©2017 Turkish League Against Rheumatism. All rights reserved. Granulomatosis with polyangiitis is an autoimmune disease, characterized by involvement of small vessels and presence of anti-cytoplasmic antibodies (ANCA). From the 2011, according to recommendations of the American College of Rheumatology and the American Society of Nephrology, the term granulomatosis with polyangiitis has been ado...

Orofacial granulomatosis is a rare chronic inflammatory disorder characterized by persistent or recurrent soft tissue swellings, oral ulceration, and other orofacial features in the absence of an identifiable granulomatous disease. We report a case of a 61-year-old woman with recurrent ulcerations and swellings in her oral mucosa. She was diagnosed as orofacial granulomatosis based upon clinico...

We present a case of a 38-year-old Japanese man with Wegener's granulomatosis complicated with pulmonary-renal syndrome, i.e., diffuse pulmonary hemorrhage and rapidly progressive renal glomerulonephritis. As this is a life-threatening condition, we promptly initiated plasma exchange with intravenous methylprednisolone therapy. Diffuse pulmonary hemorrhage and renal failure were markedly improv...

Granulomatosis with Polyangiitis (GPA), which was formerly named Wegener's Granulomatosis (WG) is a systemic disease characterized by necrotizing granulomatous inflammation and vasculitis that primarily involves upper and lower respiratory tract, as well as kidneys. Diagnosing GPA on the basis of transthoracic fine needle aspiration (TFNA) may be problematic, as it can be misdiagnosed as cancer...

Lymphomatoid granulomatosis is a necrotizing angiocentric and angiodestructive infiltrative process involving primarily the lung, skin, central nervous system, and kidney. The incidence is highest in middle-aged men and is rare in children. We report a case of lymphomatoid granulomatosis involving both skin and lung in a 4-year-old boy. The disease progressed to peripheral T-cell lymphoma, whic...

BACKGROUND Reports from specialist nephrological centres have suggested that the antineutrophil cytoplasm antibody (ANCA) test is highly specific and sensitive for patients with Wegener's granulomatosis. To determine the usefulness of the ANCA test in everyday respiratory practice the results of the test were audited in all patients in the south west of England with respiratory symptoms who und...

OBJECTIVES To determine the possible association of serum 25-hydroxyvitamin D (25OHD) levels with disease activity and respiratory infection in granulomatosis with polyangiitis patients during two different periods: winter/spring and summer/autumn. METHODS Thirty-two granulomatosis with polyangiitis patients were evaluated in the winter/spring, and the same patients (except 5) were evaluated ...

Orofacial granulomatosis (OFG) is an uncommon chronic inflammatory disorder of the orofacial region. It is characterized by subepithelial noncaseating granulomas and has a spectrum of possible clinical manifestations ranging from subtle oral mucosal swelling to permanent disfiguring fibrous swelling of the lips and face. Etiopathogenesis is unknown. A range of systemic granulomatous disorders, ...

Granulomatous hepatitis is not a uniform disease entity but a generic histopathologic diagnosis. The present review of aetiology and epidemiology of granulomas in the liver, shows that in the past tuberculosis and sarcoidosis were the most frequent but there remained a large percentage of "undiagnosis" cases; at the present drug-induced granulomatous hepatitis are frequently reported and "undia...

In adults, the term specific pulmonary renal syndrome describes disorders with pulmonary and glomerular manifestations and includes Wegener's granulomatosis, Goodpasture disease, and systemic lupus erythematosus. Nonspecific pulmonary renal syndrome refers to either pulmonary disease complicating glomerular disease, or glomerular diseases following pulmonary disease. Since little is known regar...