SARS-CoV-2 infection in systemic amyloidosis: the International Society of Amyloidosis? survey.

MARIA LOURDES POSADAS MARTINEZ

Simposio; XVIIIth International Symposium on Amyloidosis; 2022

Contribution ID : 373Track / Type : ISA 2022 Abstract SubmissionFormat : Oral PresentationTitle : SARS-CoV-2 infection in systemic amyloidosis: the International Society of Amyloidosis? surveyAuthor(s) : Milani, Paolo; Sanchorawala, Vaishali; Schonland, Stefan; Dispenzieri, Angela; Jimenez-Zepeda, Victor H; Posadas-Martinez, Maria; Pettine, Loredana; Gonzalez-Calle, Veronica; Maurer, Mathew; Lecumberri, Ramon; D'Souza, Anita; Cibeira, M. Teresa; Riva, Eloisa; Pena, Camila; Oubari, Sara; Modena...SARS-CoV-2 infection in systemic amyloidosis: the International Society of Amyloidosis? survey.MILANI, PAOLO1, SANCHORAWALA, VAISHALI2, SCHÖNLAND, STEFAN3, DISPENZIERI, ANGELA4, JIMENEZ-ZEPEDA, VICTOR H5, POSADAS-MARTINEZ, MARIA6, PETTINE, LOREDANA7, GONZALEZ CALLE , VERONICA8, MAURER, MATHEW9, LECUMBERRI, RAMON10, D?SOUZA, ANITA11, CIBEIRA, M. TERESA12, RIVA, ELOISA13,PEÑA, CAMILA14, OUBARI, SARA15, MODENA, MARIA GRAZIA16, HEGENBART, UTE3, MUSSINELLI, ROBERTA1, SAITH, SUNIL9, OBICI, LAURA1, MENDELSON, LISA2, MERLINI, GIAMPAOLO1, PALLADINI, GIOVANNI1.1Amyloidosis Research and Treatment Center, Foundation "Instituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Policlinico San Matteo", Department of Molecular Medicine, University of Pavia, Italy 2BAmyloidosis Center, Boston University School of Medicine and Boston Medical Centerr, Boston MA, USA3Medical Department V, Amyloidosis Center Heidelberg, University of Heidelberg, Germany4Mayo Clinic, Rochester (Minnesota), USA5Tom Baker Cancer Center, Department of Hematology, University of Calgary, Calgary, Canada.6Hospital italiano de Buenos Aires, Buonos Aires, Argentina7Fondazione IRCCS Policlinico di Milano, Milano, Italia8University Hospital of Salamanca, Salamanca, Spain9Columbia University, Ney York, USA10Clínica Universidad de Navarra, Pamplona, Spain11Medical College of Wisconsin, Milwaukee (WI), USA12Hospital Clinic of Barcelona, IDIBAPS, Barcelona, Spain 13Hospital Britanico, Montevideo, Uruguay14Hospital del Salvador, Santiago, Chile15University Hospital Essen, Essen, Germany16Università di Modena, Modena ItalyBackground: Systemic amyloidosis causes multi-organ dysfunction and patients can be at higher risk of complications and death due to SARS-CoV-2 infection. The ISA Board called for an international data collection on patients with amyloidosis and COVID-19 in April 2020. Objective: to report the data of the ISA survey on patients with systemic amyloidosis and COVID-19 from 4/2020 to 12/2021.Materials & Methods: All members of the ISA were invited. All patients suffering from amyloidosis who experienced a SARS-CoV-2 infection were eligible. Data collection started before vaccines were available and therefore vaccine status was not collected. However, to account the effect of vaccination we compared data of patients enrolled before and after 12/2020. This might also reflect improvement in supportive care with the increasing experience in managing the infection. Sixteen institutions contributed to the project. At the data lock of December 1, 2021, 200 patients were collected. The distribution of diagnoses were systemic AL, 61%; transthyretin amyloidosis (ATTR), 31% [ATTRwt 43 (21%) and ATTRv 20 (10%)]; localized 4.5% systemic AA, 2%; and Apolipoprotein A1 amyloidosis,1.5%]. We focused our analysis on patients with systemic AL and ATTR and main clinical data are reported in the Table.Results: Among ATTR patients, 48% were hospitalized, 8 of whom required ventilation, and an additional 12 (22%) were treated with supplementary oxygen. 54% of ATTR patients received pharmacological therapy. Among patients with AL amyloidosis, 44% were hospitalized, and 12% required ventilation, and an additional 22% were treated with oxygen. Forty-two percent of AL patients received pharmacological therapy for COVID-19. Acute distress respiratory syndrome was reported in 11 (17%) patients with ATTR and 18 (15%) with AL. Twelve of the 18 AL patients with ARDS (66%) were on active chemotherapy. The infection was fatal in 25/184 (14%) cases: ATTR, 9 (14%); and AL,16 (13%). Recovery with sequelae rates were: 3% for ATTR and 5% for AL. Fifteen patients with ARDS survived. All ATTR patients who died had heart involvement and at least one comorbidity. Seven of the 14 AL patients who died were on active chemotherapy at the time of SARS-CoV2 infection. Two patients with AL kidney involvement recovered from COVID-19 but developed subsequent worsening of renal function, requiring dialysis in one case. No differences were seen in the number of patients who had a severe presentation (pneumonia or ARDS) and in death rate between 2020 and 2021. Summary and Conclusions: participating enters are encouraged to report mild and asymptomatic patients yet we cannot exclude a referral bias. Severity and outcome of COVID-19 in patients with systemic amyloidosis is comparable to that of other frail patients. For instance, mortality rate is lower than in multiple myeloma (33%)1 and it is in the lower range of hematological malignancies in general (range from 13 to 39%)2. Being on active chemotherapy for AL amyloidosis was associated with worst presentation of the SARS-CoV2 infection. Table. Characteristics & outcomes of 184 patients with systemic amyloidosis and SARS-CoV2 infectionVariableAL, n=121 N (%) ? median (IQR)ATTR, n=63N (%) ? median (IQR)Diagnosis year, 2020 /2021, n75 / 4646 / 17Age, years64 (62, 66)75 (70, 79)Male sex68 (56)52 (82)Organ involvementHeart/kidneyLiver/Soft tissuePNS/ANS/>2 organs73 (60) / 80 (66)13 (11) / 20 (16)11 (9) / 9 (7) / 62 (51)54 (85) / 6 (9)1 (1) / 8 (12)21 (33) / 8 (12) / 8 (12)ComorbiditiesHypertension / Ischemic heart diseaseDiabetes / COPD48 (39) / 13 (11)11 (9) / 7 (6)30 (48) / 17 (27)9 (14) / 4 (6)On active treatment48 (39)35 (55)Symptoms of COVID-19Fever / CoughAnosmia / AgeusiaPneumonia / ARDS68 (56) / 59 (49)19 (16)/19 (16)55 (45) / 18 (14)38 (60) / 35 (55)5 (8) / 6 (9)38 (60) / 11 (17)Outcomes Hospitalization53 (44)30 (48)Invasive/non-invasive ventilation7 (6) / 7 (6) 1 (1) / 7 (11)Supplementary O227 (22)12 (19)Steroid Rx30 (25)16 (25)Antiviral Rx14 (11)10 (16)Anticoagulation Rx18 (15)9 (14)PNS, peripheral nervous system, ANS, autonomic nervous system, COPD, chronic obstructive pulmonary disease, ARDS, acute respiratory distress syndrome.References1.Chari et al Blood, 136(26):3033-3040, 20202.Pagano et al. J Hematol Oncol 14;14(1):168, 2021