TABLE 6.
Insurance application of apheresis by disease and maximum number of treatments
| Disease | Type of treatment | Number of times the treatment can be used | Application conditions |
|---|---|---|---|
| Hematologic diseases | |||
| Multiple myeloma | PE, DFPP | Once a week, up to 3 months | None |
| Thrombotic thrombocytopenic purpura | PE, DFPP | Generally, up to 2 days after the platelet count has reached 150 000/μL. One month after starting is the upper limit. | None |
| Hemolytic‐uremic syndrome | PE, DFPP | Up to 21 times | None |
| Macroglobulinemia | PE, DFPP | Once a week, up to 3 months | None |
| Hemophilia with inhibitors | PE, DFPP | Not specified | Inhibitor titer is ≥5 Bethesda units |
| Gastrointestinal diseases | |||
| Fulminant hepatitis | PE, PA | About 10 times | To remove bilirubin and bile acids |
| Postoperative liver failure | PE, DFPP | About 7 times | Indicated for postoperative liver dysfunction that satisfies the following conditions: |
| 1. Total bilirubin ≥5 mg/dL and continuously elevated | |||
| 2. Hepaplastin test (HPT) ≤40% or have ≥2 conditions of Coma Grade II or higher | |||
| Acute liver failure | PE, DFPP | About 7 times | Only when it can be determined that the severity is the same as fulminant hepatitis or postoperative liver failure based on findings such as prothrombin time, coma grade, total bilirubin and hepaplastin test |
| Chronic hepatitis C | PE, DFPP | Up to 5 times | Genotype lb where the blood HVC‐RNA load is ≥100K IU/mL (5 log IU/mL) even after interferon treatment |
| Hepatic coma | HA | Not specified | Not specified |
| Ulcerative colitis | c‐LCAP, f‐LCAP, GCAP | Up to 10 times, but up to 11 times for patients with fulminant conditions | Calculated only for improving the active disease phase and inducing remission for patients with severe, fulminant and refractory conditions (diagnostic criteria from the MHLW Grant‐in‐Aid for Intractable Disease Policy Research Project “Research on Intractable Inflammatory Bowel Disease”) |
| Crohn's disease | c‐LCAP, GCAP | Up to 10 times | For inducing remission in patients with moderate to severe Crohn's disease in the active phase of the disease, which is unresponsive to nutritional therapy and existing pharmacotherapy or such treatment is not indicated, and there are clear clinical symptoms caused by residual colon lesions |
| Transplantation‐related conditions | |||
| Allogenic liver transplantation with ABO incompatibility or antilymphocyte antibody positive | DFPP | Up to 4 times preoperatively and 2 times postoperatively | For allogenic liver transplantation with ABO incompatibility or antilymphocyte antibody positive allogenic liver transplantation |
| Allogenic kidney transplantation with ABO incompatibility or antilymphocyte antibody positive | DFPP | Up to 4 times preoperatively and 2 times postoperatively | For allogenic kidney transplantation with ABO incompatibility or antilymphocyte antibody positive allogenic kidney transplantation |
| Neuropathies | |||
| Myasthenia gravis | PE, DFPP, PA | 7 times a month, up to 3 months | Serious symptoms tending to worsen within 5 years after onset or insufficient response to thymectomy or corticosteroids |
| Multiple sclerosis | PE, DFPP, PA | 7 times a month, up to 3 months | None |
| Chronic inflammatory demyelinating polyneuropathy | PE, DFPP, PA | 7 times a month, up to 3 months | None |
| Guillain‐Barré syndrome | PE, DFPP, PA | 7 times a month, up to 3 months | ≥4 on Hughes Functional Grading Scale |
| Kidney diseases | |||
| Focal glomerulosclerosis | PE, DFPP, PA | Up to 12 times within 3 months | Non‐responsive to conventional pharmacotherapy, with persistent nephrotic condition, and serum cholesterol does not fall below 250 mg/dL |
| Anti‐glomerular basement membrane disease type rapidly progressive glomerulonephritis | PE, DFPP | Treatment up to 7 times in 2 weeks is one treatment course, up to 2 courses | Patients diagnosed with rapidly progressive glomerulonephritis (RPGN) positive for antineutrophil cytoplasmic antibodies (ANCA) |
| Collagen diseases | |||
| Rheumatoid arthritis | f‐LCAP | Once a week, up to 5 weeks | Patients with highly active, drug‐resistant disease or rapidly progressive drug‐resistant rheumatoid arthritis with systemic symptoms such as fever and severe synovitis in multiple joints who satisfy the following two conditions: |
| 1. ≥6 swollen joints | |||
| 2. ESR ≥50 mm/h OR CRP ≥3 mg/dL | |||
| Malignant rheumatoid arthritis | PE, DFPP, PA | Once a week | Patients recognized by the prefectural governor as a recipient of specific disease treatment, presenting with high‐grade extraarticular symptoms due to vasculitis (refractory lower leg ulcers, polyneuritis and melena due to mesenteric artery thrombosis), who is non‐responsive to conventional therapy |
| Systemic lupus erythematosus | PE, DFPP, PA | 4 times a month | Patients for whom any of the following are applicable: |
| 1. Recognized by the prefectural governor as a recipient of specific disease treatment | |||
| 2. Serum complement level (CH50) is ≤20 units, complement protein (C3) is ≤40 mg/dL and anti‐DNA antibodies are markedly elevated, and patient is unresponsive to steroid therapy or treatment is clinically inappropriate | |||
| 3. Patient diagnosed with rapidly progressive glomerulonephritis (RPGN) or central nervous system lupus (CNS lupus) | |||
| Cutaneous diseases | |||
| Pemphigus | PE, DFPP | Up to twice a week, for a maximum of 3 months. However, for patients with moderate or worse severity (based on pemphigus score proposed by the MHLW Specific Disease Research Group) after 3 months' treatment, insurance may be calculated for a further 3 months only | Limited to patients with a definitive diagnosis whose condition is refractory to other treatment or who cannot be treated with high‐dose steroids due to complications or adverse drug reactions |
| Pemphigoid conditions | PE, DFPP | Up to twice a week, for a maximum of 3 months. | Limited to patients whose condition is refractory to other treatment or who cannot be treated with high‐dose steroids due to complications or adverse drug reactions |
| Toxic epidermal necrosis | PE, DFPP | Up to 8 times | None |
| Stevens‐Johnson syndrome | PE, DFPP | Up to 8 times | None |
| Psoriasis vulgaris | c‐LCAP, GCAP | Treatment once a week, up to 5 weeks is one treatment course, with a maximum of 1 course | To improve clinical symptoms in patients with moderate or worse condition who are non‐responsive to or cannot be treated with pharmacotherapy (diagnostic criteria from the MHLW Grant‐in‐Aid for Intractable Disease Policy Research Project on Rare and Intractable Skin Diseases) |
| Psoriatic arthritis | c‐LCAP, GCAP | Treatment once a week, up to 5 weeks is one treatment course, with a maximum of 2 courses However, treatment should be stopped after one course if it is determined that the patient is non‐responsive to treatment | To improve clinical symptoms in patients who are non‐responsive to or cannot be treated with existing pharmacotherapy in accordance with guidelines issued by related associations |
| Cardiovascular diseases | |||
| Arteriosclerosis obliterans | PE, DFPP, PA | Up to 10 times for a maximum of 3 months | Calculated only for patients with arteriosclerosis obliterans applicable to all the following conditions: |
| 1. Patients presenting with symptoms at or higher than Fontaine classification II | |||
| 2. Patients with hypercholesterolemia whose total cholesterol is 220 mg/dL or LDL cholesterol will not fall below 140 mg/dL with pharmacotherapy | |||
| 3. Patients with obstruction below the popliteal artery or an extensive occlusion site for whom surgery is difficult and who are not sufficiently responsive to conventional pharmacotherapy | |||
| Familial hypercholesterolemia | PE, DFPP, PA | Once a week | Patients applicable to any of the following conditions with xanthoma and coronary arteriosclerosis is apparent on load ECG and angiography: |
| 1. Homozygous patients with fasting steady state serum total cholesterol exceeding 500 mg/dL | |||
| 2. Heterozygous patients with steady state (state where weight and plasma albumin can be maintained) serum cholesterol exceeding 400 mg/dL with diet therapy and whose serum cholesterol will not fall below 250 mg/dL with pharmacotherapy | |||
| Other | |||
| Drug poisoning | PE, HA | PE: up to 8 times, HA: not specified | None |
| Severe blood type incompatibility pregnancy | PE, DFPP | None | Blood type incompatibility pregnancies with a history of intrauterine fetal distress or neonatal jaundice due to Rh blood type incompatibility pregnancy and indirect Coombs test is 64‐times or higher at less than 20 weeks' gestation or 128‐times or higher at more than 20 weeks' gestation |
| Kawasaki disease | PE, DFPP | Up to 6 times | When immunoglobulin therapy, steroid pulse therapy or neutrophil elastase inhibitors are either ineffective or not indicated |
| Sepsis | HA | Up to 2 times | In patients older than 18 years, calculated for patients applicable to all the conditions listed from 1) to 2) below: |
| 1. Simultaneously satisfies one or more of the items listed in (a) to (c) below | |||
| a. Positive blood cultures for gram‐negative bacilli | |||
| b. Gram‐negative bacillary infection was suspected at another hospital and antibiotics were administered | |||
| c. Septic shock due to gram‐negative bacilli is strongly suspected and the criteria for acute phase DIC scores are ≥4 or equivalent state | |||
| 2. Meeting the definition of septic shock in the Japanese Guidelines for the Management of Sepsis 2016 | |||
| In patients younger than 18 years, Patients suspected endotoxin sepsis or gram‐negative bacterial infection who meet the criteria for pediatric SIRS in the Japanese Guidelines for the Management of Sepsis 2016 | |||
Abbreviations: DFPP, double filtration plasmapheresis; GCAP, granulocyte apheresis; HA, hemoadsorption; PA, plasma adsorption; PE, plasma exchange; LCAP, leukocytapheresis.