Relationship between nailfold capillaroscopy findings and the etiology and prognosis of interstitial lung disease.

OBJECTIVES: Connective tissue-associated interstitial lung diseases (CTD-ILD) are believed to be caused by microvascular damage. The objective of this study was to assess the nailfold capillaroscopy (NFC) pattern in patients diagnosed with both CTD-ILD and non-CTD-ILD to identify microvascular changes and determine the relation between capillaroscopic parameters, clinical variables, and disease-related measurements. PATIENTS AND METHODS: This cross-sectional study included 95 patients with interstitial lung disease who applied to our Rheumatology and Chest Clinics between September 2021 and July 2023. The patients were divided into two groups based on their diagnosis: non-CTD-ILD (group 1) and CTD-ILD (group 2). Nailfold capillaroscopy was performed. RESULTS: Ninety-five patients, 49 (51% female, mean age 62.31 ± 11.027 years) in group 1 and 46 (69.6% female, mean age 62.09 ± 10.887 years) in group 2, were included in the study. Abnormal capillary morphologies were both detected in the CTD-ILD group and the non-CTD-ILD groups. In patients with a usual interstitial pneumonia (UIP) pattern on chest computed tomography (CT), tortuosity was higher than in patients with non-specific interstitial pneumonia (NSIP) (P = 0.041), and the proportion of tortuosity increased significantly as the duration of the disease increased (P = 0.016). CONCLUSION: Our study highlights capillaroscopic abnormalities alone may not be sufficient to differentiate CTD-ILD (other than systemic sclerosis) from non-CTD-ILD. The presence of NFC abnormalities in non-CTD-ILD may suggest that fibrotic lung disease could potentially play a role in the deterioration of the microvascular structure or abnormal angiogenesis. Our study demonstrated that a multidisciplinary approach, incorporating clinical, morphological, pathological, and serological evaluations, is necessary for interpreting ILD. Key Points • Capillaroscopic abnormalities can also be seen in non-CTD-ILD. • Capillaroscopy findings do not distinguish the non-Ssc etiology of ILD. • Nailfold capillaroscopy may have the potential to serve as a useful tool in predicting prognosis and monitoring the disease progression in patients with idiopathic pulmonary fibrosis (IPF).

Extended autoantibody panel in Turkish patients with early-stage systemic sclerosis: Coexpressions and their influences on clinical phenotypes.

BACKGROUND/AIM: To investigate the frequency and clinical relevance of an extended autoantibody profile in patients with systemic sclerosis (SSc). MATERIALS AND METHODS: In this cross-sectional study, serum from 100 consecutive patients was subjected to indirect immunofluorescence (IIF) (HEp-20-10/primate liver mosaic) and Systemic Sclerosis Profile by EUROIMMUN to evaluate anti-nuclear antibodies (ANA) and autoantibodies against 13 different autoantibodies in patients with SSc less than 3 years. RESULTS: Ninety-three of 100 patients were positive for ANA by IIF. Fifty-three patients showed single positivity, 26 anti-topoisomerase antibodies (anti-Scl70 ab), 16 anticentromere antibodies (ACAs), six anti-RNA polymerase III antibodies (anti-RNAPIII ab), one anti-Ku antibody, one anti-PM/Scl100 antibody, two anti-PM/Scl75 antibodies, one anti-Ro52 antibody, whereas 32 patients had multiple autoantibody positivities. Among classic SSc-specific autoantibodies, anti-Scl70 and anti-RNAPIII abs showed the highest cooccurrence (n = 4). One patient was simultaneously positive for anti-RNAPIII ab and ACA, and one was positive for ACA and anti-Scl70 ab. The clinical features were not statistically different between single and multiple autoantibody-positivity for classic SSc-specific autoantibodies (ACA, anti-Scl70 ab, and anti-RNAPIII ab), except for digital ulcer in the multiantibody positive ACA group (p = .019). CONCLUSION: Based on our results, coexpression of autoantibodies is not uncommon in SSc patients. Although autoantibodies specific to SSc in early disease show generally known clinical features, it remains to be investigated how the coexpression of autoantibodies will affect clinical presentation.

Anti-inflammatory treatment improves systolic and diastolic tissue doppler parameters in patients with newly diagnosed rheumatoid arthritis.

BACKGROUND: The frequency of cardiovascular system involvement is increased in rheumatoid arthritis (RA) and may result in serious morbidity and mortality. Early intervention and control of the disease activity may reduce the risk of cardiovascular events. The purpose of this study is to examine the effects of steroids and methotrexate (Mtx) on the heart functions of newly diagnosed RA patients. METHODS: Our study is a prospective cohort study involving thirty-six newly diagnosed RA patients according to the American Society of Rheumatology classification criteria. Right and left ventricular echocardiography, and Doppler parameters were evaluated in these patients thrice; before treatment, after one month of steroid treatment, and after three months of Mtx treatment, and laboratory/clinical parameters were noted. RESULTS: The mean age of the patients was 52.66 ± 13.66 years. After the treatment, a significant decrease was observed in the values of inflammatory markers (ESR and CRP) and disease activity score (DAS28) [p < .05]. Left ventricular tissue Doppler showed an increase in lateral S, septal S, and mitral S waves compared to baseline (8.37 ± 1.89 vs 10.0 ± 1.8 cm/s p = .001). While there was a decrease in tissue Doppler tricuspid a wave (18.33 ± 4.76 vs 15.63 ± 4.36 p = .016), an increase in Tricuspid E/e' value and Tricuspid tissue Doppler e/a value was detected after treatment (0.76 ± 0.30) vs 0.94 ± 0.53) p < .010). Significant changes were found to be more prominent after the Mtx treatment. CONCLUSION: In RA patients, steroid and Mtx treatment significantly positively affects left ventricular systolic and right ventricular diastolic functions.

Microvascular damage evaluation based on nailfold videocapillarosopy in sarcoidosis.

OBJECTIVES: Microvascular damage is thought to play a role in the pathogenesis of sarcoidosis. We aimed to evaluate the nailfold capillaroscopy (NVC) pattern to detect microvascular changes in patients with sarcoidosis and the relationship of capillaroscopic parameters with clinical variables and disease-related measurements. PATIENTS AND METHODS: Forty-two patients with sarcoidosis and 42 age- and sex-matched patients with systemic sclerosis (SSc) and healthy individuals were included in this cross-sectional case-control study. Patients aged 18-80 years who met the current American Thoracic Society criteria for sarcoidosis were included. NVC was performed by a digital microscope under a magnification of × 200. Capillary density, number of dilated, giant and neoangiogenic capillaries, capillary loop diameter, capillary shape, micro-hemorrhages, and number of avascular areas, were evaluated by an assessor who was blind to the groups allocation. RESULTS: Among the capillaroscopic parameters, neoangiogenesis and dilated capillaries, which can be seen in the pattern of scleroderma, were detected in patients with sarcoidosis but not significantly different from the control group (p = 0.055; p = 0.433, respectively). The rate of tortuosity and crossing capillaries of 50% and above was significantly higher in the sarcoidosis group than in SSc and healthy controls (p < 0.05). In patients with sarcoidosis, the only statistically significant finding was; forced expiratory volume (FEV1) in one second was lower in patients with a crossed capillary rate > 50% than in patients with a crossed capillary rate of less than 50% (FEV1; 87.21 ± 19.3, 102.5 ± 14.8, p = 0.04; respectively). CONCLUSION: Patients with a diagnosis of sarcoidosis have different capillaroscopic patterns. The presence of these nonspecific abnormal patterns may reflect microvascular damage in the pathophysiology of sarcoidosis. Key Points • Microvascular damage may play a role in the pathogenesis of sarcoidosis. • There may be some nonspecific abnormal findings in capillaroscopy findings in sarcoidosis. • Capillaroscopy may be valuable in reflecting sarcoidosis lung injury.

The effect of a home-based orofacial exercise program on oral aperture of patients with systemic sclerosis: A single-blind prospective randomized controlled trial.

OBJECTIVES: This study aims to evaluate the effect of a home-based orofacial exercise program on the oral aperture (OA) of systemic sclerosis (SSc) patients. PATIENTS AND METHODS: This single-blind prospective randomized controlled study performed between March 2017 and January 2019 included 56 SSc patients (3 males, 53 females; mean age 52.9±10.6 years; range, 31 to 70 years) with an OA of <40 mm. Patients were divided into two groups. Group 1 (n=28, mean age 53.8±9.6 years) was given orofacial exercise program twice a day for one month in addition to oral hygiene care advices, followed by no activity for the next month. Group 2 (n=28, mean age 50.0±11 years) received oral hygiene care advices for the first month followed by the same exercise program for the next month. Patients' OA was measured at baseline, and at first and second months. RESULTS: After the first month, OA increased in Group 1 (p<0.001), whereas no change was observed in Group 2 (p=0.579). At the end of two months, there was no additional increase in Group 1 (p=0.352), while there was a significant increase in Group 2 (p<0.001). There was no difference between OAs of the groups at the end of the trial (p=0.564). CONCLUSION: Our results suggest that home-based orofacial exercise program improves OA of SSc patients.

Correlations Between Clinical Features and Mouth Opening in Patients With Systemic Sclerosis.

OBJECTIVES: This study aims to investigate the correlations between clinical features and mouth opening in patients with systemic sclerosis (SSc). PATIENTS AND METHODS: Eighty patients with SSc (13 males, 67 females; mean age 53.39±11.65 years; range, 31 to 76 years) followed in our clinic were enrolled in the study. Skin involvement was assessed using the modified Rodnan skin score (mRSS), joint/tendon involvement with finger-tip to palm (FTP) distance and interstitial lung disease (ILD) was evaluated with clinical and radiologic methods. The interincisal distance (ID) measurement was used to assess the maximal mouth opening capacity. We examined associations between the ID and clinical features of SSc. RESULTS: The ID was lower in females compared with males, and in diffuse type compared with the limited type (p<0.001 and p<0.001, respectively). A significant negative correlation was found between the ID with mRSS and FTP distance (p<0.001 and p=0.001, respectively). The ID was lower in patients with ILD than in patients without ILD (p=0.006). A formula was constituted to predict the maximal mouth opening of the patient with regression analysis. According to the formula, being male was associated with an increase of 6.14 mm, the presence of ILD with a decrease of 3.19 mm, every 10 mm increment in mRSS with a decrease of 3.72 mm and FTP distance >0 mm with a decrease of 5.13 mm in mouth opening. CONCLUSION: Microstomia is associated with low quality of life in patients with SSc. In our study, sex, ILD, mRSS, and FTP distance were observed to be the most important factors that were related with mouth opening in patients with SSc.

Sacroiliitis Detected by Magnetic Resonance Imaging in Patients With Systemic Sclerosis.

OBJECTIVES: This study aims to evaluate the magnetic resonance imaging (MRI) findings of sacroiliac joints in a selected group of patients with systemic sclerosis (SSc). PATIENTS AND METHODS: This retrospective study included 30 patients (2 males, 28 females; mean age 44.1±12.5 years; range, 24 to 70 years) with SSc who underwent MRI of sacroiliac joints. Lesions were defined according to Assessment of SpondyloArthritis International Society (ASAS)/Outcome Measures in Rheumatology MRI group criteria. Clinical features, conventional radiograms of sacroiliac joints, presence of inflammatory back pain, human leukocyte antigen B27 (HLA-B27), and C-reactive protein (CRP) levels were extracted from patient records. RESULTS: Eleven of thirty patients (37%) revealed sacroiliitis on MRI (five with chronic, three with active sacroiliitis, and three with both active and chronic forms). On conventional radiographic examination, six patients had sacroiliitis (20%). In all of these six patients, sacroiliitis was also detected on MRI. CRP levels and number of patients with inflammatory back pain were found to be higher in the patients with active sacroiliitis (p<0.05). Seven patients were diagnosed as spondyloarthritis according to ASAS criteria. CONCLUSION: Sacroiliitis was detected more frequently by MRI compared to conventional radiographic examination. MRI is suggested to be the preferred method for evaluating sacroiliitis in SSc patients.

Malnutrition, associated clinical factors, and depression in systemic sclerosis: a cross-sectional study.

INTRODUCTION: The aim of this study was to evaluate the associations between malnutrition and the clinical features of the disease and depression in patients with systemic sclerosis (SSc). METHOD: Patients with SSc who were followed up in our clinic were enrolled in the study. Malnutrition risk was assessed using the Malnutrition Universal Screening Tool (MUST). Skin involvement was assessed using the modified Rodnan skin score (mRSS) and interincisal distance (ID) measurements were used to assess the maximal mouth opening capacity. The Beck Depression Inventory (BDI) was used for measuring the severity of depression. RESULTS: Ninety-eight patients with SSc (84.7% women; mean age 52.67 ± 11.26 years) were included in the study. According to the MUST scores, 61.2%, 15.3%, and 23.5% of patients had low, medium, and high risk for malnutrition, respectively. The mRSS was significantly higher in the group with high malnutrition risk compared with low-risk group (p = 0.014). Malnutrition risk was associated with interstitial lung disease and bowel involvement (p = 0.044 and p = 0.021, respectively). Interincisal distance was lower in the group with high malnutrition risk compared with the low-risk group (p = 0.003). Malnutrition risk was higher in patients who had mild-to-severe depressive symptoms than in those without (p = 0.012). Interincisal distance and bowel involvement were the most relevant factors for malnutrition. CONCLUSIONS: The risk of malnutrition is increased in patients with SSc. In our study, microstomia and bowel involvement were the most relevantly associated factors with malnutrition. KEY POINTS: • The risk of malnutrition is increased in patients with systemic sclerosis (SSc). • Microstomia and bowel involvement are found to be the most important factors associated with malnutrition. • Depressive symptoms are seen frequently among patients with SSc, and depression seems to be one of the etiologic factors or the result of malnourishment in SSc. • Assessment of nutritional status and the presence of depression should be a part of routine clinical visits of patients with SSc.

The effect of oral and parenteral vitamin D supplementation in the elderly: a prospective, double-blinded, randomized, placebo-controlled study.

Hypovitaminosis D in the elderly causes falls and fractures as a result of impaired neuromuscular functions and also may be a reason for nonspecific musculosceletal pain. The aim of this study is to investigate the benefits of a single dose per os or parenterally administrated vitamin D on increasing the quality of life and functional mobility and decreasing the pain in the elderly. The community-dwelling elderly subjects over 65 years age were included in the study. The subjects were given 300.000 IU Vitamin D via per os and parenteral route and assessed after 4 weeks. The serum creatinine, calcium, phosphorous, ALT, ALP, 24-h urine calcium excretion, PTH, and vitamin D levels, as well as VAS (visual analog scale) for pain assessment, functional mobility with TUG (timed up and go test) and quality of life with SF-36 before and after the treatment were evaluated. The serum vitamin D levels were measured by the RIA method. The subjects were divided into four groups each consisting of 30 subjects. The 1st group took i.m. vitamin D, the 2nd group took i.m. placebo, the 3rd group took p.o. vitamin D, and the 4th group took p.o. placebo. The mean age of all the participants was 70.1 ± 4.3 years. There was no difference in the age and gender between the groups (P > 0.05). After treatment, the PTH level of first group was decreased (P = 0.0001) and the vitamin D level increased (P = 0.0001) significantly. In the third group, the PTH level of first group was decreased (P = 0.0001) and the vitamin D level increased (P = 0.004) and the 24-h calcium excretion in urine (P = 0.015) increased significantly. When the pain, the functional mobility, and the quality of life were evaluated, in the first group, the TUG (P = 0.0001) and the VAS (P = 0.0001) decreased significantly, whereas the SF-36 subtitles: physical functioning (P = 0.0001), role physical (0.006), bodily pain (P = 0.0001), general health (P = 0.007), social functioning (P = 0.05), and mental health (P = 0.048) increased significantly. In group two, the VAS (P = 0.001) decreased, the role physical (P = 0.009), and role emotional (P = 0.034) increased significantly; In group three, the TUG (P = 0.0001) and the VAS (P = 0.002) decreased, whereas the physical function (P = 0.0001) and role physical (0.001) increased significantly; In group four, the VAS (P = 0.007) decreased significantly. The megadose vitamin D administration increases quality of life, decreases pain, and improves functional mobility via po or im route in the elderly.

High-frequency sensorineural hearing loss in patients with ankylosing spondylitis: is it an extrarticuler feature of disease?

OBJECTIVE: The aim of this study was to investigate the ear involvement, especially at extended higher frequencies than those previously studied, in patients with ankylosing spondylitis (AS). PATIENTS AND METHODS: We prospectively evaluated 45 consecutive patients with AS. All patients underwent a complete physical examination of the ear, nose, and throat and an audiologic evaluation that included pure-tone audiometry at conventional and extended high frequencies, the determination of a speech discrimination score and the uncomfortable loudness level, and impedance audiometry. Thirty healthy volunteers were included as controls. RESULTS: The mean age of the patients was 39.6 +/- 9.1 years (range 19-63 years) and that of the controls was 10.6 +/- 8.1 years (range 1-30 years). There was no statistically significant difference between the two groups with respect to conventional frequency air conduction threshold and bone conduction threshold. There was a statistically significant difference at 14,000-16,000 Hz at extended high frequencies in 32 patients with AS (71.1%) versus 12 controls (40%). At 14,000-16,000 Hz, eight patients demonstrated a sensorineural hearing loss caused by extraspinal involvement. There was a significant difference between the patients with or without extraspinal involvement, and a positive correlation was noted between the duration of disease and the hearing level at 10,000-16,000 Hz. CONCLUSION: Sensorineural hearing loss, especially at extended high frequencies, is common in patients with AS and may be an extra-articular feature of that disease. A long duration of disease and extraspinal involvement are important parameters for ear involvement in patients with AS.

Echocardiographic findings in patients with Behçet's disease.

Case-controlled studies have shown that the prevalence of left ventricular (LV) diastolic dysfunction is higher in patients with Behçet's disease. However, there are no data evaluating the value of Doppler tissue imaging (DTI) in Behçet's disease. The aim of this study was to evaluate the cardiac involvement and the LV function at rest and at the end of isometric exercise by pulse-wave Doppler and DTI methods in patients with Behçet's disease. Fifty-four patients with Behcet's disease and 50 control subjects were studied. Dimensions of cardiac chambers, aortic root dimension, valvular abnormalities, and systolic function were similar in both groups. The mean E/A ratio was significantly lower in patients than controls (1.22 +/- .09 vs 1.36 +/- 0.30, p = 0.01). The E/A ratio was <1 in 12 patients (22%) and in 3 controls (6%) (p = 0.02). By DTI, no difference was found in the mean S-, e-, and a-wave velocities, and e/a ratio between the 2 groups. The e/a ratio was <1 in 13 patients (24.1%) and in 7 controls (14%) (p = 0.2) by DTI. There were no differences in the mitral pulse-wave Doppler and DTI parameters in patient and control groups at the end of exercise. The prevalence of cardiac pathology in Behçet's disease did not differ appreciably from the controls. In conclusion, the LV systolic and diastolic functions in the patients with Behçet's disease and controls were similar not only at rest but also at the end of exercise.

Budd-Chiari syndrome associated with visceral leishmaniasis and factor V Leiden mutation.

We here report a case of subacute Budd-Chiari syndrome (BCS) related to Factor V Leiden (FVL) mutation in the presence of visceral leishmaniasis. A 17-year-old man was admitted to hospital because of abdominal pain, pretibial edema and fever. The clinical picture of BCS had been developed within several months. BCS was diagnosed by radiographic examination. On DNA analysis, a heterozygote Arg506Gln mutation in the factor V gene was found. Histological examination of the bone marrow showed intracellular leishmania amastigotes. Despite appropriate treatment patient's clinical condition deteriorated rapidly and died with multiorgan failure. FVL mutation is the most common procoagulant disorder and account for many cases of BCS. This case report demonstrates that in addition to duration and severity of the disease accompanying conditions including infections are prognostically significant for the outcome of this potentially lethal disease.

Methylenetetrahydrofolate reductase gene C677T mutation and plasma homocysteine level in Behçet's disease.

OBJECTIVE: The aim of this study was to assess whether homozygosity for the 5, 10-methylenetetrahydrofolate reductase (MTHFR) C677T mutation and plasma homocysteine concentration are related to deep vein thrombosis in Behçet's disease (BD) patients. METHODS: Forty BD patients (23 males, 17 females; mean age 40.2+/-8.4 years) and 60 healthy controls (HC) (34 males, 26 females; mean age 41.6+/-6.9 years) were included in the study. Fourteen of the BD patients had a history of deep venous thrombosis (DVT), as confirmed by Doppler ultrasound. RESULTS: The rates of homozygosity for the MTHFR C677T mutation in the BD and HC groups were 7.5% and 10%, respectively. The distribution of MTHFR genotypes was similar in the two groups ( p>0.05), and analysis showed that homozygosity for the mutation was not a risk factor for DVT. The mean plasma homocysteine levels were 13.4+/-4.2 micro mol/l for the overall BD patients and 12.6+/-3.8 micromol/l for HC ( p>0.05). However, the mean plasma homocysteine level in the BD patients with DVT history (15.9+/-4.6 micromol/l) was significantly higher than the level in the BD patients with no DVT history (12.1+/-3.3 micromol/l) ( p=0.013) and the level in the HC group (12.6+/-3.8 micromol/l) ( p=0.025). CONCLUSION: The study results suggest that elevated plasma homocysteine level may play a role in the pathogenesis of venous thrombosis in BD.