Atypical Sjögrenʼs Syndrome Initially Presenting as Lymphocytic Interstitial Pneumonitis followed by Immune Thrombocytopenia

Background. Sjögrenʼs syndrome is an autoimmune disease characterized primarily by decreased exocrine gland function leading to eye and mouth dryness. Extraglandular manifestations occur less frequently. Case Report. A 74-year-old man with hypertension was admitted with productive cough and fever. O...

Full description

Saved in:
Bibliographic Details
Main Authors: Maham Mehmood, Abhishrut Jog, Masooma Niazi, Arlene Tieng, Giovanni Franchin
Format: Article
Language:English
Published: Wiley 2021-01-01
Series:Case Reports in Rheumatology
Online Access:http://dx.doi.org/10.1155/2021/6681590
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1832547631455272960
author Maham Mehmood
Abhishrut Jog
Masooma Niazi
Arlene Tieng
Giovanni Franchin
author_facet Maham Mehmood
Abhishrut Jog
Masooma Niazi
Arlene Tieng
Giovanni Franchin
author_sort Maham Mehmood
collection DOAJ
description Background. Sjögrenʼs syndrome is an autoimmune disease characterized primarily by decreased exocrine gland function leading to eye and mouth dryness. Extraglandular manifestations occur less frequently. Case Report. A 74-year-old man with hypertension was admitted with productive cough and fever. On physical examination, he had bilateral lower lung decreased breath sounds. A chest radiograph showed bibasilar patchy infiltrate. Laboratory studies revealed hemoglobin of 11.9 g/dL, white blood cell count of 16,000/uL, and platelet count of 250,000/uL. Empiric antibiotic therapy was begun for suspected community acquired pneumonia, and then he was discharged home. However, his cough recurred. Chest computed tomography demonstrated adenopathy throughout the mediastinum and multiple ill-defined patchy groundglass opacities with a lower lobe prominence. He underwent a transbronchial biopsy to rule out malignancy; however, it showed lymphocytic interstitial pneumonitis. Antinuclear antibody was 1 : 80 homogeneous, and anti-SSA antibody was 6.3 AI (normal <1.0 AI). The patient was treated with prednisone 20 mg/day with marked improvement in his symptoms. Repeat chest computed tomography showed decreased groundglass opacities and decreased mediastinal lymph nodes. After more than a year, he was readmitted due to petechiae on his buccal mucosa and a platelet count of 2000/μL. The patient was started on prednisone 80 mg/d and intravenous immunoglobulin 80 g/d for 2 consecutive days. The platelet count eventually increased to 244,000/μL. Conclusion. We report a rare presentation of Sjogrenʼs syndrome manifesting as acute lymphocytic interstitial pneumonitis and followed by immune thrombocytopenia. Both extraglandular manifestations responded well to corticosteroid therapy.
format Article
id doaj-art-482b4b6220b04851be176aa5170d7dba
institution Kabale University
issn 2090-6889
2090-6897
language English
publishDate 2021-01-01
publisher Wiley
record_format Article
series Case Reports in Rheumatology
spelling doaj-art-482b4b6220b04851be176aa5170d7dba2025-02-03T06:43:58ZengWileyCase Reports in Rheumatology2090-68892090-68972021-01-01202110.1155/2021/66815906681590Atypical Sjögrenʼs Syndrome Initially Presenting as Lymphocytic Interstitial Pneumonitis followed by Immune ThrombocytopeniaMaham Mehmood0Abhishrut Jog1Masooma Niazi2Arlene Tieng3Giovanni Franchin4Department of Internal Medicine, BronxCare Health System, Bronx, NY, USADepartment of Internal Medicine, BronxCare Health System, Bronx, NY, USADepartment of Pathology, BronxCare Health System, Bronx, NY, USADepartment of Internal Medicine, BronxCare Health System, Bronx, NY, USADepartment of Internal Medicine, BronxCare Health System, Bronx, NY, USABackground. Sjögrenʼs syndrome is an autoimmune disease characterized primarily by decreased exocrine gland function leading to eye and mouth dryness. Extraglandular manifestations occur less frequently. Case Report. A 74-year-old man with hypertension was admitted with productive cough and fever. On physical examination, he had bilateral lower lung decreased breath sounds. A chest radiograph showed bibasilar patchy infiltrate. Laboratory studies revealed hemoglobin of 11.9 g/dL, white blood cell count of 16,000/uL, and platelet count of 250,000/uL. Empiric antibiotic therapy was begun for suspected community acquired pneumonia, and then he was discharged home. However, his cough recurred. Chest computed tomography demonstrated adenopathy throughout the mediastinum and multiple ill-defined patchy groundglass opacities with a lower lobe prominence. He underwent a transbronchial biopsy to rule out malignancy; however, it showed lymphocytic interstitial pneumonitis. Antinuclear antibody was 1 : 80 homogeneous, and anti-SSA antibody was 6.3 AI (normal <1.0 AI). The patient was treated with prednisone 20 mg/day with marked improvement in his symptoms. Repeat chest computed tomography showed decreased groundglass opacities and decreased mediastinal lymph nodes. After more than a year, he was readmitted due to petechiae on his buccal mucosa and a platelet count of 2000/μL. The patient was started on prednisone 80 mg/d and intravenous immunoglobulin 80 g/d for 2 consecutive days. The platelet count eventually increased to 244,000/μL. Conclusion. We report a rare presentation of Sjogrenʼs syndrome manifesting as acute lymphocytic interstitial pneumonitis and followed by immune thrombocytopenia. Both extraglandular manifestations responded well to corticosteroid therapy.http://dx.doi.org/10.1155/2021/6681590
spellingShingle Maham Mehmood
Abhishrut Jog
Masooma Niazi
Arlene Tieng
Giovanni Franchin
Atypical Sjögrenʼs Syndrome Initially Presenting as Lymphocytic Interstitial Pneumonitis followed by Immune Thrombocytopenia
Case Reports in Rheumatology
title Atypical Sjögrenʼs Syndrome Initially Presenting as Lymphocytic Interstitial Pneumonitis followed by Immune Thrombocytopenia
title_full Atypical Sjögrenʼs Syndrome Initially Presenting as Lymphocytic Interstitial Pneumonitis followed by Immune Thrombocytopenia
title_fullStr Atypical Sjögrenʼs Syndrome Initially Presenting as Lymphocytic Interstitial Pneumonitis followed by Immune Thrombocytopenia
title_full_unstemmed Atypical Sjögrenʼs Syndrome Initially Presenting as Lymphocytic Interstitial Pneumonitis followed by Immune Thrombocytopenia
title_short Atypical Sjögrenʼs Syndrome Initially Presenting as Lymphocytic Interstitial Pneumonitis followed by Immune Thrombocytopenia
title_sort atypical sjogren s syndrome initially presenting as lymphocytic interstitial pneumonitis followed by immune thrombocytopenia
url http://dx.doi.org/10.1155/2021/6681590
work_keys_str_mv AT mahammehmood atypicalsjogrenʼssyndromeinitiallypresentingaslymphocyticinterstitialpneumonitisfollowedbyimmunethrombocytopenia
AT abhishrutjog atypicalsjogrenʼssyndromeinitiallypresentingaslymphocyticinterstitialpneumonitisfollowedbyimmunethrombocytopenia
AT masoomaniazi atypicalsjogrenʼssyndromeinitiallypresentingaslymphocyticinterstitialpneumonitisfollowedbyimmunethrombocytopenia
AT arlenetieng atypicalsjogrenʼssyndromeinitiallypresentingaslymphocyticinterstitialpneumonitisfollowedbyimmunethrombocytopenia
AT giovannifranchin atypicalsjogrenʼssyndromeinitiallypresentingaslymphocyticinterstitialpneumonitisfollowedbyimmunethrombocytopenia