Sjogren’s Syndrome with Interstitial Lung Disease

@#Sjogren’s syndrome (SS) is an autoimmune disease affecting exocrine glands with known properties to cause chronic systemic multi-organ disease involvement. It produced the typical features of sicca syndrome, but due to insidious onset of the disease, patient may present with complications of the condition at the initial presentation. We present a case of primary Sjogren’s syndrome (SS) who first presented with pulmonary manifestations and was subsequently diagnosed as Lymphocytic Interstitial Pneumonia (LIP). The patient was extensively investigated and received appropriate treatment modalities.

Neumonía intersticial linfocítica en una paciente con lupus eritematoso sistémico y síndrome de Sjögren secundario: reporte de caso y revisión de la literatura / Lymphocytic interstitial pneumonia in a patient with systemic lupus erythematosus and secondary Sjögren's syndrome: Case report and literature review

RESUMEN La neumonía intersticial linfocítica es una complicación, poco frecuente, asociada con el lupus eritematoso sistémico, sin embargo, con gran impacto en la calidad de vida. Se asocia con la presencia de anti Ro/SSA, anti La/SSB y con el diagnóstico de síndrome de Sjögren secundario. No es clara la estrategia terapéutica y la información existente está basada en reportes de caso sin disponibilidad de estudios adecuadamente diseñados. En el presente documento se expone el caso de una paciente con lupus eritematoso sistémico y síndrome de Sjögren secundario, que presentó una enfermedad pulmonar intersticial con características de neumonía intersticial linfocítica.

ABSTRACT Lymphocytic interstitial pneumonia is an infrequent complication associated with systemic lupus erythematosus and has a great impact on quality of life. It is associated with the presence of anti-Ro/SSA, anti-La/SSB, and the diagnosis of secondary Sjögren Syndrome. Its therapeutic strategy is not clear, and the existing information is based on case reports, with there being no properly designed studies available. The case is presented here of a patient with systemic lupus erythematosus and secondary Sjögren's syndrome, who also presented with interstitial lung disease with characteristics of lymphocytic interstitial pneumonia.

Lymphocytic interstitial pneumonia: computed tomography findings in 36 patients / Pneumonia intersticial linfocítica: achados na tomografia computadorizada em 36 pacientes

Abstract Objective: To analyze the computed tomography (CT) findings of lymphocytic interstitial pneumonia (LIP). Materials and Methods: We retrospectively reviewed the clinical and CT findings of 36 patients with LIP, including 25 women and 11 men, with a mean age of 52.5 years (age range, 22-78 years). Results: The main associated diseases with LIP were Sjögren syndrome (42%), human immunodeficiency virus infection (17%), amyloidosis (17%), Sjögren syndrome associated with secondary amyloidosis (11%), idiopathic (8%), and systemic lupus erythematosus (5%). The predominant CT abnormalities were multiple cystic airspaces (n = 35), small nodules (n = 15), ground-glass opacities (n = 13), bronchiectasis and/or bronchiolectasis (n = 8), and thickening of the bronchovascular bundles (n = 8). Other CT findings included reticular opacities (n = 7), calcified nodules (n = 4), airspace consolidation (n = 4), emphysema (n = 3), honeycombing (n = 3), lymph node enlargement (n = 2), mosaic attenuation pattern (n = 1), and cavitated nodules (n = 1). Conclusion: The main CT findings of LIP were multiple cysts, small nodules, and ground-glass opacities.

Resumo Objetivo: Analisar os achados da tomografia computadorizada da pneumonia intersticial linfocítica. Materiais e Métodos: Trinta e seis pacientes com diagnóstico de pneumonia intersticial linfocítica (25 mulheres, 11 homens; faixa etária de 22 a 78 anos; idade média de 52,5 anos) tiveram os achados clínicos e tomográficos revisados retrospectivamente. Resultados: As principais doenças associadas à pneumonia intersticial linfocítica foram síndrome de Sjögren (42%), infecção pelo HIV (17%), amiloidose (17%), síndrome de Sjögren associada a amiloidose secundária (11%), síndrome de Sjögren idiopática (8%) e lúpus eritematoso sistêmico (5%). As alterações predominantes na TC consistiram em múltiplos espaços aéreos císticos (n = 35), pequenos nódulos (n = 15), opacidades em vidro fosco (n = 13), bronquiectasias e bronquiolectasias (n = 8) e espessamento de feixes broncovasculares (n = 8). Outros achados da TC incluíram opacidades reticulares (n = 7), nódulos calcificados (n = 4), consolidação do espaço aéreo (n = 4), enfisema (n = 3), faveolamento (n = 3), linfonodomegalia linfática (n = 2), padrão de atenuação em mosaico (n = 1) e nódulos escavados (n = 1). Conclusão: Os principais achados tomográficos observados em pacientes com pneumonia intersticial linfocítica foram múltiplos cistos, pequenos nódulos e opacidades em vidro fosco.

Lymphocytic interstitial pneumonia in a patient with Sjögren's syndrome / 영남의대학술지

Lymphocytic interstitial pneumonia (LIP) is a rare benign lymphoproliferative disorder characterized by diffuse infiltration of the pulmonary parenchymal interstitium by polyclonal lymphocytes and plasma cells. LIP has been associated with a variety of clinical conditions; such as connective tissue disorders and other immune system abnormalities. Treatment usually involves administration of corticosteroids and other immunosuppressants. We report on a 38-year-old female patient who complained of shortness of breath, dry mouth, and dry eyes for more than 1 month, and was positive for Raynaud's phenomenon. Based on surgical biopsy, she was diagnosed as having LIP accompanied by Sjögren's syndrome. The patient was treated with high-dose steroids followed by maintenance therapy for approximately 2 years, and her condition improved.

Remission of Lymphocytic Interstitial Pneumonia in Sjogren's Syndrome after Autologous Peripheral Blood Stem Cell Transplantation

Interstitial pneumonia occurs in approximately 25% of patients with primary Sjogren's syndrome. Interstitial pneumonia combined with primary Sjogren's syndrome usually responds well to systemic steroids, and fatal cases are rare. Lymphocytic interstitial pneumonia shows diffuse infiltration of polyclonal B and T cells. Autologous stem cell transplantation is performed in cases of primary Sjogren's syndrome as an optional treatment when the condition responds poorly to conventional treatment. The hypothesis that primary Sjogren's syndrome improves after transplantation relies on the role of B-cell abnormalities in pathogenesis or the strong effects of immunosuppressive therapy. We experienced the case of a patient diagnosed with primary Sjogren's syndrome and lymphocytic interstitial pneumonia progression refractory to conventional treatment (steroid and immunosuppressive drugs) and cyclophosphamide pulse therapy. Our patient demonstrated improvement of lung manifestations and autoimmune disease activity after autologous stem cell transplantation.

A Case of Epstein-Barr-Virus-Associated Lymphocytic Interstitial Pneumonia / 소아알레르기및호흡기학회지

Lymphocytic interstitial pneumonia (LIP) is a benign lymphoproliferative disorder characterized histologically by diffuse infiltration with predominantly mature lymphocytes, plasma cells and reticuloendothelial cells in the alveolar septae and along the lymphatic vessels. LIP is an uncommon disease frequently associated with autoimmune diseases such as Sj?gren's syndrome, systemic lupus erythematosus, chronic active hepatitis, thyroiditis, myasthenia gravis, autoimmune hemolytic anemia and Human Immunodeficiency Virus (HIV) or Epstein- Barr virus (EBV) infection. LIP can progress to pulmonary or systemic lymphoma. LIP in children has variable disease courses such as spontaneous resolution, episodic worsening or progression to frank respiratory failure. Intravenous immunoglobulin and corticosteroids yield variable results, and some children may respond to them for several years. We report herein a case of EBV-associated, HIV-negative LIP diagnosed by thoracoscopic lung biopsy and a clonality study in a 5 year-old boy who was admitted with recurrent coughing and dyspnea. The patient was treated and fully recovered with methylprednisolone and high dose acyclovir. At the 3-year follow-up, he showed no clinical symptom despite increased EBV copy in bronchoalveolar lavage and progressive bronchiectasis in the right middle lobe.

A Case of Lymphocytic Interstitial Pneumonia Manifested as a Multi-focal Consolidation / 결핵및호흡기질환

Lymphocytic interstitial pneumonia (LIP) is a rare disorder characterized by a diffuse infiltration of the alveolar space, interstitium by lymphocytes, plasma cells, and reticuloendothelial cells. Although its etiology is unknown, LIP has been associated with autoimmune disorders and with viral infections. Because it's clinical and radiographic features are nonspecific, a confirmatory diagnosis is performed by open lung biopsy. A 59-year-old female presented dry cough, which had been present for 1 month. On initial findings of multifocal consolidation at the right middle lobe on both lower lobes in chest radiography, the first diagnosis of cryptogenic organizing pneumonia was suggested. On open lung biopsy, LIP was diagnosed. The patient had no autoimmune disease, viral infection or monoclonal gammopathy. After 3 months of corticosteroid treatment, the patient experienced improved symptoms, reduced abnormalities on chest radiography, and improved pulmonary function testing.

Síndrome de Sjögren primario con neumonía intersticial linfocítica y enfermedad quística pulmonar / Primary Sjögren's syndrome with lymphocitic interstitial pneumonia and cystic pulmonary disease

El síndrome de Sjögren primario (SSP) es una exocrinopatía autoinmune crónica caracterizada por infiltración linfocítica de tejido glandular y extraglandular; el compromiso pulmonar es heterogéneo; el objetivo del presente estudio es informar el primer caso en Colombia de SSP con neumonía intersticial linfocítica y enfermedad quística pulmonar, plantear diagnósticos diferenciales, abordaje diagnóstico y alternativas terapéuticas.

The primary Sjögren's syndrome (PSS) is a chronic autoimmune exocrinopathy characterized by lymphocytic infiltration of glandular and extraglandular tissue, the lung involvement is heterogeneous; the aim of this study is report the first case in Colombia of PSS with lymphocytic intersticial pneumonia and cystic pulmonary disease discuss differential diagnosis and the initial diagnostic and therapeutic work up.

Diffuse Nodular Interstitial Infiltrations with Bilateral Hilar Lymphadenopathy / 결핵및호흡기질환

Lymphocytic interstitial pneumonia(LIP) is an uncommon condition in which the alveolar septa and extra-alveolar interstitial space are markedly expanded by small lymphocytes, plasma cells and histiocytes. Chest radiographs generally show nonspecific patterns with the most common pattern showing bibasilar reticular or reticulonodular infiltrates. Hilar or mediastinal lymphadenopathy and pleural effusions are usually absent. We encountered a 42-year-old female patient who was admitted to hospital because of exertional dyspnea and palpitation. The chest X-ray showed an enlarged bilateral hilar shadow and diffusely increased bronchovascular markings in both lung fields. The chest CT showed diffuse nodular infiltrations with mild septal thickening and combined patchy ground glass opacity in both lungs, and conglomerated mediastinal and bilateral hilar lymphadenopathy. A diagnosis of LIP was made from the tissue pathology taken by a thoracoscopic lung biopsy. The patient showed clinical and radiographic improvement after 3 months of treatment with prednisolone. We report a case of LIP presenting as diffuse nodular interstitial infiltrations with multiple mediastinal and bilateral hilar lymphadenopathy.

A Case of Lymphocytic Interstitial Pneumonia / 결핵

Lymphocytic interstitial pneumonia(LIP) is characterized by a massive infiltration of the interstitium of the lung by mature lymphocytes, plasma cells and reticuloendothelial cells. LIP may be associated with autoimmune diseases including Sjogrens syndrome, SLE, myasthenia gravis, pernicious anemia, autoimmune hemolytic anemia, and HIV or an EB virus infection. There is a possibility of LIP progressing to a pulmonary or systemic lymphoma. The therapeutic response to corticosteroids and/or immunosuppressive drugs varies. Here we report a case of LIP that was diagnosed by an open lung biopsy and clonality study. The patient was a 36 year-old man without antuimmune disease or HIV infection. He was admitted as a result of severe hypoxemia showing PaO2 of 48.3 mmHg. The patient was treated with corticosteroids after the diagnosis and had fully recovered without a sequalae or relapse.

Clonality of Lymphocytic Interstitial Pneumonia in Common Variable Immunodeficiency / 대한소아혈액종양학회지

Common variable immunodeficiency (CVID) is a heterogenous syndrome characterized by hypogammaglobulinemia, various immunologic abnormalities and recurrent bacterial infections. Associated immunologic abnormalities consists of various kinds of autoimmune diseases and lymphoproliferative disorders. The lymphoproliferative disorder take several forms, such as malignant lymphoma, atypical lymphoid hyperplasia, and beniegn lymphoid hyperplasia. Lymphocytic interstitial pneumonia (LIP), which is a kind of atypical lymphoid hyperplasia, develop in young age groups and has controversy on its clonality. We experienced a 14-year-old female patient with LIP and CVID. We analysed the third complementarity-determining region (CDR3) of the immunoglobulin heavy chain gene for clonality analysis. Clonality analysis of lung biopsy specimen revealed that 6 of 13 colony and 4 of 13 colony have identical sequences respectively. We speculate that one of these 2 lymphoid cell clone may develop into malignant lymphoma in the future.

A Case of Lymphocytic Interstitial Pneumonia

Lymphoid interstitial pneumonia (LIP) is an uncommon process in childhood, and is characterized by interstitial accumulation of mature lymphocytes, plasma cells and reticuloendothelial cells. LIP is believed to have autoimmune or immunologic pathogenesis, because it is frequently associated with acquired immune deficiency syndrome, Epstein-Barr virus infection, common variable immunodeficiency. The patient described in this report had idiopathic thrombocytopenic purpura, Evans syndrome and LIP. The diminished levels of IgG1 & IgG2 were the only immunologic abnormality, which suggest that this patient may be in the early phase of common variable immunodeficiency or selective IgG2 & IgG4 deficiency due to immunoglobulin heavy chain deletion. We can rule out the possibility of lymphoma by clonality study, which showed polyclonal lymphocyte infiltration. On our knowledge, this is the first report of LIP in Korean children.