CHEST Guidelines-A-29-Year-Old-Woman-With-Cough,-Dry-Eyes,-Pulmonar

A-29-Year-Old-Woman-With-Cough,-Dry-Eyes,-Pulmonar

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The document describes a case of a 29-year-old woman presenting with symptoms including a dry cough, fatigue, dry eyes, and dry mouth, with no history of smoking or lung disease. Examination and imaging (CT scan) revealed clear lungs but bilateral diffuse cysts and nodules, some calcified. Laboratory tests showed proteinuria and positive antinuclear antibodies and autoantibodies, suggestive of an autoimmune condition. <br /><br />The patient was diagnosed with Pulmonary Light Chain Deposition Disease (PLCDD) secondary to Sjögren’s syndrome. PLCDD is a rare condition characterized by extracellular deposition of monoclonal immunoglobulins, leading to diffuse cystic lung disease. This condition can affect other organs, primarily the kidneys, liver, and heart. PLCDD is often linked to other underlying conditions, with Sjögren’s syndrome and multiple myeloma being common causes.<br /><br />Typically, pulmonary involvement in Sjögren’s syndrome manifests as cystic lung disease. Cysts in PLCDD are often asymptomatic and discovered incidentally. The document notes that treatment of PLCDD focuses on the underlying condition. For this patient, mycophenolate mofetil was administered to address the Sjögren’s syndrome, resulting in symptom improvement. <br /><br />The clinical discussion emphasizes that PLCDD has an indolent course but may require treatment for autoimmune conditions. Lung transplantation could be considered for progressive respiratory failure. Few cases (about 55 reported) exist, with most patients having Sjögren's syndrome or multiple myeloma. It suggests tailored diagnostic workups, including serological studies and imaging, to identify the underlying condition and guide treatment.

Keywords

Pulmonary Light Chain Deposition Disease

Sjögren’s syndrome

cystic lung disease

autoimmune condition

mycophenolate mofetil

antibodies

immunoglobulins

proteinuria

lung transplantation

diagnostic workup