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EP04?Is this scleroderma lung or COVID-19 lung?
#MMPMIDC7607361
Malik DS
; Younas DH
; Dhahir DLA
Rheumatol Adv Pract
2020[Oct]; 4
(Suppl 1
): ? PMIDC7607361
show ga
CASE REPORT - INTRODUCTION: This is a case of Pakistani female with limited
systemic sclerosis and associated mild interstitial lung disease. The lung
disease was complicated by SARS-COV-2 related pneumonitis in April 2020 and that
led to treatment challenges. She was previously seen in multiple private
hospitals and labelled as Rheumatoid arthritis. She was being treated with long
term steroids and Methotrexate. After her initial presentation to our
Rheumatology services, her diagnosis was correctly revised to Systemic Sclerosis
with phenotype of CREST. Her treatment was adjusted to Vasodilators and
Mycophenolate due to skin and Lung involvement. CASE REPORT - CASE DESCRIPTION:
This is a case of 40-year-old Pakistani female who had been having multiple joint
pains since 2010. She also experienced severe Raynaud?s. She presented to our
Rheumatology clinic in December 2018. Her symptoms included recurrent digital
ulcers, tight and tough skin at fingers and Raynaud?s worse during winter months.
Her examination confirmed peripheral cyanosis with multiple digital ulcers with
superimposed infection, marked sclerodactyly and calcinosis. She was started on
Vasodilator therapy including calcium channel blocker and PDE5 inhibitor due to
severity of ulceration. Infection was managed with prolonged course of
antimicrobial therapy. Her immunology showed positive anti nRNP/Sm.
Anti-centromere and anti Scl 70 were negative. Her condition fit description of
CREST (Calcinosis, RP, Oesophageal dysmotility, telangiectasia). Her management
included weaning off Methotrexate and reduction in the dose of corticosteroids.
In February 2019, Respiratory work up showed normal Chest radiograph, High
resolution CT chest showing no significant abnormality and FEV1 82%, FVC 86%, and
DLCO 77%. Her PASP was 25mmHg. Overall, her condition remained stable over the
course of next year. Her medication included Cellcept, low dose prednisolone,
hydroxychloroquine, and Sildenafil. More importantly, Digital ulcers have been
well controlled with combined vasodilator therapy. In April 2020, she developed
SARS-CoV-2 with mild respiratory symptoms and was admitted to a different
hospital. Fortunately, she responded well to ward based supportive and
symptomatic treatment with no need for respiratory support. Subsequently, she has
seen a different respiratory physician and had repeat imaging of chest which has
led to dilemma whether the ground glass opacities in both lungs is due to
scleroderma lung or COVID-19 related lung disease. She was given high dose
prednisolone by the respiratory physician which has been reduced in rheumatology
clinic. The new findings on chest imaging are sequelae of SARS-COV-2. CASE REPORT
- DISCUSSION: This case highlights few important points as below: 1. Systemic
sclerosis diagnosis was not made for many years even though she has had severe
digital ulcers for a long time. She was being managed as Rheumatoid arthritis.
Systemic sclerosis remains a difficult disease to diagnose and is still under
recognised. . 2. SARS-COV-2 related illness has not affected this patient
adversely despite the fact of being on long term maintenance prednisolone of
7.5mg daily dose and Cellcept 2gm. Her cellcept was temporarily stopped during
acute illness. . 3. We know that viral pneumonitis can present with typical
ground glass opacities in bilateral areas of lungs and differential diagnosis
does include connective tissue related lung disease but this lady had no
significant respiratory involvement prior to COVID-19 illness and follow up scan
will help to decide if this is disease progression or related to viral cause.
CASE REPORT - KEY LEARNING POINTS: There are multiple learning points in this
case: 1. Continuity of care under same primary team can avoid confusion related
to diagnosis and diagnosis related complications. This lady had none, or mild
subclinical lung involvement related to systemic sclerosis prior to contracting
COVID-19 illness. Her CT chest findings after the episode of SARS-COV-2 were
attributed to systemic sclerosis as she was seen by different respiratory team.
This continuity is not always possible, but MDT collaboration needs to be
improved across hospitals and across various departments. . 2. Systemic sclerosis
remains an under diagnosed and under recognized complex rheumatic disorder and
more primary care physicians need to be educated so they can appropriately refer
these cases to Rheumatology services. 3. Multi-disciplinary collaboration between
Rheumatology, Respiratory and other specialties is the key point to manage these
complex cases. . 4. This case also highlights an interesting observation that
presence of significant immune disorder and immunosuppressant medication does not
always equate to worse outcome if patient contracts SARS-COV-2. Supportive care,
appropriate observation, and temporary suspension of DMARD in such cases can
avoid any further complications.