Clinical and imaging findings of lymphoma in patients with Sjögren syndrome

OBJECTIVE: To describe and correlate the clinical and imaging findings of lymphomas in patients with Sj?gren syndrome. METHODS: The authors reviewed the medical and imaging records of 27 cases of lymphoma from among a total of 463 patients with Sj?gren syndrome. The estimated prevalence of lymphoma in patients with Sj?gren syndrome was 5.8%. There were 22 women and 5 men. Histopathologically, 26 of the 27 neoplasms were non-Hodgkin lymphoma, including 6 mucosa-associated lymphoid tissue lymphomas, and the other neoplasm was Hodgkin lymphoma. The clinical and imaging findings of lymphomas were analyzed. RESULTS: No obvious correlations were present between the duration or severity of Sj?gren syndrome and the lymphoma development. At the initial diagnosis, extranodal involvement was observed in 14 (52%) of the 27 patients, including the salivary gland (n = 9), lacrimal gland (n = 2), lung (n = 2), and thyroid gland (n = 1), mostly in the neck organs. On the other hand, nodal involvement was observed in 21 (78%) of the 27 patients. Of these 21 patients, 19 had at least cervical lymph node involvement. CONCLUSION: Patients with Sj?gren syndrome are at increased risk of lymphoma development. Because most lymphomas initially involve the neck organs, including the lymph nodes, meticulous imaging studies mainly focused on the cervical regions are recommended in the follow-up of patients with Sj?gren syndrome.     

CT and MR findings of bilateral lacrimal gland enlargement in Sjögren syndrome

Two women with primary Sjögren syndrome underwent computed tomography (CT) and magnetic resonance (MR) imaging because of bilateral lacrimal gland enlargement. Histopathologic confirmation was obtained in both patients. Of the four lacrimal glands, one had lymphoepithelial disease, two had pseudolymphoma and one had mucosa-associated lymphoid tissue (MALT) lymphoma, respectively. From the imaging findings, however, it was not possible to differentiate benign lymphoproliferative disorders and malignant lymphoma.     

MR imaging findings in hands in early rheumatoid arthritis: comparison with those in systemic lupus erythematosus and primary Sjögren syndrome

PURPOSE: To evaluate prospectively the use of magnetic resonance (MR) imaging for differentiating true rheumatoid arthritis (RA) from systemic lupus erythematosus (SLE) or primary Sj?gren syndrome in patients who have inflammatory polyarthralgia of the hands but no radiographic evidence of RA. MATERIALS AND METHODS: This study had institutional review board approval, and patient informed consent was obtained. Twenty-eight patients (16 female and 12 male patients; mean age, 42 years) with early RA and 19 patients (18 female and one male patient; mean age, 46 years) with SLE (n = 14) or primary Sj?gren syndrome (n = 5) underwent MR imaging of both hands. All patients had inflammatory polyarthralgia of the hands and no evidence of erosive changes on radiographs. Coronal T2-weighted short inversion time inversion-recovery, transverse T1-weighted spin-echo, transverse fat-suppressed gadolinium-enhanced T1-weighted spin-echo, and transverse gadolinium-enhanced three-dimensional gradient-echo MR images were obtained. The following MR imaging variables were assessed in the wrist and nonthumb metacarpophalangeal joints: synovitis, bone lesions (erosion, defect, and edema), and tenosynovitis. Synovitis and bone lesions were scored with the OMERACT RA-MRI scoring system. Findings in patients with RA and those without RA were compared by means of Mann-Whitney, chi2, and Fisher exact tests. RESULTS: The only significant difference between the two groups in terms of individual scores for synovitis, bone lesions, and tenosynovitis was the more frequent presence of tenosynovitis of the right fourth extensor tendon in patients without RA (P = .04). There were no significant differences between patients with RA and those without RA in terms of global scores for synovitis, bone lesions, and tenosynovitis. However, bone marrow edema in the metacarpophalangeal joints was seen more frequently in patients with RA (P < .001). CONCLUSION: It may be impossible to distinguish between patients with early RA and those without RA (ie, those with SLE or primary Sj?gren syndrome) by means of MR imaging.     

Gallium-67 scintigraphic findings in a patient with breast lymphoma complicated with Sjögren syndrome

We report here a patient with mucosa associated lymphoid tissue (MALT)-lymphoma of the breast complicated with Sjögren syndrome. It is speculated that Ga-67 could accumulate not only in lymphoma lesions but also in benign lymphoproliferative locations of Sjögren syndrome. Gallium-67 scintigraphy might be useful for the diagnosis and therapeutic monitoring of MALT-lymphoma complicated with Sjögren syndrome.     

Pulmonary and thymic lymphoid hyperplasia in primary Sjögren’s syndrome

Pulmonary and thymic lymphoid hyperplasia with characteristic clinicoradiological manifestations were seen in a 71-year-old woman who was diagnosed with primary Sjögren’s syndrome. A hazy opacity 12 mm in diameter and an anterior mediastinal nodule 20 mm in diameter were incidentally detected on computed tomography. Thoracoscopic biopsy revealed lymphoid hyperplasia in the lung accompanying thymic lymphoid hyperplasia. Immunohistochemically, the pulmonary lesion was considered to be nodular lymphoid hyperplasia (NLH), not lymphoma. A previously undescribed finding in the NLH was the hazy opacity containing an air bronchiologram, not a solid nodule. This finding might suggest a common causal relation for NLH, thymic hyperplasia, and primary Sjögren’s syndrome.     

Pulmonary lesions associated with Sjögren's syndrome: radiographic and CT findings

The aim of this study was to analyse and compare the chest radiographic and CT findings in patients with primary and secondary Sj?gren's syndrome. We retrospectively evaluated the frequency of abnormality and findings of both the chest radiography (n=107) and CT (n=59) in patients with Sj?gren's syndrome. Abnormal cases were classified into five patterns based on predominant CT findings. Chest radiographic and CT abnormalities were seen in 24 (22%) and in 34 (58%) patients, respectively. Most frequently observed abnormal findings were linear and reticular opacities on chest radiograph, and ground-glass opacity, interlobular septal thickening and intralobular interstitial thickening on CT in both primary and secondary Sj?gren's syndrome. Centrilobular abnormalities were significantly more common in patients with primary Sj?gren's syndrome (p=0.018). According to our CT classification, interstitial pneumonia (IP) pattern was the most common in patients with both primary and secondary Sj?gren's syndrome. Bronchiolitis pattern was more common in patients with primary Sj?gren's syndrome and lymphoproliferative disorder (LPD) pattern was only observed in primary Sj?gren's syndrome. In conclusion, although the most frequently observed pattern in our CT classification was IP pattern in both primary and secondary Sj?gren's syndrome, centrilobular abnormalities and LPD pattern were relatively characteristic in patients with primary Sj?gren's syndrome.     

Sudden death from ischaemic heart disease in a female patient with Sjögren syndrome: a case report

Primary Sj?gren syndrome (SjS) has a comparatively good prognosis except when it is complicated by non-Hodgkin's lymphoma. We performed an autopsy on a young female patient with primary SjS who had died suddenly during a meal, and we discuss the relationship between primary SjS and the cause of death. Eosinophilic change of the cardiomyocytes and severe arteriolosclerosis were observed within the myocardium. In the conduction system, lymphocyte infiltration was detected in the bundle of His, in addition to arteriosclerosis in the sinoatrial node and atrioventricular node arteries. The cause of death was diagnosed as ischaemic heart disease induced by arteriolosclerosis: its development can probably be attributed mostly to primary SjS. It should thus be kept in mind that primary SjS can occasionally result in the development of cardiovascular complications, such as ischaemic heart disease, as well as systemic lupus erythematosus or rheumatoid arthritis.     

Postmortem computed tomography findings in the thorax – Experimental evaluation

ObjectiveExperimental fatal models were prepared to investigate the time-related course of lung changes using postmortem CT (PMCT). This study was approved by our institutional animal ethics committee.Materials and methodsTwenty-four NZW rabbits (female 24, 2.30–4.30 (mean 3.10) kg) were divided into 4 fatal groups; drowning, hypothermia, bag suffocation, and Potassium Chloride intravenous (control) group. All individuals were examined by CT (Aquilion CX, Toshiba, Japan) on postmortem time course until detection of putrefaction air. The percent of aerated lung volume (%ALV = 100 * (ALV/total lung volume)) was measured and the pleural space fluid was investigated by axial imaging. A paired t-test and Bonferroni/Dunn study were employed for statistical evaluation.ResultsIn intra-group analysis, the %ALV showed statistically different periods compared with each pre-image: 4–48 h in control, 1–24 h in drowning, 5–6 h in hypothermia, and 1–4 h in bag suffocation. In inter-group comparison (compared with control group), the %ALV increased in suffocation and decreased in drowning within 12 h. The %ALV remained significantly high in hypothermia until 24 h. The earliest detection times of pleural space fluid collection were different in each group: control (20 h), drowning (18 h), suffocation (36 h), and hypothermia (95 h).ConclusionThe lung hypostasis and the appearance of pleural space fluid collection presented differently in individual causes of death and depending on the postmortem time.     

Bronchiolitis obliterans syndrome in lung transplant recipients: can thin-section CT findings predict disease before its clinical appearance?

PURPOSE: To determine whether there are thin-section computed tomographic (CT) features that predict bronchiolitis obliterans syndrome (BOS) in lung transplant recipients before the clinical appearance and during the early stages of the disease. MATERIALS AND METHODS: Two hundred ninety-eight thin-section CT scans obtained in 26 lung transplant recipients who did (study group) and 26 lung transplant recipients who did not (control group) develop BOS were reviewed for the presence of mosaic perfusion, bronchiectasis, bronchial wall thickening, and air trapping. BOS was defined by using the recently revised definition of the International Society for Heart and Lung Transplantation. CT scans obtained in the BOS group were divided into three groups: Group A consisted of the last scans obtained before the clinical appearance of BOS; groups B and C consisted of, respectively, the first and last scans obtained after the clinical appearance of BOS. Scans obtained in the control group were acquired during similar posttransplantation periods and matched to scans in each BOS group. Sensitivity, specificity, and positive and negative predictive values were calculated separately for each subgroup. The optimal threshold for each thin-section CT-depicted abnormality was defined by using receiver operating characteristics analysis. RESULTS: The sensitivities of air trapping for the diagnosis of BOS during the periods in which the scans in groups A, B, and C were obtained were 50%, 44%, and 64%, respectively; specificities were 80%, 100%, and 80% respectively. Sensitivities of mosaic perfusion were 4%, 20%, and 36%, respectively; specificities were 100%, 96%, and 96%, respectively. Sensitivities of bronchiectasis were 25%, 24%, and 32%, respectively; specificities were 80%, 80%, and 96%, respectively. Sensitivities of bronchial wall thickening were 4%, 24%, and 40%, respectively; specificities were 96%, 84%, and 80%, respectively. Air trapping was seen intermittently in nine (43%) of 21 patients with CT scans that depicted this finding at least once. CONCLUSION: The value of the finding of air trapping before the clinical appearance and during the early stages of BOS is lower than has been previously reported. When using the recently revised criteria for BOS, the role of thin-section CT as a screening test to evaluate patients with lung transplants appears to be limited.     

Muscular involvement of Behçet's disease: ultrasonography, computed tomography, and magnetic resonance imaging findings

Behçet's disease (BD) is a form of systemic vasculitis with the classic triad of recurrent oral and genital ulcers along with uveitis. In BD, muscular involvement is very rare. We report a case of muscular involvement in BD with characteristic findings using ultrasonography, computed tomography, and magnetic resonance imaging.     

Primary Sjögren's syndrome initially manifested by optic neuritis: MRI findings

We herein describe the MRI findings in a patient clinically diagnosed with primary Sj?gren's syndrome (SjS) initially manifested by retrobulbar optic neuritis. A 63-year-old woman suddenly had left ocular pain and progressive visual disturbance. MR T2-weighted images revealed hyperintensity in the left optic nerve, with swelling. Contrast-enhanced T1-weighted images showed no abnormal enhancement. Follow-up MRI 6 months after admission revealed no significant changes in the affected optic nerve. To our knowledge, optic neuritis as a complication of SjS has been reported in ten patients [1, 2, 3, 4, 5, 6] and MRI findings in only one of them [6]. We thought MR images were useful for visualizing optic nerve involvement in SjS and observing its course.     

Air trapping in primary Sjögren syndrome: correlation of expiratory CT with pulmonary function tests

PURPOSE: The purpose of our study was to correlate the extent of air trapping on expiratory CT scans with results of pulmonary function tests (PFTs) in a large group of patients with primary Sj?gren syndrome and to determine whether these techniques may be complementary in assessing small airways obstruction. METHOD: Thirty-four nonsmoking patients with proven primary Sj?gren syndrome and 10 healthy nonsmokers underwent paired inspiratory-expiratory thin section CT and PFTs. Expiratory scans were scored for the presence and extent of areas of air trapping. Extent of air trapping was assessed visually and given a score. The functional significance of the extent of air trapping was evaluated in both groups and then correlated with the results of PFTs. RESULTS: Bronchiolar abnormalities were seen in 11 (32%) of 34 patients with primary Sj?gren syndrome. On the expiratory CT scans, a mosaic pattern of lung attenuation was identified in 17 patients. Air trapping was found in 44 of 204 lobar observations on the expiratory scans. The median point scale score at end-expiration was 3.6 (20%, Grade 1), ranging from 1 (5.5%, Grade 1) to 9 (50%, Grade 2). The mean total score of air trapping was more prevalent in lower (46/68) lobes (22.4%) than in upper (22/136) lobes (5.3%) (p < 0.001). PFTs were normal in the primary Sj?gren syndrome patients as well as the healthy subjects. Air trapping was found more frequently in patients with primary Sj?gren syndrome than in the healthy group. Only during exhalation was there evidence of minimal lobular-sized areas or air trapping (Grade 1) in three of the healthy subjects. We did not find any correlation between air trapping and PFTs including the forced expiratory flow rate between 25 and 75% of the forced vital capacity (FEF25-75). CONCLUSION: Expiratory high resolution CT revealed the extent of bronchiolar disease in patients with primary Sj?gren syndrome. We also found that the extent of air trapping did not correlate with PFTs, which suggests the existence of a subclinical bronchiolar inflammatory process that may precede detectable abnormalities in lung function tests.     

A comparative study between MR sialography and salivary gland scintigraphy in the diagnosis of Sjögren syndrome

PURPOSE: The purpose of this work was to compare the diagnostic accuracy of MR sialography with that of salivary gland scintigraphy in Sj?gren syndrome. METHOD: One hundred thirty patients clinically suspected of having Sj?gren syndrome were examined by MR sialography and salivary gland scintigraphy. A labial gland biopsy was performed in all patients. Imaging findings of MR sialography and salivary gland scintigraphy were compared with the results of labial gland biopsy. RESULTS: From the results of labial gland biopsy, the diagnosis of Sj?gren syndrome was established in 80 patients. Abnormally high T2 signal intensity areas on MR sialography and decreased uptake and delayed excretion of [(99m)Tc]pertechnetate on salivary gland scintigraphy were well seen in patients with Sj?gren syndrome. For the diagnosis of Sj?gren syndrome, salivary gland scintigraphy showed higher sensitivity than MR sialography. On the other hand, MR sialography showed higher specificity and positive predictive value (PPV) than salivary gland scintigraphy. Overall diagnostic accuracy was 83% for MR sialography and 72% for salivary gland scintigraphy. CONCLUSION: The high PPV of MR sialography suggests that MR sialography is the preferred imaging modality in patients suspected of having Sj?gren syndrome.     

Scoring analysis of salivary gland scintigraphy in patients with Sjögren’s syndrome

The purpose of the present study was to evaluate the validity of a scoring system based on excretion rate of salivary gland scintigraphy in patients with Sjögren’s syndrome (SjS). Total scintigraphic scores were compared with the results of the Saxon test. One hundred and twenty-four subjects who were clinically diagnosed with SjS and 11 normal ones underwent salivary gland scintigraphy and the Saxon test. In salivary gland scintigraphy, the difference between maximum and minimum counts after stimulation using vitamin C divided by maximum counts was defined as the excretion rate. We then defined a scoring system with 4 grades: severe dysfunction = 3 (excretion rate < 25%), moderate dysfunction = 2 (25% ≤ excretion rate < 40%), mild dysfunction = 1 (40% ≤ excretion rate < 50%) and normal function = 0 (50% ≤ excretion rate). The summation of the total scintigraphic score (0–12) of all 4 salivary glands was used as a semi-quantitative index indicating total salivary gland function, and total scintigraphic scores were compared with the results of the Saxon test. A significant inverse linear correlation (R² = 0.95) was observed between total scintigraphic scores and mean values of the Saxon test within a range of abnormal scintigraphic scores (≥ 4). The scoring system developed in the present study is a clinically available, objective, and reproducible method for evaluation of salivary gland function in patients with SjS.     

Paired inspiratory–expiratory thin-section CT findings in patients with small airway disease

The objective of this study was to evaluate minimal small airway disease (SAD) as reflected on paired inspiratory–expiratory CT findings. Seventy-two subjects, 34 with SAD, 11 with normal lung function, and 27 with chronic obstructive pulmonary diseases (COPD), underwent thin-section CT during deep inspiration and expiration at upper, middle, and lower lung levels. Evaluation of CT measurement was performed between inspiratory and expiratory CT attenuation of lung parenchyma, in non-dependent and dependent lung at each of the three levels. Visual assessment of mosaic patterns and attenuation differences was also performed using the inspiratory and expiratory images. Patients with SAD were differentiated from those with COPD, by visual assessment and from the CT measurements. Visual assessment failed to differentiate between the SAD and normal groups. However, one measurement, an inspiratory–expiratory attenuation difference in the dependent lower lung, was different between SAD and normal group. Early small airway disease may be indicated by an inspiratory–expiratory attenuation difference in the dependent lower lung using the simple method of a paired inspiratory–expiratory CT. Received: 10 April 2000 Revised: 7 June 2000 Accepted: 8 June 2000     

Acquired peripheral pulmonary artery aneurysms: morphological spectrum of disease and multidetector computed tomography angiography findings—cases series and literature review

Background

Acquired peripheral or intraparenchymal pulmonary artery aneurysms (PPAA) are rare entities but are important to recognize because of the associated morbidity. Hemoptysis is their principal complication and is a potentially fatal condition.

Purpose

To illustrate the causes, multidetector CT angiography (MDCTA) findings and differential diagnosis of acquired PPAA.

Materials and methods

The institutional review boards approved this study. We conducted a retrospective review of the demographic data and the results of clinical and laboratory examinations, and imaging studies of patients managed between January 2012 and January 2017 in two institutions.

Results

A total of 19 patients had acquired PPAA that were detected at MDCTA, 9 patients with normal pulmonary artery pressures and 10 with pulmonary hypertension. Nine patients developed PPAA-related acute symptoms. MDCTA features of PPAA include: a lobulated vascular mass, an indistinct irregular arterial wall, aneurysmal thrombosis or wall calcification, findings of impending rupture including perianeurysmal edema, gas or a soft tissue mass.

Conclusion

PPAA are rare. In our series, endocarditis and pulmonary hypertension are the PPAA leading causes. The treatment modality preferred is embolization, especially as surgery poses a very high risk for patients with severe pulmonary hypertension. Further clarification of the natural history of these rare arterial aneurysms is needed.