Abnormalities in alpha-1 antitrypsin (AAT) proteins are risk factors for human disease. While the most common is AAT deficiency, a genetic disorder associated with chronic obstructive pulmonary disease, additional disorders associated with AAT abnormalities are increasingly recognised. We describe a middle-aged woman who presented with fulminant hepatic and multiorgan failure. Evaluation reveal...

Microscopic polyangiitis (MPA) is one of the vasculitides that is included in the pulmonary renal syndromes. Pathologically, MPA has been defined as necrotizing vasculitis with few or no immune deposits, primarily affecting small vessels including arterioles, venules, or capillaries. Pulmonary interstitial fibrosis (PIF) as an accompanying manifestation in MPA has not been widely appreciated. I...

background worldwide, the incidence of inflammatory bowel disease (ibd) is increasing. this study aims to evaluate the diagnostic value of two serological markers, atypical perinuclear anti-neutrophil cytoplasmic antibodies (atypical-p-anca) and anti-saccharomyces cerevisiae antibodies (asca), with the intent to determinetheir relationship to ulcerative colitis (uc) and crohn's disease (cd), in...

conclusions patients treated with ptu should be closely monitored and followed up, even if the drug has been used for several years. when patients develop progressive dyspnea with alveolar opacities on chest imaging that cannot be explained otherwise, alveolar hemorrhage should be an important differential diagnosis while investigating the case. early diagnosis and prompt discontinuation of the...

Inflammatory bowel disease (IBD) is the general term used for a heterogeneous group of intestinal disorders, including Crohn's disease (CD) and ulcerative colitis (UC). Serological markers such as anti-Saccharomyces cerevisiae antibodies (ASCA) and atypical perinuclear antineutrophilic cytoplasmic antibody (atypical pANCA) have proven useful in the diagnosis and differentiation of CD and UC. Im...

RATIOINALE Relapsing polychondritis (RP) is a rare and heterogeneous disease complex of unknown origin which basically affects cartilaginous structures, 40% of which accompanied by rheumatic, hematologic, and endocrine disease. Among them, vasculitis is the most common accompanying type and usually presented with positive antineutrophilic cytoplasmic antibody (ANCA). The presence of ANCA could ...

The goal of this study was to investigate the correlation between perinuclear antineutrophilic cytoplasmic antibody (pANCA) and clinical scores before and after treatment in diarrheic dogs with food-responsive disease (FRD) or inflammatory bowel disease (IBD). pANCA serology was evaluated prospectively by indirect immunofluorescence in 65 dogs with signs of gastrointestinal disease, and if posi...

1Department of Nephrology, Hormozgan University of Medical Sciences, BandarAbbas, Iran 2Department of Nephrology, Shahid Beheshti University of Medical Sciences, Tehran, Iran 3Department of Pathology, Shahid Beheshti University of Medical Sciences, Tehran, Iran CASE A 51-year-old cigarette-smoker man was admitted because of progressive generalized weakness and early fatigability since 3 months ...