The impact of Covid-19 on rare and complex connective tissue and musculoskeletal diseases and conditions: state of the art and future perspectives

Prof. Marta Mosca, ERN ReCONNET Coordinator

Azienda Ospedaliero Universitaria Pisana

Summary

2

Clinical issues

Impact on clinical management

Impact on the organisation of care

ERN ReCONNET initiatives on COVID-19

Lesson learned from the pandemic

Diseases covered by ERN ReCONNET

3

Systemic Sclerosis

MTCD

IIM

UCTD

APS

SLE

Sjögren Syndrome

Ehlers Danlos Syndromes

IgG4 related diseases

Relapsing Polychondritis

Rare connective

tissue diseases

Rare hereditary connective

tissue syndromes

Complex connective

tissue diseases

rCTDS

Chronic conditions

Wide variability of clinical manifestations

Development of damage and comorbidities

High impact on the quality of life of the patient

Diagnostic delay

Complex treatments, immunosuppressiveand

biotechnological therapies

4

Rare connective

tissue diseases

Rare hereditary connective

tissue syndromes

Complex connective

tissue diseases

Summary

5

Clinical issues

Impact on clinical management

Impact on the organisation of care

ERN ReCONNET initiatives on COVID-19

Lesson learned from the pandemic

Clinical issues: COVID-19 and rCTDs

6

• Do rCTDs patients have a higher risk of contracting

SARS-CoV-2?

• Do rCTDs patients have a worse prognosis in case of

SARS-CoV-2 infection?

• Is immunosuppressive therapy an additional risk for

rCTDs patients?

Do rCTDs patients have a higher risk of contracting SARS-CoV-2?

o Phone surveys

• 916 patients (397 SLE, 182 AAV, 176 SSc, 111 RA, 50 IIM), 65 (7.1%) underwent SARS-CoV-2 nasopharyngeal swab, 2 (0.21%) tested positive, a proportion similar to that observed in the general population of the Veneto region.

Zen et al. J Autoimmunity 2020

▰ Phone surveys

▻ Our experience: 1006 patients

▻ 12 patients (1.2%) resulted SARS-CoV2 +; 5 patients required hospitalization; 3 asymptomatic patients

▻ Among SLE patients 3.4% positive

▻ Among Sjögren patients 1.6%

▻ Among APS patients 6.5%

▻ Among IIM patients 2.2%

▰ SARS-CoV-2 infection is more common among CTDs patients than inthe general italian population (1.2% vs 0.4%), with higher incidenceamong SLE patients

Personal unpublished data

Do rCTDs patients have a higher risk of contracting SARS-CoV-2?

COVID-19 and rheumatic diseases: International Registries

-COVID-19 Global Alliance Registry

-Eular COVID-19 database

-Italian Society of Rheumatology Registry

Outcomes

▰ 600 cases from 40 countries

▰ Hospitalised patients 277 (46%)

▰ Deceased 55 (9%)

Do rCTDs patients have a worse prognosis in case of SARS-CoV-2 infection?

Preliminary data from the registries suggests that rheumatic patients treated with immunosuppressant drugs seem not to have a higher risk of hospitalization when infected by the virus.

The risk of a poor outcome of COVID -19 in patients with rCTDsseems to be associated with the same risk factors as the general population

Apparently, the disease per se is not an adjunctive risk factor for infection or poor outcomes

Summary

18

Clinical issues

Impact on clinical management

Impact on the organisation of care

ERN ReCONNET initiatives on COVID-19

Lesson learned from the pandemic

ERN ReCONNET Survey on COVID-19 challenges

70

57

48

39

35

30

26

0 10 20 30 40 50 60 70 80

Strong limitations of the diagnostic and routinely monitoring of rCTDs patients(e.g. diagnostic process not completed)

Impossibility of performing face to face evaluations

Switch infusion therapies to subcutaneous therapies (e.g. Tocilizumab)

Management of risk of infection when starting a new immunosuppressivetherapies (e.g. pre-biological agents screening)

Tensions in the supply of some rCTDs drugs (e.g. Hydroxychloroquine)

Relapse of the disease(s) due to patients suspending therapies autonomously (e.g.for fear of being more exposed to the virus contagion)

Management of risk of infection in patients taking immunosuppressive therapies

Due to the SARS-Cov2 pandemic, many healthcare providers encountered several clinical challenges. Among the following options, please select up to 5 clinical

challenges that you have experienced in your clinic.

%

%

%

%

%

%

%

Preventive measures

The same preventive measures advised to the general population should be advised to rCTDs patients

Assessment of specific risks: based on steroid dose, IS therapy, comorbidities

Preliminary guidance from Scientific Societies

23

Landewé RB et al, EULAR provisional recommendations for the management of

rheumatic and musculoskeletal diseases in the context of SARS-CoV-2, Ann Rheum Dis,

June 2020.

Mikuls TR et al, ACR guidance for the management of adult patients with rheumatic

disease during the COVID-19 pandemic. Arthritis Rheumatol, June 2020

In patients with a contact with COVID 19

▰ Continue chronic steroids, hydroxychloroquine

▰ Consider continuing SSZ

▰ Patients with contact with a positive individual: continue treatment/consider temporary stopping IS therapy

Documented or Presumptive COVID-19 Regardless of COVID-19 severity

▰ HCQ/CQ may be continued

▰ SSZ, MTX, LEF, immunosuppressants, non-IL-6 biologics,and JAK inhibitors should be stopped or held.

▰ In select circumstances, as part of a shared decision-making process, IL-6 receptor inhibitors may becontinued.

Summary

28

Clinical issues

Impact on clinical management

Impact on the organisation of care

ERN ReCONNET initiatives on COVID-19

Lesson learned from the pandemic

ERN ReCONNET Survey on COVID-19 challenges

%

%

%

%

%

%

%

%

%

Impact on the organisation of care

Joint efforts needed to reorganise the hospital services to ensure the

adequate number of resources needed for COVID-19 units

Summary

31

Clinical issues

Impact on clinical management

Impact on the organisation of care

ERN ReCONNET initiatives on COVID-19

Lesson learned from the pandemic

The initiatives ERN ReCONNET on COVID-19

COVID-19 section on ERN ReCONNET website

Endorsement of Fai2r recommendations

ePAGs translating Fai2r documents in 16 languages

Webinar ISS CNMR COVID-19 and rCTDs (26th May)

Webinar COVID-19 ERN ReCONNET (1st June)

Webinar COVID-19 European Commission (15th June)

Publication on COVID-19 and rCTDs and survey

https://reconnet.ern-net.eu/update-on-covid-19-outbreak/

Summary

33

Clinical issues

Impact on clinical management

Impact on the organisation of care

ERN ReCONNET initiatives on COVID-19

Lesson learned from the pandemic

Lesson learned from the pandemic

Usage of eHealth and telemedicine

Developement of emergency strategy plan for rCTDs in case of new health emergency

Important to ensure the continuity of adequate care to rCTDs patients

ERNs to contribute in bringing the needs and priorities of the EU rCTDs communties

Contacts

ERN ReCONNET website: http://reconnet.ern-net.eu/

ERN ReCONNET Twitter: ern_reconnet

ERN ReCONNET YouTube channel: ERN ReCONNET

Subscribe to our Newsletter

36

Prof.ssa Marta Moscamarta.mosca@med.unipi.itern.reconnet@ao-pisa.toscana.it