Sjögren’s syndrome as a cause of both lymphoid interstitial pneumonia and light chain deposition disease in a single patient

Steward, Matthew; Yu, J.H.; Gibbons, Michael

doi:10.1136/bcr-2022-249747

Cited by 2 publications

(1 citation statement)

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“…The infiltrating cells usually consist of lymphocytes, immunoblasts, plasma cells and histiocytes, including epithelioid cells and macrophages. Nodular lymphoid aggregates with reactive germinal centers are present in up to 50% of patients with LIP with idiopathic and connective tissue-related disease, in most cases with a greater proportion of lymphocytes compared to plasma cells, [ 6 , 15 ] with B cells usually located in nodular lymphoid follicles and T cells mostly found in the interstitium. [ 16 ] Compared to LIP, the interstitial lymphocytic infiltrate is less diffuse and intense in nonspecific interstitial pneumonia and hypersensitivity pneumonitis, and the lesions of nodular lymphoid hyperplasia are well-defined.…”

Section: Discussionmentioning

confidence: 99%

Lymphocytic interstitial pneumonia presenting with a ground glass nodule: A case report and literature review

et al. 2023

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Rationale: Lymphocytic interstitial pneumonia (LIP) is a rare benign lymphoproliferative disorder, often associated with autoimmune diseases. Most LIPs present with multiple bronchial cysts and diffuse interstitial infiltration. It is histologically characterized by widespread diffuse lymphocytic infiltration of the pulmonary interstitium, and the enlargement and widening of the alveolar septum. Patient concerns: A 49-year-old woman was admitted to hospital for finding pulmonary nodules for more than 2 months. 3D imaging chest computed tomography (CT) examination of both lungs showed that there was a middle lobe of the right lung with a size of about 1.5 cm × 1.1 cm ground-glass nodules. Diagnoses: A single operating port thoracoscopic wedge resection biopsy of a right middle lung nodule was performed. The pathology showed diffuse lymphocytic infiltration with varying numbers of small lymphocytes, plasma cells, macrophages and histiocytes infiltrating the alveolar septa, widened and enlarged alveolar septa, and scattered lymphoid follicles. Immunohistochemically, CD20 positive in follicular area, CD3 positive in interfollicular area. LIP was considered. Interventions: The patient was regularly followed without any specific treatment. Outcomes: Follow-up chest CT showed no significant abnormalities in the lungs 6 months after surgery. Lessons: To the best of our knowledge, our case may be the second reported case of a patient with LIP presenting with a ground glass nodule on chest CT, and it is speculated that the ground glass nodule may be an early manifestation of idiopathic LIP.

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Section: Discussionmentioning

confidence: 99%

Lymphocytic interstitial pneumonia presenting with a ground glass nodule: A case report and literature review

et al. 2023

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Diagnosis of light‑chain deposition disease after renal transplantation: A case report and literature review

Yue,

Xu,

Zhang

et al. 2024

Exp Ther Med

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Light chain deposition disease (LCDD) is a rare, clonal plasma cell proliferative condition. The deposition of nonamyloid monoclonal immunoglobulin light chains predominantly affects the kidneys, which may lead to end-stage renal disease, eventually requiring renal replacement therapy. The present study reported a rare case of LCDD that was confirmed after renal transplantation. A 49-year-old man initially presented with heavy proteinuria, hypoproteinemia, hyperlipidemia and renal insufficiency. The patient was diagnosed with nephrotic syndrome and pathological examination revealed fibrillary glomerulonephritis in 2014. Treatment was started with prednisolone. About 5 years later, the patient began to receive continuous hemodialysis due to worsening serum creatinine levels. Renal allograft transplantation was performed in 2020 and dialysis independence was achieved. Laboratory findings before renal transplantation revealed that serum and urine immunofixation electrophoresis was negative. Allograft kidney biopsy established the pathological diagnosis of LCDD at >1 year after renal transplantation for renal dysfunction. The treatment is challenging due to the lack of generally accepted standard treatment practices. Administration of bortezomib combined with dexamethasone was started. As anemia and renal failure developed progressively, the treatment was switched to anti-CD38 antibody and continuous hemodialysis was restarted. The best response achieved was hematological partial response and relief of anemia. However, the patient's renal function did not improve and he remains to have end-stage kidney disease. LCDD is easily missed in cases in which serum and urine immunofixation electrophoresis is negative. Hence, early recognition of LCCD based on kidney biopsy is important. To the best of our knowledge, the use of anti-CD38 antibody therapy in patients with LCDD is rarely reported. Anti-CD38 antibody is effective in treating LCDD, but it may not reverse the marked deterioration of renal function.

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Sjögren’s syndrome as a cause of both lymphoid interstitial pneumonia and light chain deposition disease in a single patient

Cited by 2 publications

References 10 publications

Lymphocytic interstitial pneumonia presenting with a ground glass nodule: A case report and literature review

Lymphocytic interstitial pneumonia presenting with a ground glass nodule: A case report and literature review

Diagnosis of light‑chain deposition disease after renal transplantation: A case report and literature review

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