ABSTRACT
We present a case with significant diagnostic and therapeutic challenges;a 57 year old female with history of bi-ventricular failure status post Left Ventricular Assisted Device (LVAD) in 2020 and Rheumatoid Arthritis (RA) who presented with Shortness of breath and refractory hypoxemia, Computerized tomography(CT) showed dense ground glass opacity and superimposed traction bronchiectasis which was not present in a prior CT. The management included high FiO2 therapy High flow nasal cannula (HFNC), initial heart failure measures with diuresis and antibiotics treatment were attempted, the clinical and radiological diagnosis of acute exacerbation (AE) of connective tissue disease associated interstitial lung disease (CTD-ILD) with progressive fibrosing phenotype was made, The decision of therapeutic pulse corticosteroids with Rituximab was based on the degree of severity of acute exacerbation, following treatment, the course of the disease was reversed with complete oxygen weaning with impressive clinical and radiological response. The case is considered puzzling from multiple aspects, first the complex comorbidities, LVAD placement made heart failure the main differential diagnosis (DD), the absence of interstitial infiltrate in the old CT made the initial diagnosis of AE of ILD less likely, other DD as Covid-19 pneumonia, pulmonary embolism, bacterial pneumonia were worked up. Another major dilemma was the optimal management of the life threatening hypoxemia in the setting of new CT findings. Connective tissue disease associated ILD is a diffuse parenchymal lung disease characterized by both inflammation and fibrosis. The progressive fibrosing phenotype carries poor prognosis, even worse is the prognosis of AE that characterized by marked deterioration and alveolar abnormalities with high mortality. There is extremely limited data of optimal treatment of AE and lack of blinded randomized controlled trials. Management is mainly supportive care, oxygen therapy is considered the cornerstone, otherwise no formal therapeutic strategy. The potential reported therapies included corticosteroids, immunosuppressant, anticoagulants, antibodies targeted therapy with no conclusive evidence. Although corticosteroids were described based on anecdotal evidence but recently published case series described novel combination of treating AE with combination of pulse steroid, Rituximab and plasma exchange resulting in promising outcome. In our case we followed the regimen of pulse steroids in combination with Rituximab which lead to satisfactory results in short interval. The rational of steroids use for its potent anti-inflammatory properties and since AE is linked to autoimmune antibody-driven inflammation, treatment with Rituximab causes Bcell depletion, the impressive clinical outcome of our patient signals a promising therapeutic potentials in treating fatal AE. (Figure Presented).