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This case explores the atypical discovery of lymphocytic interstitial pneumonia through renal findings instead of respiratory symptoms.

May 20, 2026

B35-26 Incidental Nephrocalcinosis Leading to the Diagnosis of Lymphocytic Interstitial Pneumonia and Sjogren’s Syndrome

Key Points

This case explores the atypical discovery of lymphocytic interstitial pneumonia through renal findings instead of respiratory symptoms.
Assessment of a 35-year-old female with acute cholecystitis leading to imaging revealing nephrocalcinosis and pulmonary cysts.
Laboratory evaluation for metabolic acidosis and serological testing for autoimmune markers including ANA.
High-resolution CT scan analysis and renal biopsy for definitive diagnosis.
The patient had diffuse thin-walled cysts in the lungs suggestive of Sjögren’s-related lymphocytic interstitial pneumonia.
Treatment led to reduced renal inflammation, evidenced by decreased ANA titer and beta-2 microglobulin levels.
Biopsy findings indicated lymphoplasmacytic interstitial nephritis, supporting the diagnosis of Sjögren’s syndrome.

Abstract

Abstract Lymphocytic interstitial pneumonia (LIP) is a rare interstitial lung disease characterized by diffuse lymphoplasmacytic infiltration of the alveolar septa, most often associated with autoimmune conditions such as Sjögren’s syndrome. Patients typically present with respiratory symptoms or incidental pulmonary findings. Diagnosis through renal manifestations is exceptionally uncommon. We report a previously healthy 35-year-old female who presented with acute cholecystitis and was incidentally found to have medullary nephrocalcinosis and numerous thin-walled pulmonary cysts upon initial imaging. Laboratory evaluation showed chronic non-anion gap metabolic acidosis with hypokalemia consistent with distal renal tubular acidosis (dRTA). Serologic testing revealed positive ANA and elevated SSA/SSB antibodies, consistent with Sjögren’s syndrome. High-resolution CT demonstrated diffuse, thin-walled cysts with lower lobe predominance concerning for Sjögren’s-related LIP. Renal biopsy demonstrated lymphoplasmacytic interstitial nephritis with immune complex deposition, further supporting the diagnosis. The patient was treated with corticosteroids and mycophenolate sodium with biochemical improvement, including decreased ANA titer and beta-2 microglobulin, reflecting reduced renal inflammation. This case highlights an atypical presentation of LIP discovered through nephrogenic rather than pulmonary symptoms, further elucidating the esoteric nature of Sjögren’s pathology. It underscores the importance of a broad differential in young patients with renal abnormalities and recognition that extra-glandular features may provide an early diagnostic clue to underlying systemic autoimmune disease. Clinicians should consider baseline pulmonary imaging and function testing in Sjögren’s patients, even in the absence of respiratory complaints, to facilitate early recognition and treatment of pulmonary manifestations such as LIP, which carry risks of progression to fibrosis or lymphoma. This abstract is funded by: None

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Cite This Study

Wei et al. (Fri,) studied this question.

synapsesocial.com/papers/6a0d4fbff03e14405aa9b277 https://doi.org/https://doi.org/10.1093/ajrccm/aamag162.753

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