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  • Title: A Rare Case of Primary Sjogren's Syndrome with Idiopathic Pulmonary Fibrosis with Variable Presentations: A case report.
    Author: Yadav A, Nepali RB, Alam AMJ.
    Journal: Mymensingh Med J; 2024 Jan; 33(1):298-302. PubMed ID: 38163807.
    Abstract:
    We diagnosed and treated a case of Primary SjoGren's Syndrome with Idiopathic Pulmonary Fibrosis (IPF) in a 65 years old woman who presented with dyspnoea and multiple joint pains for 5 years and remained undiagnosed. She had variable presentation and was initially established as a case of mixed connective tissue disease which consists of Systemic Lupus Erythematosus (SLE), Systemic Sclerosis and Dermatomyositis. She complained of xerostomia, xerophthalmia, difficulty in opening mouth, progressive dysphagia with solid foods and raynaud's phenomenon. In addition to this she noticed photosensitive rash, oral ulcers and difficulty in raising arms above head especially while combing hair. Examination revealed bi basal fine end inspiratory crepitations unaltered while coughing, bed side 6 minutes walking test showed exertional desaturation of SpO2 from 92.0% to 84.0%. Grade 2 tenderness was noted in wrists, knees, elbows and small joints of hands and feet except DIP. However, no oral lesions or dental carries were found. Unstimulated salivary flow rate was 1.0 ml in 15 minutes and sublingual salivary pool was significantly reduced. Schirmer's test was positive. HRCT lung revealed reticulonodular shadowing, honey combing and traction bronchiectasis in basal segments of both lobes, suggestive of usual interstitial pneumonia in both lungs. Auto antibody tests revealed ANA, RA, anti CCP and anti ds DNA negative, CPK was 63U/L. ENA (Extractable Nuclear Antigen) profile demonstrated positive Anti SS- A antibody while it remained insignificant for anti SS-B, anti RNP, anti Sm antibody, anti Scl-7o, anti Jo-1. According to the American-European Consensus Criteria for SjoGren's Syndrome, it meets all the criteria to be diagnosed as Primary Sjogren's Syndrome. We finally diagnosed a case of Primary SjoGren's syndrome with IPF and the patient was treated with pirfenidone, prednisolone, artificial tears and vaccination against Haemophilus influenzae and Streptococcal pneumoniae. The 10 year survival rate for such patients is nearly 80.0%.
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