Table.
Characteristics of 27 Japanese Cases of Fibrosing Mediastinitis.
| Case | Age | Sex | Main symptoms | Location | Histological stage | Histoplasma (Serum Ab/Tissue) | Serum IgG4 (mg/dL) | Immunostaining of IgG4 | Serum CRP (mg/dL) | Symptomatic compressed organs | Main treatment | Outcome/ Recurrence | Ref |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | 32 | M | Facial swelling, neck tightening | Diffuse | NA | NA | NA | NA | 2.23 | SVC | Bypass surgery | Improved | 9 |
| 2 | 54 | M | Dyspnea, leg edema | Diffuse | NA | NA | NA | NA | Elevated | Thoracic duct | Steroid | Not improved | 10 |
| 3 | 28 | F | Chest pain, left shoulder pain, hoarseness | Diffuse | NA | NA | NA | NA | NA | Nerves | Resection, steroid, tranilast | Improved | 11 |
| 4 | 51 | F | Fever | Local | NA | NA | NA | NA | 6.8 | No | Resection | Improved | 12 |
| 5 | 56 | M | Dyspnea | Diffuse | NA | NA | NA | NA | 7.66 | No | Steroid | Partially improved | 13 |
| 6 | 68 | M | Hoarseness | Diffuse | II | NA/negative | NA | NA | 6.69 | Nerves | Steroid | Improved | 14 |
| 7 | 63 | F | Facial swelling | Local | NA | NA | NA | NA | NA | SVC | Resection, bypass surgery | Improved/partially recurred | 15 |
| 8 | 62 | M | Headache, face and right shoulder swelling | Local | NA | NA | NA | NA | NA | SVC | Balloon dilatation, stenting | Improved/partially recurred | 16 |
| 9 | 7 | M | None | Local | II | Negative/NA | NA | NA | NA | No | Medical observation | Stable | 17 |
| 10 | 67 | F | Fever | Local | NA | NA | NA | NA | NA | No | Resection | Improved | 18 |
| 11 | 52 | M | Back pain | Diffuse | NA | NA | 392* | Positive | 9.3 | No | Steroid | Improved | 19 |
| 12 | 65 | F | Hoarseness | Diffuse | NA | NA/negative | NA | NA | 0.4 | Nerves | Steroid | Improved | 20 |
| 13 | 61 | M | None | Diffuse | NA | NA | 583* | Positive | NA | Ureter | Steroid | Improved | 21 |
| 14 | 75 | F | Left neck swelling | Diffuse | NA | NA/negative | NA | NA | NA | Carotid artery | Steroid | Improved | 22 |
| 15 | 56 | F | Dyspnea | Diffuse | NA | NA | 127 | Positive | NA | Main bronchus | Steroid | Improved | 23 |
| 16 | 54 | M | None | Local | NA | NA | NA | NA | NA | No | Resection | Stable | 24 |
| 17 | 68 | M | Intermittent claudication | Diffuse | NA | NA | NA | NA | 0.26 | Iliac artery | Steroid, saireito | Partially improved | 25 |
| 18 | 28 | M | Dyspnea | Local | NA | Negative/negative | 20.9 | Negative | NA | Thoracic duct | Steroid, octreotide | Improved (steroid ineffective) | 26 |
| 19 | 36 | M | Abdominal pain | Local | II | Negative/NA | NA | NA | 5.2 | No | Resection | Improved | 27 |
| 20 | 54 | M | Chest pain, dyspnea | Diffuse | II | NA | NA | Negative | 0.07 | Pulmonary artery, main bronchus | Steroid | Partially improved | 28 |
| 21 | 52 | F | Malaise, weight loss | Diffuse | NA | NA/negative | NA | NA | 12.6 | No | Steroid, CPA | Improved | 29 |
| 22 | 61 | F | None | Local | NA | NA | 7.9 | Negative | NA | No | Resection, bypass surgery | Stable | 30 |
| 23 | 68 | F | None | Local | NA | NA | NA | NA | NA | No | Resection, steroid | Improved | 31 |
| 24 | 66 | F | None | Diffuse | NA | NA | 276* | Positive | 4.11 | No | Steroid | Improved | 32 |
| 25 | 78 | F | None | Diffuse | NA | NA | 1940* | Positive | 0.03 | No | Steroid | Improved | 32 |
| 26 | 62 | F | Chest pain | Diffuse | II | Negative/NA | 97.5 | Negative | 8.17 | No | Steroid | Improved/recurred | Present Case |
| 27 | 77 | M | None | Diffuse | II | Negative/NA | 487* | Negative | 0.27 | No | Steroid | Improved | Present Case |
Ab: antibody, NA: not available, SVC: superior vena cava, CPA: cyclophosphamide
* Cutoff value of serum IgG4>135 mg/dL