Table 1.
Case [Ref.] | Sex/Age (years) | Underlying disease (s) | Reduction of IS level before symptoms onset of IRD | Symptoms & signs at IRD; change of lymphocyte count before & during IRD (if mentioned) | Treatment, clinical progress & outcome |
1–7 [22] | M/F: 4:3 Median age 12, range 2–25 | Acute leukemia in remission (4), acute leukemia in relapse (1), Hodgkin's disease (1), embryonal carcinoma of testes (1) | P ↓ from 100 mg to 40 mg over 3 weeks in 1 patients; In another 6 patients, P stopped in a median of 10.5 days, range (1–21 days) before symptoms onset |
NM | Died (5) & survived (2) |
8–15 [23] | NM | Primary brain tumour (8) | Dexa ↓ over a median of 12.5 days, range (1–63 days) | Fever (4), nonproductive cough (4), productive cough (2), dyspnoea (7), chest pain (4); CXR: bilateral infiltrates (3), diffuse infiltrates (3), focal infiltrates (1), clear (1) | Died (3) & survived (5) |
16 [24] | M/55 | Primary brain tumour (glioblastoma multiforme) | Dexa ↓ from 16 mg qd to 2 mg qd over 8 weeks | Intermittent fever, nonproductive cough, progressive dyspnoea; CXR: bilateral interstitial infiltrates; PaO2 (RA): 51 mmHg | Treated with intravenous cotrimoxazole; survived |
17 [24] | F/74 | Primary brain tumour (meningioma) | Dexa ↓ from 12 mg qd to 4 mg qd over 2 weeks | Intermittent fever, nonproductive cough; CXR: bilateral interstitial infiltrates; PaO2 (RA): 45 mmHg | Treated with intravenous cotrimoxazole; survived |
18 [24] | M/50 | Primary brain tumour (astrocytoma) | Dexa ↓ from 16 mg qd to 1 mg qd over 8 weeks | Fever, nonproductive cough, dyspnoea; CXR: bilateral interstitial infiltrates; PaO2 (RA): 73 mmHg | Treated with intravenous cotrimoxazole; mechanical ventilation; survived |
19 [24] | M/75 | Primary brain tumour (glioblastoma multiforme) | Dexa ↓ from 16 mg qd to 4 mg qd over 6 weeks | Fever, nonproductive cough, bloody diarrhoea; CXR: clear; PaO2 (RA): 89 mmHg | Treated with intravenous cotrimoxazole; survived |
20 [25] | M/24 | ACTH- producing metastatic bronchial carcinoid | Serum cortisol ↓ from 138 pg/ml to 18 pg/ml over 54 days | Fever, nonproductive cough, weakness, sweats; CXR: bilateral fluffy infiltrates; PaO2 (RA): 40 mmHg | Treated with intravenous cotrimoxazole; mechanical ventilation; died of malignancy |
21 [26] | F/38 | Endogenous Cushing's syndrome | Metyrapone 750 mg qd added 1 day before symptoms onset | Productive cough, dyspnoea; CXR: right lower upper lobe infiltrates; PaO2 (RA):31 mmHg | Treated with intravenous cotrimoxazole; mechanical ventilation; died |
22–28 [32] | M/F 4:3 Mean (SD) age 53.1 (13.6) | ITP (2), GN (2), bullous pemphigoid (1), endogenous Cushing's syndrome (1), and renal transplantation (1) | Reduction of steroid but details of tailing regimen was not mentioned | An upsurge of lymphocyte counts from the reduction of immunosuppression (median 300/μL, range 290 to 740/μL) to the onset of IRD (median 1500/μL, range 600 to 5620/μL) | Treated with steroid as anti-PJP therapy in 7 (100%); mechanical ventilation in 6 (85.7%), died in 3 (42.9%) |
29 | M/33 (Our patient) | Systemic lupus erythematosus/dermato-myositis overlapping syndrome | P ↓ from 45 mg to 15 mg over 4 days | Fever, dyspnoea; CXR: increased perihilar infilitrates; lymphocyte count increased from 600 to 1300/μL | Treated with intravenous cotrimoxazole and steroid; survived |
Note. Aza, azathioprine; CXR, chest radiograph; Dexa, dexamethasone; IRD, immunorestitution disease; ITP, immune thrombocytopenia purpura; IS, immunosuppression; GN, glomerulonephritis; P, prednisolone; PCP, Pneumocystis jiroveci pneumonia; RA, room air.