Unveiling cancer risk in ANCA-associated vasculitis: result from the Turkish Vasculitis Study Group (TRVaS).

To investigate cancer incidence in patients with ANCA-associated vasculitis (AAV), compare it with the age/sex-specific cancer risk of the Turkish population, and explore independent risk factors associated with cancer. This multicenter, incidence case-control study was conducted using the TRVaS registry. AAV patients without cancer history before AAV diagnosis were included. Demographic and AAV-related data of patients with and without an incident cancer were compared. Standardized cancer incidence rates were calculated using age-/sex-specific 2017 Turkish National Cancer Registry data for cancers (excluding non-melanoma skin cancers). Cox regression was performed to find factors related to incident cancers in AAV patients. Of 461 AAV patients (236 [51.2%] male), 19 had incident cancers after 2022.8 patient-years follow-up. Median (IQR) disease duration was 3.4 (5.5) years, and 58 (12.6%) patients died [7 with cancer and one without cancer (log-rank, p = 0.04)]. Cancer-diagnosed patients were older, mostly male, and more likely to have anti-PR3-ANCA positivity. The cumulative cyclophosphamide dose was similar in patients with and without cancer. Overall cancer risk in AAV was 2.1 (SIR) ((1.3-3.2), p = 0.004); lung and head-neck [primary target sites for AAV] cancers were the most common. In Cox regression, male sex and ≥ 60 years of age at AAV diagnosis were associated with increased cancer risk, while receiving rituximab was associated with decreased cancer risk. Cancer risk was 2.1 times higher in AAV patients than the age-/sex-specific cancer risk of the Turkish population population, despite a high rate of rituximab use and lower dose of cyclophosphamide doses. Vigilance in cancer screening for AAV patients covering lung, genitourinary, and head-neck regions, particularly in males and the elderly, is vital.

Neuropathic pain in axial spondyloarthropathy is underdiagnosed and a confounding factor in biologic drug-switching decision: a cross-sectional study.

OBJECTIVES: The aim of this study was to evaluate the relationship between the presence of neuropathic pain (NeP), disease activity scores and biologic drug-switching decisions in the subjects with axial spondyloarthritis (axSpA) receiving biologic treatment. METHODS: PainDETECT Questionnaire was used to evaluate the presence of NeP in the patients with axSpA aged ≥18 years who had been receiving biologic treatment for at least 6 months. The relationships between disease activity scores, inflammatory markers, life quality index, biologic drug-switching decisions and the presence of NeP were analyzed. RESULTS: A total number of 175 patients with axSpA [ankylosing spondylitis (AS) (n:150) and non-radiographic axSpA (nr-axSpA) (n:25)] were enrolled in the study. NeP was detected in 41.7% of the patients and it was more common in females than in males (p:0.009). PainDETECT scores were positively correlated with disease activity scores, but they were not correlated with inflammatory marker levels. NeP was found to be significantly more common in whom the biologics had been switched 3 or more times (p:0.007). PainDETECT scores were higher and NeP was more prevalent (p:0.028) in the patients for whom drug-switching decisions had been made due to primary or secondary unresponsiveness. CONCLUSION: NeP is more common than estimated in the patients with axSpA and current disease activity scores are insufficient to make a distinction between NeP and inflammatory pain. NeP is a confounding factor in the evaluation of treatment response to biologic agents. In the subjects with AS and nr-axSpA with primary or secondary treatment unresponsiveness, the presence of NeP must be considered before biologic drug-switching decisions. Key Points • Neuropathic pain (NeP) is common in subjects with AxSpA treated with multiple biologic agents. • Current disease activity scores for AxSpA are insufficient to make a differentiation between NeP and inflammatory pain. • NeP is a confounding factor in the evaluation of treatment response to biologic agents. • Patients with AxSpA should be re-evaluated in terms of the presence of neuropathic pain before making biologic drug-switching decisions.

Pneumomediastinum, pneumatosis intestinalis and pneumoperitoneum in a patient with polymyositis: case-based review.

Pneumomediastinum (PnM), pneumatosis intestinalis (PI), and pneumoperitoneum (PP) are rare complications of inflammatory myositis. We present a 59-year-old polymyositis (PM) patient who experienced all three complications simultaneously. The patient who presented with proximal muscle weakness, dysphagia, and weight loss was diagnosed with PM due to elevated muscle enzymes and consistent electromyography and muscle biopsy with inflammatory myopathy. On the 45th day of her immunosuppressive treatment, PnM, PI, and PP were detected incidentally in 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET)/computed tomography (CT) scan performed for severe weight loss and treatment-resistant severe disease. Since the patient had no symptoms or signs of PnM and PP, no additional intervention was applied to the current treatment, and spontaneous regression was observed in the follow-up. In addition to this case, we reviewed patients with PM who developed PBM, PP, and PI in the literature.

The risk of cancer in patients with primary Sjögren syndrome: a single-center study from Turkey.

BACKGROUND: The aim of this study is to determine the risk of cancer in patients with primary Sjögren syndrome (pSS) from a single center in Turkey. METHODS: Clinical data of the subjects with pSS were retrospectively analyzed. The incidence of cancer for general population was obtained from GLOBOCAN 2018. Age- and sex-specific standardized incidence ratios (SIR) of solid and hematological cancers were calculated compared with the general population. RESULTS: Four hundred thirty patients with pSS were included in the study. The majority of the patients were female (n = 396, 92.1%), and the mean age was 58.6 ± 12.0 years. Thirty-four patients (7.9 %) were diagnosed with cancer (26 solid and 8 hematological) during follow-up. The SIR for all cancers was 2.45 (95% CI, 1.625-3.275). The SIR was 2.42 (95% CI, 1.542-3.298) for solid cancers and 8.42 (95% CI, 2.394 - 14.446) for hematological cancers. The most diagnosed malignancies were breast cancer (n = 6), ovarian cancer (n = 6), and non-Hodgkin lymphoma (NHL) (n = 4). There was an increased risk for ovarian cancer (SIR 12.76, 95% CI, 2.545-22.975). The SIR values were 2.08 (95% CI, 0.419-3.741) and 10.81 (95% CI, 0.216-21.404) for breast cancer and NHL, respectively. DISCUSSION: The risk of hematological and solid cancers was higher in the patients with pSS when compared to general population. In our pSS cohort, the risk for ovarian cancer was found to be increased, which has not been previously reported in the literature.

The Diagnostic Value of Fluoro-18 Fluorodeoxyglucose (F-18 FDG) PET/CT in Fever or Inflammation of Unknown Origin: A Retrospective Study at a Rheumatology Clinic.

Introduction Further diagnostic procedures are necessary for patients with fever of unknown origin (FUO) and unknown cause of inflammation (inflammation of unknown origin - IUO) for the identification of the definitive diagnosis. The aim of this study was to evaluate the contribution and roles of F-18 FDG PET/CT (fluoro-18 fluorodeoxyglucose-positron emission tomography/computed tomography) in the diagnostic process of patients with FUO/IUO. Methods The data of 58 patients who had F-18 FDG PET/CT scans for FUO/IUO were re-evaluated retrospectively. The relationships between definitive diagnosis and fluorodeoxyglucose uptake and SUVmax (maximum standardized uptake value) were examined. Results  Rheumatic disease was diagnosed in 26 patients (44.5%), malignancy in 20 patients (34.5%), and infectious diseases in six patients (10.3%). The most prevalent rheumatic disease in patients with FUO/IUO was systemic vasculitis (n:10, 17.2%), especially large vessel vasculitis. There were 37 patients (63.7%) with clinically significant true positive fluorodeoxyglucose uptake. True positive fluorodeoxyglucose uptake was significantly higher in patients diagnosed with malignancy (85%, 17/20 patients) compared to other diagnoses. Fluorodeoxyglucose uptake above physiological levels was determined in 15 of the 26 patients (57.6%) diagnosed with rheumatic diseases. Conclusion The results of this study showed that F-18 FDG PET/CT is a useful imaging modality in FUO/IUO patients, who present a challenging diagnostic process for clinicians. In addition to malignancies, the presence of chronic inflammatory diseases, especially early period systemic vasculitis, were diagnosed in these patients.

The use of anti-interleukin-1 agents and tumor necrosis factor-alpha inhibitors in renal transplant recipients.

OBJECTIVES: The aim of this study was to investigate the efficacy and safety of anti-interleukin-1 (anti-IL-1) agents and tumor necrosis factor-alpha (TNF-α) inhibitors in renal transplant patients. PATIENTS AND METHODS: Between February 2014 and February 2020, data of 12 renal transplant recipients (9 males, 3 females; median age: 51 years; range, 19 to 70 years) who received anti-IL-1 agents or TNF-α inhibitors for inflammatory diseases in the post-transplant time period and were followed in a single transplant center (n=12) were retrospectively analyzed. A total of 46 cases were reported in the literature, before the data were collected. The overall outcomes of all cases were analyzed in this study. RESULTS: Thirty-seven patients received anti-IL-1 agents in the post-transplant period. The main indications for anti-IL-1 agents were familial Mediterranean fever (FMF) and amyloidosis (75.7%). The continuation rate of colchicine treatment in patients with FMF was 85.7%. Anti-IL-1 agents prevented attacks completely in 89.3% of FMF patients. The number of cases used TNF-α inhibitors among renal transplant patients was lower (n=21). The TNF-α inhibitors were used mainly for inflammatory bowel diseases (57.1%) and ankylosing spondylitis (33.3%) and suppressed the disease activity in most of the patients with inflammatory diseases (72.7%). Death (n=3) and malignancies (n=3) were reported in patients who received TNF-α inhibitors, but not in patients who received anti-IL-1. The renal outcomes and graft survival rates were satisfactory in patients who received both anti-IL-1 agents and TNF-α inhibitors. CONCLUSION: Our results support that anti-IL-1 agents can be used effectively and safely in renal transplant patients.

The risk of cancer in patients with primary Sjögren syndrome; A single-center study from Turkey.

BACKGROUND: The aim of this study is to determine the risk of cancer in the patients with primary Sjögren syndrome (pSS) from a single-center in Turkey. METHODS: Clinical data of the subjects with pSS were retrospectively analyzed. The incidence of cancer for general population was obtained from GLOBOCAN 2018. Age- and sex-specific Standardized Incidence Ratios (SIR) of solid and hematological cancers were calculated compared with the general population. RESULTS: Four hundred thirty patients with pSS were included in the study. The majority of the patients were female (n=396, 92.1%), and the mean age was 58.6 ±12.0 years. Thirty-four patients (7.9 %) were diagnosed with cancer (26 solid and 8 hematological) during follow-up. The SIR for all cancers was 2.45 (95% CI, 1.625- 3.275). The SIR was 2.42 (95% CI, 1.542-3.298) for solid cancers and 8.42 (95% CI, 2.394 - 14.446) for hematological cancers. The most diagnosed malignancies were breast cancer (n=6), ovarian cancer (n=6), and non-Hodgkin lymphoma (NHL) (n=4). There was an increased risk for ovarian cancer (SIR 12.76; 95% CI, 2.545-22.975). The SIR values were 2.08 (95% CI, 0.419-3.741) and 10.81 (95% CI, 0.216-21.404) for breast cancer and NHL, respectively. CONCLUSION: The risk of hematological and solid cancers was higher in the patients with pSS when compared to general population. In our pSS cohort, the risk for ovarian cancer was found to be increased, which has not been previously reported in the literature.

The effects of biological agents on vascular structural lesions in Takayasu's arteritis.

OBJECTIVES: The aim of the present study was to evaluate the effects of biological disease-modifying antirheumatic drugs (bDMARDs) administered to patients with Takayasu's arteritis (TAK) on disease activity and vascular damage. METHODS: This study included TAK patients who were receiving bDMARDs for at least six months. Disease activity (National Institutes of Health [NIH]), vascular lesions, and vascular damage (Combined Arteritis Damage Score [CARDS]) scores were determined. RESULTS: There were 21 TAK patients who received infliximab (INF) and/or tocilizumab (TCZ) (mean age = 38.6±11.8 years; female proportion = 20 [95.2%]). The erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) level, and NIH disease activity score were found to significantly decrease with bDMARD treatments. There were also significant decreases in the mean CARDS and the total number of vascular lesions after treatment (p<0.05). Unlike occlusions, an important decrease was observed in the occurrences of stenosis and aneurysms with bDMARD treatments. Regression was detected in the vascular lesions of 15 (71.4%) patients compared to the last image before bDMARD therapies. CONCLUSIONS: Our study results indicate that biological agents, such as INF and/or TCZ, that are used in the treatmentof TAK are capable of remedying certain vascular lesions and may provide additional benefits to patients with TAK who do not sufficiently respond to conventional synthetic disease-modifying antirheumatic drug (DMARD) treatment.

Disease characteristics of psoriatic arthritis patients may differ according to age at psoriasis onset: cross-sectional data from the Psoriatic Arthritis-International Database.

OBJECTIVES: To explore the impact of early versus late-onset psoriasis (PsO) on the disease characteristics of psoriatic arthritis (PsA) in a large-multicentre cohort. METHODS: The data from a multicentre psoriatic arthritis database was analysed. Patients were grouped according to age at psoriasis onset (early onset; <40 years of age, late-onset; >40 years of age) and disease characteristics of the groups were compared by adjusting for BMI and PsA duration, where necessary. RESULTS: At the time of analyses, 1634 patients were recruited [62.8% females; early onset 1108 (67.8%); late-onset, 526 (32.2%)]. The late-onset group was more over-weight [66.8% vs. 86.8%, p<0.001; adjusted for age - aOR 1.55 (1.11-2.20; 95% CI)]. The early onset group had more scalp psoriasis at onset (56.7% vs. 43.0%, p<0.001), whereas extremity lesions were more common in the late-onset group (63.8% vs. 74.2%, p<0.001). Axial disease in males and psoriatic disease family history in females were significantly higher in the early onset group [38.0% vs. 25.4%; p=0.005; adjusted for PsA duration - aOR 1.76 (1.19-2.62; 95% CI) / 39.5% vs. 30.1%; p=0.003; OR 1.51 (1.15-1.99; 95% CI), respectively]. Psoriatic disease activity parameters, patient-physician reported outcomes and HAQ-DI scores were similar in both groups. CONCLUSIONS: Clinical features of PsA may be affected by the age at onset of PsO. Different genetic backgrounds in early and late-onset PsO may be driving the differences in psoriasis and PsA phenotypes.

Clinical and serological characteristics of seronegative primary Sjögren's syndrome: a comparative study.

OBJECTIVES: This study compared the clinical and serological characteristics of seronegative and seropositive primary Sjögren syndrome (pSS) and examined whether current classification criteria for pSS cover seronegative pSS. METHODS: The study group comprised 375 patients (341 women and 34 men) diagnosed with pSS. A clinical diagnosis by an expert rheumatologist was considered the "gold standard" for the diagnosis of pSS. The clinical and serological characteristics of the patients were retrospectively collected from hospital medical files. RESULTS: Fifty-eight of the 375 pSS patients (15.5%) were seronegative for ANA, RF, anti-Ro, and anti-La autoantibodies. Seronegative pSS was diagnosed based on lymphocytic infiltrations in lip biopsy samples. There were no statistically significant differences in terms of patient age, age at diagnosis, sex distribution, clinical features, and laboratory findings between seronegative and seropositive pSS. The frequency of hypergammaglobulinemia was higher in seropositive pSS. The 2016 ACR/ULAR criteria best covered most seronegative pSS cases (84.5%). For seronegative pSS, the agreement between the 2002 AECG, 2012 ACR, and 2016 ACR/EULAR criteria was relatively low. CONCLUSIONS: The clinical features of seronegative pSS (i.e., a lack of four autoantibodies in serum) were similar to those of seropositive pSS. The current classification criteria for pSS should not be used in the diagnosis of seronegative pSS, as the agreement between the different sets of criteria was low, and some patients fell outside the classification. Further clinical and laboratory studies are needed to identify the features that distinguish seronegative pSS. Key Points • Approximately 15% of the pSS patients were seronegative for ANA, RF, anti-Ro, and anti-La autoantibodies. • Seronegative pSS was diagnosed based on lymphocytic infiltrations in lip biopsy samples. • The clinical features of seronegative pSS were similar to those of seropositive pSS. • The current classification criteria for pSS should not be used in the diagnosis of seronegative patients, as the agreement between the different sets of criteria was low, and some patients fell outside the classification.

Survival analysis of patients with Sjögren's syndrome in Turkey: a tertiary hospital-based study.

OBJECTIVES: This study was conducted to determine long-term survival rates and the factors associated with mortality in Turkish primary Sjögren syndrome (pSS) patients. METHODS: All patients diagnosed with pSS between 2004 and 2014 were included in this study. By January 2019, all subjects still living by the end of the study, as well as any death, were identified. Survival rates and standard mortality rates (SMRs) using general population mortality data were calculated. Mortality-related factors were determined by univariate and multivariate analysis. RESULTS: During follow-up, 33 cases of 372 pSS patients resulted in death (8.9%). Of those patients, they were typically older at disease onset, at recruitment, and had shorter follow-up times (p < 0.001 for all). The overall SMR of all pSS patients compared with the general population was 2.11 (95% confidence interval (CI) 1.39-2.83). Male pSS patients had a higher SMR than that of general male patients. Overall survival rates were 97.8% at five years, 90.2% at 10 years, and 87.1% at 15 years in patients with pSS. The survival rate of pSS patients was significantly lower than the general Turkish population (p = 0.011). Multivariate Cox regression analysis showed that older age at disease onset and the presence of interstitial lung disease (ILD) were independent risk factors for mortality. CONCLUSIONS: Based on these data, mortality rates of Turkish pSS patients are higher compared with the general population. Survival significantly decreased in the pSS patients with ILD, especially in older male patients at disease onset. Male gender and malignancy may also be associated with a worse prognosis in pSS patients.Key Point• Mortality in Sjögren's syndrome.

The Role of Real-Time Tissue Elastography and Gray-Scale Ultrasound Histogram Analysis in the Diagnosis of Patients With Sjögren's Syndrome.

OBJECTIVES: This study aims to evaluate gray-scale histogram analysis of B-mode ultrasound (US) images and US elastographic features of the parotid glands in patients with Sjögren's syndrome (SS) and to explore relationships with the ultrasonographic and disease activity scores in the light of histopathological findings. PATIENTS AND METHODS: A total of 57 consecutive female patients (mean age 47.9±10.4 years; range 25 to 76 years) with a diagnosis of SS and 48 healthy female individuals (mean age 51.1±10.8 years; range 20 to 70 years) underwent parotid ultrasonography and real-time tissue elastography imaging. Quantitative measurements of gray-scale US images were performed using the histogram software of the scanner. The histogram ratios of the parotid glands from both sides were obtained (histogram ratio; mean gray-scale histogram parotid/mean gray-scale histogram fat). Strain ratio (SR; ratio of fat to gland parenchyma) was calculated from the color-coded images. Subjective B-mode US scoring of electronically recorded gray- scale US images was performed by two radiologists independently for intra- and inter-observer agreement. Subjective assessments, quantitative measurements, and clinical parameters were compared. RESULTS: The SR of the patient group (1.4±0.8 right side, 1.5±0.9 left side) was significantly higher than that of the control group (1.0±0.3 right side, 1.1±0.3 left side) (p<0.05). The gray-scale histogram ratio of the patient group (1.3±0.5 right side, 1.4±0.9 left side) was lower than that of the control group (1.8±0.7 right side, 1.9±0.7 left side) (p<0.05). Receiver-operating-characteristics curve yielded 66% sensitivity for both sides and 50% and 52% specificity for the right and left sides, respectively, for a cut-off SR of 1.02; 76% and 86% sensitivity for the right and left sides, respectively, and 63% specificity for both sides for a cut-off histogram ratio of 1.35. The quantitative histogram ratio method had a higher positivity rate for the diagnosis of abnormal parotid glands than subjective assessments of US images. CONCLUSION: Sonoelastography and gray-scale histogram analysis of the parotid glands may be used as auxiliary tools to detect parotid gland sonographic abnormalities in patients with SS.

Brain abscess caused by Micrococcus luteus in a patient with systemic lupus erythematosus: case-based review.

Central nervous system infections, which are rarely seen in systemic lupus erythematosus (SLE), have considerably high mortality but they are difficult to distinguish from neuropsychiatric manifestation of lupus. This article reports the case of a patient with SLE with brain abscess which developed during immunosuppressive therapy for lupus nephritis. The patient completely recovered without neurological sequelae by open surgical drainage and 12-week antibiotic therapy. It is recommended that CNS infections must be excluded in patients with SLE, particularly who are receiving immunosuppressive therapy.

The Frequency of Primary Sjogren's Syndrome and Fibromyalgia in Irritable Bowel Syndrome.

OBJECTIVE: To determine the frequency of sicca complex, Sjogren's Syndrome (SS) and Fibromyalgia (FM) in patients with Irritable Bowel Syndrome (IBS). METHODS: Seventy seven IBS patients who fulfilled the Rome-III criteria were included in the study. All patients were assessed for FM according to the American College of Rheumatology (ACR) 2010 criteria. After examination for objective evidence of sicca complex by Schirmer test, TBUT and Ocular Staining Score (OSS), serological tests were performed. And the diagnosis of SS was made according to the American College of Rheumatology (ACR) classification criteria for SS - 2012. RESULTS: Thirteen (16.9%) of IBS patients had FM. Dry eye was detected in 20(26.0%), 7(9.1%) and 29(37.7%) patients by OSS, Schirmer test and TBUT, respectively. Of 77 patients with IBS, the diagnosis of SS was established in two patients (2.6%). CONCLUSION: The frequency of Sjogren's Syndrome among patients with IBS is relatively higher than the general population. All IBS patients should be questioned for dryness of the mouth and eyes, and if necessary, should be evaluated for SS.

Primary Sjögren's syndrome in patients with celiac disease.

BACKGROUND/AIM: Many autoimmune diseases occur concomitantly with celiac disease (CD). We aimed to determine the frequency of Sjögren's syndrome (SS) in CD patients based on SS-specific serology verified by minor labial salivary biopsy. MATERIALS AND METHODS: Eight-two patients with CD were included in the study. After examination for objective evidence of sicca complex, all patients were tested for serological presence of rheumatoid factor (RF) and antinuclear antibodies (ANAs) and for ANA profile. Minor labial salivary biopsy was performed for patients with positive serology and/or clinical signs of SS. RESULTS: Of the patients included, 24 (29.3%) had dry eye symptoms while 20 (24.4%) had dry mouth symptoms. Dry eye was detected by Schirmer test in 10 patients (12.2%) and by ocular staining score in only 2 patients (2.4%). All samples were negative for RF while 12 (14.6%) samples were positive for ANAs. Of 82 patients with CD, the diagnosis of SS was established in only one patient (1.2%), while one patient (1.2%) was diagnosed with morphea and 4 patients (4.9%) were classified as having undifferentiated connective tissue disease. CONCLUSION: The prevalence of SS in CD is low, so there is no need for serologic screening of all patients with CD for SS.

Leptin and leptin receptors in salivary glands of primary Sjögren's syndrome.

BACKGROUND: The role of leptin in primary Sjögren's syndrome (SS) pathogenesis is unknown. The aim of this study was to investigate the expression of leptin and leptin receptor (LEPR) in minor salivary glands in patients with SS. MATERIALS AND METHODS: The expression of leptin and LEPR in minor salivary gland specimens obtained from patients with primary SS (n=50) and control subjects (n=50) were examined using immunohistochemical staining. RESULTS: Acinar cells, epithelial cells and adipocytes in salivary glands can express leptin and LEPR. It was observed that there was intense staining in the focal lymphocytic infiltration areas in SS patients. The intensity of leptin and LEPR staining under microscopy (400×) were graded semiquantitatively as negative, mild, moderate or strongly positive, and scored as 1, 2 or 3, respectively. The expression levels of leptin and LEPR in patients with primary SS were not higher than in controls. There was no significant difference in degrees of leptin and LEPR staining, staining intensity, and immunoreactive scores between groups. The expression of leptin and LEPR were not correlated with autoantibodies such as RF, ANA, anti-Ro, and/or anti-La positivity. CONCLUSIONS: These findings indicate that leptin and its receptors do not play an important role in primary SS pathophysiology.

Lung involvement in patients with primary Sjögren's syndrome: what are the predictors?

The aim of this study was to investigate the prevalence, predictors and radiological findings of primary Sjögren's syndrome (pSS)-associated lung involvement. This retrospective cohort study included 123 patients with demographic, clinical, laboratory and radiological data who were diagnosed with pSS. Lung involvement was defined based on the presence of pulmonary signs/symptoms and/or impaired pulmonary function tests along with alterations in high-resolution computerized tomography (HRCT). Thirty patients (24.4%) had pulmonary signs/symptoms at the initial presentation and/or during the follow-up period. Based on the criteria, 14 patients (11.4%) were defined as having pSS with lung involvement. The smoking rate, male/female ratio and the mean ages were found to be higher in patients with lung involvement (P < 0.05). Positive IgM-rheumatoid factor (RF), anti-La and anti-Ro results, the presence of hypergammaglobulinemia and lymphopenia had high specificity despite the low sensitivity rates to detect pSS-associated lung disease. A significant difference was found in forced vital capacity (FVC) and forced expiratory volume in 1 s (FEV(1)) results between the patients with and without lung involvement. Impaired FEV(1) had high specificity and positive predictive value compared to impaired FVC, particularly in non-smoker patients. The most frequent HRCT finding was ground-glass attenuation (64.3%). Other common findings were bronchiectasis, reticular pattern and honeycombing. The lesions involved predominantly the lower lobes. In conclusion, the presence of hypergammaglobulinemia and lymphopenia, positivity for RF, anti-La and anti-Ro, and impaired (FVC) and/or FEV(1) values could be the predictive parameters with a high specificity despite the low sensitivity rates. Smoking history, male gender and age are also risk factors. These parameters may be helpful to distinguish pSS-associated lung involvement from lung disorders unrelated to pSS.

A case of primary Sjögren's syndrome with pulmonary-limited Wegener's granulomatosis.

A 60-year-old woman had a history of dyspnea for 5-6 weeks. The chest radiograph and computed tomography scans revealed bilateral patchy reticulonodular pattern. The patient had positive test results for antineutrophil cytoplasmic antibody against proteinase-3 (c-ANCA), antinuclear antibody and anti-Ro antibody. According to European Study Group on Classification Criteria for Sjögren's Syndrome, the patient was diagnosed as primary Sjögren's syndrome based on the presence of clinical features, positive findings on Schirmer's test and parotis scintigraphy. Lung biopsy obtained by wedge resection showed granulomatous inflammation with extensive multinuclear giant cells involving the lung parenchyma and vascular structures. There was neither upper airway nor renal involvement. Thus, the patient was simultaneously diagnosed as pulmonary-limited Wegener's granulomatosis. With this unique case, we would like to emphasize that the awareness of ANCA-associated vasculitis as a diagnostic possibility in primary Sjögren's syndrome is important during the work-up of lung lesions.