multiple system atrophy. Many of these diseases have had no cure until now, but in recent years, the emergence of new biologics has led to an increase in treatable diseases. Furthermore, because treatment is long-term, continuous support is also necessary for patients and their families.
Gastroenterology
Gastroenterology provides medical care for intractable diseases of the digestive system that are eligible for national medical subsidies. Representative diseases include ulcerative colitis, which has the largest number of registered patients among all designated diseases, as well as related diseases such as Crohn's disease, primary biliary cholangitis, and several liver diseases, including autoimmune hepatitis. These diseases often require long-term treatment and management, and there is a need for support for patients and their families, as well as research and advances in further diagnostic and therapeutic methods. We Gastroenterology are committed to playing a part in this effort.
Cardiology
Cardiology diagnoses and treats many intractable diseases designated by the government as specific diseases and eligible for medical assistance. Representative diseases include cardiomyopathies such as idiopathic dilated cardiomyopathy and hypertrophic cardiomyopathy. In recent years, we have seen an increase in cases of patients receiving treatment for congenital heart disease, such as tetralogy of Fallot and transposition of the great arteries, in childhood, who then visit our department as adults with adult congenital heart disease. Furthermore, we work in collaboration with Respiratory Medicine to treat conditions such as chronic thromboembolic pulmonary hypertension and pulmonary arterial hypertension. Treatments are constantly evolving, and some diseases can achieve dramatic improvements through not only drug therapy but also invasive treatment to improve symptoms and prognosis.
Respiratory Medicine
Idiopathic interstitial pneumonia is the most common designated intractable disease treated in Respiratory Medicine. Starting in 2024, the diagnostic criteria and severity classification will be revised, making it easier to receive medical assistance. Therefore, patients receiving home oxygen therapy or taking expensive anti-fibrotic drugs (Pirespa, Ofev) should consult with their doctor. Other designated intractable diseases treated in Respiratory Medicine include sarcoidosis, lymphangioleiomyomatosis, autoimmune pulmonary alveolar proteinosis, ciliary dyskinesia syndrome, eosinophilic granulomatosis with polyangiitis, and obesity-hypoventilation syndrome.
Department of Renal Endocrinology and Metabolism
Some of the diseases treated in Department of Renal Endocrinology and Metabolism have been designated as specific diseases by the government and are eligible for medical assistance.
Representative diseases in the kidney field include IgA nephropathy, polycystic kidney disease, Alport syndrome, rapidly progressive glomerulonephritis, antiglomerular basement membrane nephritis, primary nephrotic syndrome, primary membranoproliferative glomerulonephritis, purpura nephritis, IgG4-related disease, etc. These diseases are characterized by varying degrees of damage not only to the kidneys but also to various organs throughout the body, so early diagnosis through kidney biopsy and therapeutic intervention are important to improve symptoms.
Representative diseases in the endocrine field include pituitary ADH insufficiency, pituitary TSH hypersecretion, pituitary PRL hypersecretion, Cushing's disease, pituitary gonadotropin hypersecretion, pituitary growth hormone hypersecretion, anterior pituitary insufficiency, etc. These diseases cause symptoms due to abnormal hormone secretion, so treatment involves hormone replacement or hormone suppression.
Representative diseases in the field of diabetes include mitochondrial disease, familial hypercholesterolemia, etc. If left untreated, these diseases can damage various organs throughout the body to varying degrees, so it is important to diagnose them early and provide treatment appropriate to the condition.
dermatology
dermatology diseases include autoimmune bullous diseases such as pemphigus and pemphigoid, severe drug eruptions such as Stevens-Johnson syndrome and toxic epidermal necrolysis, pustular psoriasis, neurofibromatosis, and idiopathic acquired generalized anhidrosis, all of which are designated as intractable diseases. Many patients suffer from these diseases, the causes of which have not yet been elucidated, or the causes of which have been elucidated but which are severe and intractable. While patient treatment is ongoing, we hope that medical advances will lead to the elucidation of the causes and the development of new treatments.