|LETTER TO EDITOR
|Ahead of print publication
A successful rescue therapy with plasma exchange for rapidly progressive interstitial lung disease associated antisynthetase syndrome
Roopa Tekkatte1, Pavan Yadav2, Shivraj Kumar Venkatachala2, Vinod Dibbur3
1 Department of Rheumatology, Aster RV Hospital, Bengaluru, Karnataka, India
2 Department of Pulmonary Medicine, Aster RV Hospital, Bengaluru, Karnataka, India
3 Department of Nephrology, Fortis Hospital, Bannerghatta Road, Bengaluru, Karnataka, India
|Date of Submission||22-Mar-2021|
|Date of Acceptance||15-Apr-2021|
Department of Rheumatology, Aster RV Hospital, JP Nagar, Bengaluru 560078
Source of Support: None, Conflict of Interest: None
|How to cite this URL:|
Tekkatte R, Yadav P, Venkatachala SK, Dibbur V. A successful rescue therapy with plasma exchange for rapidly progressive interstitial lung disease associated antisynthetase syndrome. Indian J Rheumatol [Epub ahead of print] [cited 2022 Oct 1]. Available from: https://www.indianjrheumatol.com/preprintarticle.asp?id=323369
Antisynthetase syndrome (ASS) is a chronic autoimmune condition that may include interstitial lung disease (ILD), myositis, arthritis, Raynaud's phenomenon, fever, and mechanic's hands. ILD associated with ASS is often rapidly progressive, and conventional treatment with glucocorticoids and immunosuppressants can be less effective. The use of therapeutic plasma exchange (TPE) in this condition is not validated. We report a case where a rescue therapy with TPE was successful in of rapidly progressing ILD associated with ASS.
A 23-year-old female presented, 2 months postpartum, with fever, exertional dyspnea, nonproductive cough, and skin rash. Clinical examination showed heliotrope sign [Figure 1], Gottron's papules over the hands, Shawl's sign, and V sign rash over the neck and upper back. There was no proximal muscle weakness or wasting. Auscultation of the lungs showed fine crepitations at both the lung bases. Vital observation showed pyrexia at 99°F and hypoxia with oxygen saturation of 88% on room air.
|Figure 1: (a) Heliotrope sign, (b) timeline of the case with diagnostic and therapeutic events|
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Initial investigations revealed elevated C-reactive protein of 71 mg/L (<5 mg/L), erythrocyte sedimentation rate of 40 mm/h (<20 mm/h), ferritin of 1485 ng/mL (6–137 ng/mL), lactate dehydrogenase of 445 U/L (81–234 U/L), serum glutamic oxaloacetic transaminase of 555 U/L (0–32 U/L), serum glutamic pyruvic transaminase of 206 U/L (0–33 U/L), alkaline phosphatase of 245 U/L (35–105 U/L), and gamma-glutamyl transferase of 248 U/L (<40 U/L). Creatine phosphokinase, full blood count, and renal function were normal. Chest X-ray showed bilateral lower and left mid zone infiltrates. Oxygen and broad-spectrum intravenous (IV) antibiotics were commenced. Ro52 and anti-PL7 antibodies were positive. A high-resolution computed tomography scan of the chest showed patchy areas of ground-glass opacities with areas of consolidation in both lower lobes. Pulmonary artery pressure was normal.
She was commenced on treatment for ASS with daily pulses of IV methylprednisolone 1 g and IV cyclophosphamide (CYC) at 15 mg/kg. Despite the treatment, her clinical condition deteriorated requiring mechanical ventilation. She also developed pneumomediastinum with subcutaneous emphysema. She was commenced on plasma exchange as a rescue therapy. Her clinical condition improved and was later able to wean off the mechanical ventilation. She completed six cycles of IV CYC, following which mycophenolate mofetil at a daily dose of 1.5 g per day was commenced. She has remained in remission at 12 months of follow-up.
According to the American Society for Apheresis 2019, TPE use is not validated in connective tissue diseases (CTD) or myositis. Dequeker et al. have reported TPE as a valuable adjunct therapy in acute exacerbation of CTD. Cozzi et al. reported three cases of patients with dermato/polymyositis (PM/DM) successfully treated with TPE during acute phase of the disease with inadequate response to conventional treatment. A retrospective evaluation of 18 patients with ILD associated with PM/DM by Ning et al. showed that 61% responded to treatment with TPE. In this study, subcutaneous/mediastinal emphysema and high serum ferritin levels were identified as poor prognostic factors. However, our patient had good outcome despite having similar poor prognostic factors. This case highlights that the TPE could be considered as a rescue treatment, especially when conventional treatment has failed, and can have favorable outcome even in the presence of poor prognostic factors.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that names and initials will not be published, and due efforts will be made to conceal patient identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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