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As its name suggests, the Rheumatology and Collagen Disease Department treats "rheumatism" and "collagen diseases" as internal medicine. While some hospitals refer to it as "rheumatology" or "collagen disease," it is not uncommon for general hospitals (even university hospitals) to lack such departments or to merge them with other departments. Our hospital opened its doors in June 2005 as a branch of the Immunohistochemistry and Hematology Department and provides outpatient care every weekday. Collagen diseases encompass many intractable illnesses, and our hospital is one of 12 hospitals designated as a core hospital for the treatment of intractable diseases in Osaka Prefecture. Collagen diseases, including rheumatoid arthritis, can damage various organs throughout the body and can sometimes be fatal. Our hospital boasts highly specialized departments in nearly every field, enabling us to collaborate and provide multidisciplinary diagnosis and treatment. As treatment is often long-term, we will continue to strive to be a trusted partner.
Autoimmune diseases are conditions in which the immune system, which is supposed to protect the body from foreign substances, malfunctions and damages the body's own cells and organs. Among these, collagen diseases, or broader rheumatic diseases, are those that cause inflammation in connective tissues such as blood vessels, skin, muscles, joints, and internal organs, resulting in fever and joint pain. Depending on the organ primarily affected, these diseases are classified as systemic lupus erythematosus (SLE), rheumatoid arthritis, scleroderma, polymyositis/dermatomyositis, polyarteritis nodosa, and mixed connective tissue disease. Because the cause is unknown, many of these diseases are designated as specified diseases (intractable diseases) by the Ministry of Health, Labor, and Welfare. Other related collagen diseases include Behçet's disease and Still's disease, which affect multiple organs in a manner similar to collagen diseases.
Although diagnostic criteria have been established for each type of collagen disease, they are not always met from the beginning, and since many cases have a chronic course, long-term follow-up is necessary. Accurate diagnosis and assessment of disease activity are important for treatment. High-dose corticosteroids and immunosuppressants are required during periods of high activity, while low-dose maintenance steroids, supportive therapy, or no treatment at all are often required during periods of low activity. Patients are often referred as positive for antinuclear antibodies and rheumatoid reactions, but these reactions can be positive even in cases other than collagen diseases. Therefore, we strive to determine whether or not a collagen disease truly requires treatment, and even in cases where treatment is not required, we carefully monitor the patient's progress, considering the possibility of future symptoms developing.
Rheumatoid arthritis is a systemic inflammatory disease of unknown cause that chronically progresses to joint destruction. Synovial proliferation and inflammation gradually spread to surrounding cartilage and bone, resulting in joint destruction and deformity. Extra-articular symptoms may include subcutaneous nodules, mononeuritis multiplex, vasculitis, pulmonary fibrosis, and amyloidosis. Autoimmune diseases such as Sjögren's syndrome are often present. Early initiation of initial treatment and the use of DMARDs (deadbeat syndrome) are essential for rheumatoid arthritis management. Steroids are used as initial treatment when the disease is highly active. Since treatment is often prolonged, careful monitoring of the efficacy and side effects of each medication is essential. As mentioned in the Topics section, biologic agents targeting TNF-α, a molecule that exacerbates the disease, have recently become available for the treatment of rheumatoid arthritis (RA). While these agents offer great promise for therapeutic efficacy, caution is required regarding side effects, such as relapse of tuberculosis.
It is thought that there are approximately 40,000 patients across Japan, and the disease is common among women aged 15 to 65. The cause is currently unknown, but immune abnormalities play an important role in the development of the disease. UV rays, viral infections, injuries, surgery, pregnancy and childbirth, and certain medications are known to be triggers that can cause or worsen the disease.
Various symptoms occur, including fever, general fatigue, joint problems, skin and mucous membrane problems (butterfly rash, discoid rash, hair loss, stomatitis), and internal organs (kidneys, heart, lungs, abdomen, nerves). Not all symptoms will occur, and the number of symptoms and affected organs will vary from person to person.
Treatment varies depending on the severity of the disease, but treatment is centered around corticosteroids and immunosuppressants. If these are ineffective, extracorporeal circulation therapy may be performed to remove the immune complexes and lymphocytes that cause the disease. If antiphospholipid syndrome, which makes blood clots more likely, is also present, anticoagulant therapy is used, if there is severe circulatory disorder, vasodilators are used, and dialysis is used for renal failure.
In mild cases, it is not uncommon for patients to be able to lead a normal life, almost the same as a healthy person. Before corticosteroids were known, the five-year survival rate was around 50%, but it has now improved to over 90%. With the use of immunosuppressants, disease control has become even better.
Scleroderma is a disease whose main symptom is hardening of the skin, but there are two types: localized scleroderma, in which hardening is only seen in the skin, and systemic scleroderma, in which lesions affect multiple organs, including the lungs, kidneys, and digestive tract. Raynaud's syndrome is seen in many patients with scleroderma. In systemic scleroderma, the presence or absence of pulmonary lesions such as interstitial pneumonia and pulmonary fibrosis, renal lesions, and pulmonary hypertension are related to the prognosis.
Scleroderma lesions generally progress slowly, but in some cases, skin hardening, interstitial pneumonia, and kidney failure can progress rapidly.
Steroids may be effective in cases of interstitial pneumonia, pericarditis, and pleurisy, but they are not very effective for hardening skin lesions. It is difficult to suppress Raynaud's phenomenon with drug therapy. Antiplatelet aggregators and vasodilators are used to improve peripheral circulatory disorders.
Mixed connective tissue disease (MCTD) is a disease characterized by a combination of systemic lupus erythematosus (SLE)-like symptoms, scleroderma-like symptoms, and polymyositis-like symptoms, and high serum anti-RNP antibodies. In addition to Raynaud's phenomenon or swelling of the fingers and dorsum of the hands, MCTD also exhibits the erythema and arthritis of SLE, the skin hardening and pulmonary fibrosis of scleroderma, and symptoms of polymyositis, but these symptoms are relatively mild. Five to ten percent of patients with MCTD develop pulmonary hypertension, a serious complication that affects the prognosis.
If MCTD is diagnosed, the need for steroid therapy is determined based on the type of organ affected and the extent of the damage. If steroids are not appropriate, symptomatic treatment will be performed. Treatment for pulmonary hypertension is the same as for primary pulmonary hypertension, but in early stages, steroid treatment may be effective.
Polymyositis is a disease in which muscle destruction occurs through an autoimmune mechanism, resulting in muscle weakness and muscle pain. Proximal muscles (muscles close to the trunk of the body) are particularly affected. When myositis is accompanied by a characteristic skin rash (dermatomyositis), it is called dermatomyositis.
In addition to muscle involvement, other symptoms include pulmonary fibrosis, malignant tumor complications (approximately 30% of dermatomyositis cases), joint symptoms, carditis symptoms, and gastrointestinal symptoms (dysphagia, emphysematous cysts, etc.). Pulmonary fibrosis is observed in approximately half of patients and is a complication that affects life prognosis. Pulmonary fibrosis is said to be particularly serious in cases of dermatomyositis with minimal myoinflammatory symptoms. Malignant tumors must be actively searched for.
Treatment varies depending on the symptoms and the organs affected. Drug therapy for myositis involves steroid therapy and, in some cases, immunosuppressants, but care must be taken to avoid side effects. If a malignant tumor is present, treatment of the malignant tumor takes priority.
It is estimated that there are approximately 5,000 patients across Japan. The cause is still unknown, but it is thought to be caused by an immune abnormality.
This disease is characterized by habitual miscarriage, arterial and venous thrombosis, and a low platelet count, and is often associated with systemic lupus erythematosus (SLE).
It is necessary to eliminate risk factors for thrombosis in daily life, quit smoking, improve high blood pressure and hyperlipidemia, and discontinue oral contraceptives. Even if antiphospholipid antibodies are positive, if there is no history of thrombosis or symptoms, there is no need for active treatment and the patient is simply monitored. Thrombolytic therapy and anticoagulant therapy are performed for acute arterial and venous thrombosis. In the chronic phase, antiplatelet and anticoagulant therapy are used to prevent recurrence. In rare cases, there is a fulminant type in which multiple microthrombi occur in organs throughout the body, and plasma exchange, plasma adsorption, high-dose steroid therapy, the use of immunosuppressants, and strong anticoagulant therapy are performed.
Steroids is a general term for hormones including sex hormones (female and male), glucocorticoids produced by the adrenal cortex, and mineral steroids produced by the adrenal medulla. However, steroids used clinically (hereafter abbreviated as steroids) are glucocorticoids. Steroids have the effects of suppressing abnormal immune responses, inflammation, and allergies, and are highly effective and rapid, making them extremely useful in treatment. However, they are also known for their many side effects. To use steroids effectively, it is important to fully understand the merits and demerits of these drugs.
This article will discuss some particularly important points regarding how to use antihistamines. First, be sure to ask your doctor why you need to take the medication, as well as how much and for how long. It is important to minimize the amount and duration of antihistamine use, but in cases such as collagen disease, sudden weight loss can lead to a relapse of the disease. It is important to understand the significance and necessity of taking antihistamines and to take the prescribed dosage exactly.
It is also important to fully understand the side effects, including infections. It is important to prevent side effects and detect them early. It is especially important to check for tuberculosis and hepatitis B before taking the drug.
Another important point is that if you take steroids for a long period of time, the adrenal cortex will become atrophied. Therefore, if you suddenly stop taking steroids, the adrenal glands will no longer secrete steroids, which can lead to serious symptoms such as shock, hypoglycemia, and fever. This is very dangerous, so if you have been taking steroids for a long time, do not suddenly stop taking them. If you are unable to take the medication due to vomiting or other reasons, please see a doctor.
These are the precautions to take when taking antihistamines. It is possible to minimize side effects. It is important to minimize the amount of antihistamine and the duration of use, as well as to deal with any side effects as soon as possible.
<Outpatient treatment statistics> (April 1, 2022 to March 31, 2023)
Total number of outpatients: 15,350, average monthly number: 1,279
Number of first-time patients: 967, monthly average: 81
<Inpatient treatment results> (April 1, 2022 to March 31, 2023)
Inpatients are primarily treated for collagen diseases such as systemic lupus erythematosus and rheumatoid arthritis. In fiscal year 2022, the number of hospitalized patients was 351, with an average length of stay of 20.7 days.
Major hospitalizations in 2022 were:
Rheumatoid arthritis (50 patients), systemic lupus erythematosus (52 patients), polymyositis/dermatomyositis (15 patients), scleroderma (14 patients), mixed connective tissue disease (5 patients), vasculitis syndrome (66 patients), Sjogren's syndrome (3 patients), antiphospholipid antibody syndrome (5 patients), Behcet's disease (1 patient), polymyalgia rheumatica (16 patients), adult Still's disease (14 patients), spondyloarthritis (3 patients), crystal-induced synovitis (1 patient), relapsing polychondritis (1 patient), IgG4-related disease (6 patients), etc.
Symptoms of arthritis: joint pain and swelling, morning joint stiffness, skin symptoms: rash, swelling of the fingers and feet, Raynaud's syndrome (whitening of the fingers in cold weather), recurring mouth ulcers, muscle pain and weakness, dry mouth and eyes, unexplained fever, etc.
Positive antinuclear antibodies, positive rheumatoid reaction, hypergammaglobulinemia, and positive results for various autoantibodies are also present.
We treat a variety of conditions, including rheumatoid arthritis, malignant rheumatoid arthritis, systemic lupus erythematosus (SLE), scleroderma, mixed connective tissue disease, vasculitis syndromes (polyarteritis nodosa, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyarteritis, etc.), polymyositis/dermatomyositis, Behçet's disease, Sjögren's syndrome, adult-onset Still's disease, and polymyalgia rheumatica.
Although diagnostic criteria have been established for each of these diseases, they are not always met from the beginning, and since they often have a chronic course, long-term follow-up is necessary. Accurate diagnosis and assessment of disease activity are important for treatment; high-dose corticosteroids and immunosuppressants are required during periods of high activity, while low-dose maintenance steroids, supportive therapy, or no treatment at all are often required during periods of low activity. Patients are often referred for positive antinuclear antibodies or rheumatoid reactions, but these reactions can be positive even in cases other than collagen diseases. Therefore, we strive to determine whether or not a patient truly has a collagen disease requiring treatment, and even in cases where treatment is not required, we carefully monitor the patient's progress, considering the possibility of future symptoms.
