Interstitial Lung Disease in Systemic Sclerosis: A Single-Center Retrospective Analysis

Background: Systemic sclerosis (SSc) is a systemic autoimmune disease characterized by microangiopathy, inflammation, fibrosis. Interstitial lung disease (ILD) is common among SSc patients. Objective: This study aims to define the clinical, laboratory and serologic characteristics of SSc patients with ILD and to present the frequency of chest computed tomography features. Methods: Two hundred twenty-six SSc patients who applied to the Rheumatology Department between January 2007 and August 2019 were retrospectively examined. A total of 100 SSc patients with ILD (44.2 %) were determined. Clinical, laboratory and serological features of SSc patients with and without ILD were compared. Results : Both groups had similar characteristics in terms of age and sex. The duration of disease (p=0.001) and follow-up time (p=0.001) were longer in SSc patients with ILD. Multivariable logistic regression analysis indicated that the duration of disease (OR: 1.06 [1.01-1.13], p=0.029), presence of gastrointestinal system involvement (OR: 3.29 [1.28-8.46], p=0.013) and anti-SCL70-positivity (OR: 6.04 [2.35-15.49], p<0.001) were associated with ILD. There was an inverse relationship between Anti-CENP-B positivity and the presence of ILD (p=0.001). The assessment regarding the chest computed tomography characteristics of interstitial pneumonia patterns were as follows: 82.5% non-specific interstitial pneumonia, 14.4% usual interstitial pneumonia and 2.1% desquamative interstitial pneumonia. The most frequent abnormal findings included ground glass opacification (88.7%), reticulation (64.9%), traction bronchiectasis (57.7%), septal thickening (52.6%) and honey combing (28.9%). Conclusion: We have shown that there is a relationship between anti-SCL70, disease duration, gastrointestinal system involvement and ILD in SSc patients.

Hemolytic uremic syndrome associated with rhabdomyolysis – Need to look beyond kidneys in hemolytic uremic syndrome

Hemolytic uremic syndrome, a part of thrombotic microangiopathy, is an important cause of acute kidney injury in children. Hemolytic uremic syndrome primarily targets kidney but extrarenal organ involvement is observed in 20–40% of patients. Extra-renal organ involvement in hemolytic uremic syndrome has been associated with greater disease severity and higher mortality. We describe a 31/2-year-old boy of hemolytic uremic syndrome with rhabdomyolysis, which is a rare extrarenal manifestation of hemolytic uremic syndrome. Unlike central nervous or gastrointestinal system involvement in hemolytic uremic syndrome which manifests clinically, muscle involvement may not and, if present, may worsen the existing acute kidney injury and may worsen disease prognosis. Considering the high morbidity and mortality in acute phase of hemolytic uremic syndrome, prompt evaluation to know the extent of extrarenal organ involvement at the earliest is important for management and prognosis of these patients.

Ulcerative Lesions in Behcet's Disease

Ulcerative lesions in Behcet’s disease (BD) are regarded as important manifestation for diagnosis. Various kinds of ulcerative lesions appear in patients with BD. They present as orogenital ulcers, necrotizing vasculitis and pyoderma gengrenosum. Gastrointestinal system involvement (Gis) in Behçet’s disease affects all areas from the esophagus to the anus. Most authors believe that the Gis manifestations of Behçet’s disease should be confined to aphthous ulcers, which can occur throughout the Gis tract. All patients with oro-genital and Gis ulcerations should be fully investigated to establish a definitive diagnosis and eliminate the possibility of an underlying BD.