Lymphocytic interstitial pneumonitis (LIP) in Sjögren's syndrome: A case report and a review of the literature

Vrishali Dalvi, Emilio Gonzalez, Louis Lovett

Research output: Contribution to journalArticle

24 Citations (Scopus)

Abstract

Sjögren's disease is one of the most common rheumatological diseases and can present with a variety of extra-glandular manifestations. Lymphocytic Interstitial Pneumonitis (LIP) is the most common lung pathology in these patients. It is important to know and recognize this condition because it is potentially treatable. It is also frequently misdiagnosed and treated as infectious pneumonia multiple times before the correct diagnosis is made. It is a benign lymphoproliferative disorder characterized histologically by interstitial infiltration with polyclonal lymphocytes and plasma cells. High-resolution CT scan of the lungs shows extensive areas of ground-glass attenuation and interlobular septal thickening with scattered thin-walled cysts. An open-lung biopsy is the best method of diagnosing this condition, as less invasive techniques do not provide an adequate tissue specimen. LIP occurs in a wide variety of settings such as autoimmune disease, HIV disease, and as an adverse reaction to some medications; it is, therefore, considered to be a nonspecific response to many stimuli. The treatment usually consists of corticosteroids and other immunosuppressants, though there have been no controlled trials to date. Establishment of a registry may help better evaluate and treat this disease. We present the case of a patient who was diagnosed with LIP secondary to Sjögren's syndrome and also review the literature available.

Original languageEnglish (US)
Pages (from-to)1339-1343
Number of pages5
JournalClinical Rheumatology
Volume26
Issue number8
DOIs
StatePublished - Aug 2007

Fingerprint

Interstitial Lung Diseases
Lung
Lymphoproliferative Disorders
Immunosuppressive Agents
Plasma Cells
Diagnostic Errors
Autoimmune Diseases
Glass
Registries
Cysts
Pneumonia
Adrenal Cortex Hormones
HIV
Lymphocytes
Pathology
Biopsy
Therapeutics

Keywords

  • Lymphocytic interstitial pneumonitis
  • Lymphoproliferative disorder
  • Sjögren's syndrome

ASJC Scopus subject areas

  • Rheumatology
  • Immunology

Cite this

Lymphocytic interstitial pneumonitis (LIP) in Sjögren's syndrome : A case report and a review of the literature. / Dalvi, Vrishali; Gonzalez, Emilio; Lovett, Louis.

In: Clinical Rheumatology, Vol. 26, No. 8, 08.2007, p. 1339-1343.

Research output: Contribution to journalArticle

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