Limits...
Thymic lymphoid hyperplasia with multilocular thymic cysts diagnosed before the Sjögren syndrome diagnosis.

Minato H, Kinoshita E, Nakada S, Nojima T, Tanaka M, Usuda K, Sagawa M, Iwao H, Tanaka M, Doai M, Takahashi T, Shibata N - Diagn Pathol (2015)

Bottom Line: Lymphocytes were composed of an organized mixed population of mature T and B cells without significant atypia.The infiltrated B cells did not reveal light chain restriction or immunoglobulin heavy chain gene rearrangement.The Schirmer's, chewing gum, and Saxon tests showed decreased salivary and lacrimal secretion.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology and Laboratory Medicine, Kanazawa Medical University, 1-1 Daigaku, Uchinada, Kahoku, Ishikawa, 9200293, Japan. hminato@kanazawa-med.ac.jp.

ABSTRACT

Background: Thymic lymphoid hyperplasia is often present with myasthenia gravis as well as other autoimmune diseases such as systemic lupus erythematosus and rheumatoid arthritis. Of the 4 cases of thymic lymphoid hyperplasia associated with Sjögren syndrome that have been reported, no case with a thymic lesion diagnosis that led to the diagnosis of Sjögren syndrome has been reported. We herein report a case of thymic lymphoid hyperplasia with multilocular thymic cysts, diagnosed before Sjögren syndrome.

Case presentation: A 37-year-old Japanese woman had an approximate 5-cm anterior mediastinal mass detected by chest imaging. The resected lesion revealed multilocular thymic cysts that were filled with colloid-like material. Histology showed lymph follicular hyperplasia with many epithelial cysts. The epithelium consisted of thymic medullary epithelium, and no epithelial proliferation was seen in the lymphoid tissue. Lymphocytes were composed of an organized mixed population of mature T and B cells without significant atypia. The infiltrated B cells did not reveal light chain restriction or immunoglobulin heavy chain gene rearrangement. After the pathological diagnosis of thymic lesion, tests for the presence of autoantibodies were positive for antinuclear antibodies, rheumatic factor, and anti-SSA/Ro antibodies. The Schirmer's, chewing gum, and Saxon tests showed decreased salivary and lacrimal secretion. Lip biopsy showed focal lymphocytic sialadenitis. The signs and symptoms of Sjögren syndrome had not resolved, without aggravation, 1 year after the thymectomy.

Conclusion: When a case with thymic lymphoid hyperplasia without myasthenia gravis is encountered, it is essential to consider the presence of another autoimmune disease including Sjögren syndrome.

No MeSH data available.


Related in: MedlinePlus

Radiological imaging of the lesion. Contrast-enhancement computed tomography (CT) shows a tumor in the superior anterior mediastinum with slightly enhanced multiple septa (a-c). Magnetic resonance imaging (MRI) with fat suppression reveals the tumor with high-intensity (d). The standardized uptake value (SUVmax) of positron emission tomography (PET)-CT was 4.25, which was relatively low (e)
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
getmorefigures.php?uid=PMC4502560&req=5

Fig1: Radiological imaging of the lesion. Contrast-enhancement computed tomography (CT) shows a tumor in the superior anterior mediastinum with slightly enhanced multiple septa (a-c). Magnetic resonance imaging (MRI) with fat suppression reveals the tumor with high-intensity (d). The standardized uptake value (SUVmax) of positron emission tomography (PET)-CT was 4.25, which was relatively low (e)

Mentions: A 37-year-old Japanese woman was referred to our hospital for general malaise. Chest radiography, computed tomography (CT), and magnetic resonance imaging revealed a superior anterior mediastinal tumor that was 4.7 × 3.8 × 2.8 cm in size, with multilocular cysts. CT with contrast enhancement showed mild enhancement of the septum of the lesion (Fig. 1a-c). T1-weighted and T2-weighted images revealed isointensity and high intensity, respectively, of the lesion (Fig. 1d). Calcification or fatty components were not seen. The positron emission tomography standardized uptake value (PET SUVmax) was 4.25, which was relatively low (Fig. 1e). The following tumor markers were all within normal range: carcinoembryonic antigen, alpha-fetoprotein, squamous cell carcinoma antigen, CYFRA 21–1, sialyl Lewis X, and progastrin-releasing peptide. Anti-acetylcholine receptor antibodies were not detected. Thymoma, thymic cyst, or teratoma was suspected, and a thymectomy was performed. After the pathological diagnosis, on suspicion of an associated autoimmune disease, autoantibodies were examined and were positive for antinuclear antibodies (speckled type, ×640), rheumatic factor (114; normal range <15 IU/mL), and anti-SSA/Ro antibodies (>240; normal <10 U/mL) and negative for anti-SS-B/La, anti-DNA, anti-RNP, anti-Sm, anti-cyclic citrullinated peptide, Jo-1, Scl-70, and anti-neutrophil cytoplasmic antibodies (MPO-ANCA and PR3-ANCA). She also had a mildly dry mouth and dry eyes. The Schirmer’s (right 2 mm/left 2 mm), chewing gum (2.9 mL/10 min), and Saxon (0.62 g/2 min) tests all showed decreased secretion of the lacrimal and salivary glands. A lip biopsy specimen revealed focal lymphocytic sialadenitis. Based on these findings, Sjögren syndrome was diagnosed. The signs and symptoms of Sjögren syndrome, without aggravation, were not resolved 1 year after the thymectomy, and autoantibody titers did not show remarkable changes.Fig. 1


Thymic lymphoid hyperplasia with multilocular thymic cysts diagnosed before the Sjögren syndrome diagnosis.

Minato H, Kinoshita E, Nakada S, Nojima T, Tanaka M, Usuda K, Sagawa M, Iwao H, Tanaka M, Doai M, Takahashi T, Shibata N - Diagn Pathol (2015)

Radiological imaging of the lesion. Contrast-enhancement computed tomography (CT) shows a tumor in the superior anterior mediastinum with slightly enhanced multiple septa (a-c). Magnetic resonance imaging (MRI) with fat suppression reveals the tumor with high-intensity (d). The standardized uptake value (SUVmax) of positron emission tomography (PET)-CT was 4.25, which was relatively low (e)
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4502560&req=5

Fig1: Radiological imaging of the lesion. Contrast-enhancement computed tomography (CT) shows a tumor in the superior anterior mediastinum with slightly enhanced multiple septa (a-c). Magnetic resonance imaging (MRI) with fat suppression reveals the tumor with high-intensity (d). The standardized uptake value (SUVmax) of positron emission tomography (PET)-CT was 4.25, which was relatively low (e)
Mentions: A 37-year-old Japanese woman was referred to our hospital for general malaise. Chest radiography, computed tomography (CT), and magnetic resonance imaging revealed a superior anterior mediastinal tumor that was 4.7 × 3.8 × 2.8 cm in size, with multilocular cysts. CT with contrast enhancement showed mild enhancement of the septum of the lesion (Fig. 1a-c). T1-weighted and T2-weighted images revealed isointensity and high intensity, respectively, of the lesion (Fig. 1d). Calcification or fatty components were not seen. The positron emission tomography standardized uptake value (PET SUVmax) was 4.25, which was relatively low (Fig. 1e). The following tumor markers were all within normal range: carcinoembryonic antigen, alpha-fetoprotein, squamous cell carcinoma antigen, CYFRA 21–1, sialyl Lewis X, and progastrin-releasing peptide. Anti-acetylcholine receptor antibodies were not detected. Thymoma, thymic cyst, or teratoma was suspected, and a thymectomy was performed. After the pathological diagnosis, on suspicion of an associated autoimmune disease, autoantibodies were examined and were positive for antinuclear antibodies (speckled type, ×640), rheumatic factor (114; normal range <15 IU/mL), and anti-SSA/Ro antibodies (>240; normal <10 U/mL) and negative for anti-SS-B/La, anti-DNA, anti-RNP, anti-Sm, anti-cyclic citrullinated peptide, Jo-1, Scl-70, and anti-neutrophil cytoplasmic antibodies (MPO-ANCA and PR3-ANCA). She also had a mildly dry mouth and dry eyes. The Schirmer’s (right 2 mm/left 2 mm), chewing gum (2.9 mL/10 min), and Saxon (0.62 g/2 min) tests all showed decreased secretion of the lacrimal and salivary glands. A lip biopsy specimen revealed focal lymphocytic sialadenitis. Based on these findings, Sjögren syndrome was diagnosed. The signs and symptoms of Sjögren syndrome, without aggravation, were not resolved 1 year after the thymectomy, and autoantibody titers did not show remarkable changes.Fig. 1

Bottom Line: Lymphocytes were composed of an organized mixed population of mature T and B cells without significant atypia.The infiltrated B cells did not reveal light chain restriction or immunoglobulin heavy chain gene rearrangement.The Schirmer's, chewing gum, and Saxon tests showed decreased salivary and lacrimal secretion.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology and Laboratory Medicine, Kanazawa Medical University, 1-1 Daigaku, Uchinada, Kahoku, Ishikawa, 9200293, Japan. hminato@kanazawa-med.ac.jp.

ABSTRACT

Background: Thymic lymphoid hyperplasia is often present with myasthenia gravis as well as other autoimmune diseases such as systemic lupus erythematosus and rheumatoid arthritis. Of the 4 cases of thymic lymphoid hyperplasia associated with Sjögren syndrome that have been reported, no case with a thymic lesion diagnosis that led to the diagnosis of Sjögren syndrome has been reported. We herein report a case of thymic lymphoid hyperplasia with multilocular thymic cysts, diagnosed before Sjögren syndrome.

Case presentation: A 37-year-old Japanese woman had an approximate 5-cm anterior mediastinal mass detected by chest imaging. The resected lesion revealed multilocular thymic cysts that were filled with colloid-like material. Histology showed lymph follicular hyperplasia with many epithelial cysts. The epithelium consisted of thymic medullary epithelium, and no epithelial proliferation was seen in the lymphoid tissue. Lymphocytes were composed of an organized mixed population of mature T and B cells without significant atypia. The infiltrated B cells did not reveal light chain restriction or immunoglobulin heavy chain gene rearrangement. After the pathological diagnosis of thymic lesion, tests for the presence of autoantibodies were positive for antinuclear antibodies, rheumatic factor, and anti-SSA/Ro antibodies. The Schirmer's, chewing gum, and Saxon tests showed decreased salivary and lacrimal secretion. Lip biopsy showed focal lymphocytic sialadenitis. The signs and symptoms of Sjögren syndrome had not resolved, without aggravation, 1 year after the thymectomy.

Conclusion: When a case with thymic lymphoid hyperplasia without myasthenia gravis is encountered, it is essential to consider the presence of another autoimmune disease including Sjögren syndrome.

No MeSH data available.


Related in: MedlinePlus