Systemic Sclerosis (SSc) After COVID-19: A Case Report

Abstract

Since the start of the global pandemic caused by coronavirus disease 2019 (COVID-19), there have been numerous reports of autoimmune and rheumatological disorders developing after infection with SARS-CoV-2. To date, there has been only one reported case of systemic sclerosis (SSc) developing after SARS-CoV-2 infection. Here, we present another case of SSc developing after infection with SARS-CoV-2.A 48-year-old female with past medical history of anxiety and depression presented to the rheumatology clinic after being referred for further evaluation of abnormal labs, Raynaud's phenomenon, and other concerning symptoms. Shortly after hospitalization for COVID-19 pneumonia, she began experiencing symptoms that included fatigue, xerostomia, dysphagia, bilateral lower extremity weakness, dyspnea with exertion, unintentional weight loss, and diffuse skin hyperpigmentation. Labs ordered shortly before presentation were significant for antinuclear antibody (ANA) titer > 1:1280. Physical exam was remarkable for puffy fingers, sclerodactyly of the fingers, diffuse skin hyperpigmentation, and abnormal nailfold capillaries. Anti-RNA polymerase III, anti-Scl-70, anti-centromere, anti-SSA, anti-SSB, anti-Smith, and anti-Smith/RNP antibodies were all negative. BNP, aldolase, and serum myoglobin levels were within normal limits while creatine phosphokinase level was slightly decreased. Pulmonary function testing showed reduced diffusion capacity with normal lung mechanics and volumes. High-resolution CT scan of the chest showed interstitial lung disease, with findings suggestive of nonspecific interstitial pneumonia. Transthoracic echocardiogram showed mild elevation of right ventricular systolic pressure, but pulmonary hypertension was not found on right heart catheterization. Esophagogastroduodenoscopy (EGD) with biopsy performed for evaluation of esophageal dysphagia showed sliding hiatal hernia, irregular Z-line, and gastric hyperemia. Biopsy of the distal esophagus was consistent with Barrett's esophagus. The patient was diagnosed with SSc according to the 2013 American College of Rheumatology/European League Against Rheumatism (ACR-EULAR) classification criteria for SSc. She is currently being treated with mycophenolate mofetil, amlodipine, methotrexate, and prednisone.

A systematic review of the incidence, management and prognosis of new-onset autoimmune connective tissue diseases after COVID-19

“…Mild thoracic, abdominal, and pelvic lymphadenopathy, non-specific. Mild mesenteric stranding may be secondary to mesenteric panniculitis, with possible pancreatitis Proteinuria Holzer et al 2022 [ 19 ] 14 – < 5 – – CK 19,647 Positive ANA: 1:640 Positive anti-NXP2 MRI muscle: Bilateral myositis of muscles of the pelvic hip girdle and thighs Giuggioli et al 2022 [ 41 ] 28 – 65 – – Positive ANA, anticentromere pattern Nailfold capillaroscopy: “early scleroderma pattern” Chandra and Kahaleh 2022 [ 42 ] – – – – Positive ANA > 1:1280 HRCT chest: interstitial lung disease, findings suggestive of non-specific interstitial pneumonia Bouchard Marmen et al 2022 [ 43 ] 28 – 61 – – CK 7696, Positive ANA and anti-Jo-1 CT thorax: missed opacities with sub segmental consolidation MRI: oedema of the gluteal and thigh muscles consistent with myositis Blum et al 2022 [ 44 ] 91 – – – – Positive ANA Positive anti-RNP and ani-SSA antibodies CT chest: ground-glass opacities possibility indicating interstitial lung disease Assar et al 2022 [ 32 ] 18 53 …”

“…Subsequent hydroxychloroquine 400 mg/day and prednisolone 1 mg/kg/day (dose not specified) Yes Yes Survived Kazzi et al 2022 [ 31 ] Low None SLE No Antibiotics, corticosteroids and MMF 1500 mg twice daily with resolution. Subsequent hydroxychloroquine (dose unspecified) No Yes Survived Holzer et al 2022 [ 19 ] Unknown Unknown Dermatomyositis Unknown Corticosteroids, IVIG, MMF, ciclosporin A, tofacitinib, rituximab No Yes Survived Giuggioli et al 2022 [ 41 ] Unknown Azithromycin for 5 days; hydroxychloroquine 400 mg twice a day for 1 day and then 200 mg every 12 h for 5 days Raynaud's and systemic sclerosis No Nifedipine for Raynaud’s No Yes Survived Chandra & Kahaleh 2022 [ 42 ] Unknown Unknown Systemic Sclerosis No MMF 1500 mg twice daily, amlodipine 5 mg daily, methotrexate 12.5 mg once weekly, prednisone 5 mg twice daily No No Survived Bouchard Marmen et al 2022 [ 43 ] Unknown Unknown Anti-synthetase syndrome Unknown Pulsed IV methylprednisolone, then oral prednisolone, cyclophosphamide, rituximab, IVIG Yes Yes Survived Blum et al 2022 [ 44 …”

Summarizing Evidence of Associations of COVID‐19 With a Future Diagnosis of Inflammatory Rheumatic and Musculoskeletal Diseases: A Rapid Review

“…There were 26 studies that proposed mechanisms related to the initiation of an autoimmune process. 6,10,15,[22][23][24]31,33,38,39,45,48,49,55,56,58,65,67,[69][70][71][73][74][75][76][77] Several studies proposed multiple possible autoimmune mechanisms. 10,24,31,39,45,48,55,73,77 Proposed mechanisms included molecular mimicry, 10,15,23,24,31,33,39,45,48,58,65,69,[73][74][75][76]…”

Long COVID in autoimmune rheumatic diseases

“…The treatment with RTX lead to B-cell depletion in patients with SSc, increasing the risk of severe manifestations of SARS-CoV-2 infection [ 60 ]. A number of case reports revealed an association of Long COVID with autoantibody production and new-onset SSc in previously infected patients without history of autoimmune diseases [ 61 , 62 ].…”