Bilateral Pleural Effusions as an Initial Presentation in Primary Sjögren’s Syndrome (original) (raw)
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Primary Sjögren Syndrome With Pleural Effusion
Archivos De Bronconeumologia, 2017
Síndrome de Sjögren primario con derrame pleural To the Editor, Sjögren's syndrome (SS) is a chronic inflammatory autoimmune disease characterized by lymphocytic infiltration of the exocrine glands, particularly the lacrimal and salivary glands, causing the characteristic symptoms of xerophthalmia and xerostomia. The lung, thyroid, kidney and the hepatobiliary tract can also be affected. This disease can be present in an isolated form (primary SS), or it can be associated with other connective tissue diseases (secondary SS). Pleural effusion (PE) in primary SS is rarely described in the literature. 1 We report the case of a 40-year-old woman with insulindependent diabetes mellitus, consulting due to xerostomia and xerophthalmia for some months, and intermittent diffuse arthromyalgia. Vital signs were normal and the only notable finding on physical examination was limited mobility of the right shoulder. Routine clinical laboratory tests (including angiotensin-converting enzyme levels) and urine tests were normal. Of particular interest were rheumatoid factor 103 IU/ml (upper level of normal 14 IU/ml), positive antinuclear antibodies (ANA) (1/640), and positive anti-Ro/SS-A and anti-La/SS-B antibodies and immunoglobulin G 4050 mg/dl. The Schirmer test was positive in both eyes, a minor salivary gland biopsy showed Chisholm grade 2 lymphocytic infiltration, and scintigraphy of the salivary glands was consistent with SS. A diagnosis of primary SS was established on the basis of these results. Four years later, the patient developed right pleuritic pain, without dyspnea, cough or expectoration, but with low-grade fever in the evenings. She reported no previous chest injury and no contact with tuberculosis patients. Physical examination was unremarkable, except for signs of right PE. The chest computed tomography revealed thick-walled cystic and cavitary lesions in the right lung and a small ipsilateral PE. Fiberoptic bronchoscopy was performed that was macroscopically normal, and smear tests and PCR for Mycobacterium tuberculosis in bronchoalveolar lavage were negative. PE was an exudate (total pleural fluid protein/serum protein ratio 0.7; lactate dehydrogenase 597 IU/l and pleural fluid LDH/serum LDH 1.8), leukocytes 5830/l (neutrophils 14%, lymphocytes 24%, eosinophils 16%, macrophages 46%), with normal pH, glucose, amylase, adenosine deaminase, tumor markers and N-terminal pro-brain natriuretic peptide levels, rheumatoid factor 58.6 IU/ml, positive ANA (1/320), and positive anti-Ro/SS-A
Pulmonary Manifestations of Sjögren's Syndrome
Chest, 1976
In 9-20% of cases, Sjögren's syndrome is associated with various respiratory symptoms. The most typical manifestations are chronic interstitial lung disease (ILD) and tracheobronchial disease. The most common manifestation of ILD is nonspecific interstitial pneumonia in its fibrosing variant. Other types of ILD, such as organising pneumonia, usual interstitial pneumonia and lymphocytic interstitial pneumonitis, are rare. Their radiological presentation is less distinctive, and definitive diagnosis may require the use of transbronchial or surgical lung biopsy. Corticosteroid therapy is the mainstay of ILD treatment in Sjögren's syndrome, but the use of other immunosuppressive drugs needs to be determined. ILD is a significant cause of death in Sjögren's syndrome. Tracheobronchial disease is common in Sjögren's syndrome, characterised by diffuse lymphocytic infiltration of the airway. It is sometimes responsible for a crippling chronic cough. It can also present in the form of bronchial hyperresponsiveness, bronchiectasis, bronchiolitis or recurrent respiratory infections. The management of these manifestations may require treatment for dryness and/or inflammation of the airways. Airway disease has little effect on respiratory function and is rarely the cause of death in Sjögren's syndrome patients. Rare respiratory complications such as amyloidosis, lymphoma or pulmonary hypertension should not be disregarded in Sjögren's syndrome patients. @ERSpublications We present the features, diagnostic tests and treatments of thoracic manifestations of Sjögren's syndrome http://ow.ly/10m8vd In addition to dryness, clinical presentation of Sjögren's syndrome generally includes asthenia and arthralgia. The disease can extend beyond the exocrine glands, and systemic manifestations including vasculitis, lung, renal or neurological involvement can occur. Patients with Sjögren's syndrome also have an increased risk of lymphoma [4]. The pulmonary manifestations of Sjögren's syndrome include airway abnormalities, interstitial lung disease (ILD) and lymphoproliferative disorders (table 1). Lung involvement occurs in ∼9-20% of patients.
Cureus
Sjogren's syndrome (SS) is a chronic exocrinopathy caused by lymphocytic infiltration and is associated with numerous manifestations and morbidities. We discuss a case of a 60-year-old female who presented to the Acute Medical Assessment Unit complaining of progressive shortness of breath for one month, not associated with chest pain or lower limb swelling. She also reported joint pain involving both wrists and proximal interphalangeal (PIP) joints, oral dryness, hair loss, and numerous tongue ulcerations. Blood workup revealed triple-negative SS, negative rheumatoid factor, anti-SSA and anti-SSB, a high erythrocyte sedimentation rate (ESR), and antinuclear antibody (ANA) titer of 640. A diagnosis of SS was made. Nevertheless, her CT chest showed massive left-sided pneumothorax; subsequently, a chest tube was urgently inserted. The chest tube was removed two days later with complete resolution on chest X-ray (CXR). However, one week later, she presented with a recurrent pneumothorax that persisted and required surgical intervention that led to complete recovery afterward. Pneumothorax is an extremely rare but potentially unfavorable complication related to SS, with only two cases reported in the literature so far and usually associated with underlying lung pathology.
Pulmonary Manifestations of Primary Sjögren's Syndrome
American Journal of Respiratory and Critical Care Medicine, 2005
Rationale: Clinicopathologic pulmonary manifestations associated with primary Sjögren's syndrome have yet to be reviewed in a large series since the recognition of nonspecific interstitial pneumonia (NSIP) as a distinct histologic pattern. Objectives: To determine clinical presentations, high-resolution computed tomographic (HRCT) and histologic findings of the lung disease associated with primary Sjögren's syndrome in the light of NSIP, and to analyze prognosis of the disease. Methods: On the basis of 33 cases (31 surgical lung biopsies and 2 autopsies) collected consecutively from multiple centers, we have retrospectively evaluated clinical, radiologic, and pathologic manifestations of the disease. Prognostic factors were identified by univariate and multivariate analysis. Measurements and main results: We found that NSIP was the most frequently seen histologic pattern (20 of 33 cases [61%], 19 fibrosing and 1 cellular). Bronchiolar diseases and amyloid and malignant lymphoma were seen less frequently. HRCT-pathologic correlation resulted in a 94% positive predictive value of CT-NSIP pattern for pathologic diagnosis of NSIP, whereas the diagnostic value of HRCT was low (15%) with an HRCT pattern other than NSIP, data that may influence the decision to biopsy. The 5-year survival rate was 84% overall and 83% in patients with NSIP. Multivariate analysis on all patients showed that low Pa O 2 (p ϭ 0.02) and presence of microscopic honeycombing (p ϭ 0.04) were independently associated with survival. Patients with NSIP showed lower vital capacity (mean Ϯ SD: 68.5 Ϯ 16.6%pred) than patients without NSIP (92.5 Ϯ 18.6%pred; p Ͻ 0.001). Conclusion: Among a diversity of pulmonary lesions in primary Sjögren's syndrome, NSIP was the commonest histologic pattern and had a favorable prognosis.
Cureus, 2016
We report a case of a 24-year-old female with a history of asthma and gastroesophageal reflux disease (GERD). She presented to the emergency room with severe chest pain, chest tightness, and shortness of breath following an upper respiratory tract infection. The patient reported that she had a cough and runny nose one week prior to this presentation, followed by a sudden sharp pain in the center of the chest 8/10 in intensity on the visual analog scale and pleuritic in nature, which aggravated by deep breathing and lying down flat. It was relieved by sitting up straight and did not radiate to her left arm or jaw. Computed tomography (CT) scan of the chest, posteroanterior and lateral views, showed a mild left pleural effusion with adjacent left basilar atelectasis/infiltrate. CT angiography of the chest with axial contrast showed mild left pleural effusion as well as a small pericardial effusion with bilateral lower lobe interstitial infiltrates. There was no evidence of pulmonary e...
Rheumatology, 1998
Eighteen non-smoking women suffering from primary Sjö gren's syndrome (pSS) with previously documented alveolitis were re-examined, clinically and by pulmonary function tests (PFT ), bronchoalveolar lavage (BAL), chest X-ray and high-resolution computed tomography (HRCT ) after a 2 yr follow-up period. Longitudinal evaluation revealed unchanged PFT. The final BAL study showed a normal differential count in six of 14 patients with initial lymphocyte alveolitis, and a persistent alveolar lymphocytosis in the remaining eight patients, associated with an increased percentage of neutrophils in one of them. In four patients with initial mixed alveolitis, the BAL cell profile was unchanged 2 yr later. Five of 18 patients (28%) had abnormal HRCT, represented by isolated septal/subpleural lines in three patients, ground-glass opacities with irregular pleural margins in one patient, and ground-glass opacities associated with septal/subpleural lines in another. All these patients had abnormal BAL results with an increased proportion of both neutrophils and lymphocytes. The presence of alveolar neutrophils was associated with a significantly (P = 0.005) greater mean rate of reduction of carbon monoxide diffusing capacity (DLCO)more than four times the normal rate of loss of DLCO. Chest X-ray, repeated at the end of the 2 yr follow-up period, showed parenchymal abnormalities in only one patient who had evidence of fibrosis on HRCT. This study provides evidence that lung involvement is not an uncommon extraglandular manifestation of pSS and that a BAL neutrophilia may play an important role in the pathogenesis of pulmonary disease in this autoimmune disorder.
Inflamación bronquial, clínica respiratoria y función pulmonar en el síndrome de Sjögren primario
Archivos de Bronconeumología, 2011
There is no information available regarding the relationship between the respiratory symptoms or lung function and bronchial inflammation, measured by induced sputum. Description of the clinical characteristics, radiographic images and lung function of patients suffering from Primary Sjögren Syndrome (PSS), and to assess the relationship with the inflammatory airway profile. We analysed clinical, radiology, lung function tests, bronchial hyperresponsiveness and inflammatory data in the induced sputum from 36 consecutive patients with PSS. A total of 58% of patients had hoarseness and 42% had cough and dispnea. No lung dysfunction was observed, although 46% (n=16) had a positive bronchial response. Lymphocytosis >2.6% in induced sputum was observed in 69% of all sputa. There was chronic cough in 29% of patients with lymphocytosis (n=24), whereas 73% were normal (n=11) (P=.02). The duration time of cough was less for the former (P=.02). On the contrary a positive bronchial response was associated with lymphocytosis >2.6% (P=.02). Lipophages were present in 55% of pathological sputa (n=22) (index >15) versus 18% of the non-pathological ones (n=11) (P=.05). Hoarseness, cough and dyspnea are frequent respiratory symptoms in PSS, although there is a wide variation in the relationship with bronchial responsiveness and airway inflammation. Lymphocytosis in the airways is another site of the infiltrative process in PSS, and the induced sputum is a complementary tool in the identification of active inflammatory process.