Table 1.
Demographic, Clinical, and Functional Characteristics and Outcomes of Patients With Lymphangioleiomyomatosis Who Received a Diagnosis of COVID-19 (N = 91)
| Variable | Data |
|---|---|
| Demographic and clinical data | |
| Country | |
| United States | 23 (25.3) |
| Brazila | 20 (22) |
| Poland | 20 (22) |
| United Kingdom | 16 (17.5) |
| France | 6 (6.6) |
| Italy | 3 (3.3) |
| The Netherlands | 2 (2.2) |
| Japan | 1 (1.1) |
| Age, y | 47 ± 12 |
| Race or ethnicity | |
| White | 76 (83.5) |
| Black | 4 (4.4) |
| Hispanic | 8 (8.8) |
| Asian | 3 (3.3) |
| Subtype of lymphangioleiomyomatosis | |
| Sporadic | 77 (84.6) |
| Associated with TSC | 14 (15.4) |
| Time from diagnosis of lymphangioleiomyomatosis, mo | 14 (6-78) |
| BMI ≥ 30 kg/m2 | 21 (23.1) |
| Comorbidities | |
| Diabetes | 6 (6.6) |
| Cancer | 3 (3.3) |
| Chronic kidney disease | 6 (6.6) |
| Heart failure, coronary artery disease, or cardiomyopathy | 0 |
| Sickle cell disease | 1 (1.1) |
| Previous organ transplantation | 0 |
| Current pregnancy | 0 |
| Smoking (former or current) | 14 (15.4) |
| Use of mTOR inhibitors before COVID-19b | 47 (52.8) |
| Sirolimus | 44 (49.4) |
| Everolimus | 3 (3.4) |
| Dose of sirolimus, mg | 2 (1-2) |
| Dose of everolimus, mg | 5 (5-8.75) |
| Duration of mTOR inhibitor use, mo | 44 (12-70) |
| Pulmonary function tests | |
| FEV1, L | 2.13 ± 0.77 |
| FEV1, % predicted | 73 ± 23 |
| FVC, L | 3.05 ± 0.82 |
| FVC, % predicted | 88 ± 21 |
| Dlco, mL/min/mm Hg | 7.84 (5.71-17.00) |
| Dlco, % predicted | 62 ± 23 |
| COVID-19 diagnosis | |
| RT-PCR swab | 87 (95.6) |
| COVID-19 seroconversion and clinical manifestations | 4 (4.4) |
| Symptoms during COVID-19 | |
| Asthenia | 80 (87.9) |
| Fever | 64 (70.3) |
| Cough | 64 (70.3) |
| Anorexia | 48 (52.7) |
| Dyspnea | 43 (47.3) |
| Headache | 40 (44) |
| Anosmia | 38 (41.8) |
| Dysgeusia | 33 (36.3) |
| Diarrhea | 20 (22) |
| Otherc | 10 (11) |
| Hospital admission | |
| Hospital admission | 28 (30.8) |
| Duration of hospital stay, d | 11 ± 8 |
| Supplemental oxygen use | 27 (29.7) |
| Duration of supplemental oxygen use, d | 7 (5-14) |
| Duration of mechanical ventilation, d | 14 (6-22) |
| Suspension of mTOR inhibitors | 21 (44.7) |
| Outcomes | |
| Death | 1 (1.1) |
| New or increased supplemental oxygen | 10 (11) |
| Pulmonary embolism | 1 (1.1) |
| Pneumothorax | 1 (1.1) |
| Long COVID-19d | 20 (22) |
Data are presented as No. (%), mean ± SD, or median (interquartile range). Dlco = diffusing capacity of the lungs for carbon monoxide; mTOR = mechanistic target of rapamycin; RT-PCR = reverse-transcription polymerase chain reaction; TSC = tuberous sclerosis complex.
Partial information about six patients from Brazil was described in a previous report.5
Two patients were enrolled in a double-blind placebo-controlled trial of sirolimus vs placebo, and therefore were not included in this analysis.
Other symptoms include nausea, chest pain, night sweats, myalgia, sore throat, and dysphonia.
The most common manifestation of long COVID-19 was fatigue.