Wegener's granulomatosis is a rare disease characterized by granulomatous inflammation in the respiratory tract and other organs, with widespread vascular lesions resembling those in polyarteritis nodosa. The disease typically presents with progressive ulceration in the respiratory tract, along with signs of systemic inflammation. It is named after Fritz Wegener, who first described it in 1936. The condition is associated with granulomatous ulceration in the respiratory tract, widespread granulomatous and vascular lesions in the lungs, spleen, and kidneys, and necrotizing lesions in small vessels. The disease progresses rapidly, often leading to death within five months, though some patients may have a more chronic course with periods of remission. The clinical features include chronic cough, hemoptysis, and respiratory symptoms, along with systemic manifestations such as fever, malaise, and skin rashes. Histologically, the disease is marked by granulomatous inflammation with giant cells, necrosis, and vascular involvement. The pathogenesis is not fully understood, but it is believed to involve an immune-mediated hypersensitivity reaction. The disease is distinct from other granulomatous and vascular conditions, such as polyarteritis nodosa and malignant granuloma, though there is some overlap in clinical and pathological features. Treatment options include radiotherapy for localized lesions and corticosteroids for widespread involvement. Radiotherapy has shown success in healing respiratory tract lesions, while corticosteroids are effective in managing systemic symptoms. The disease is often diagnosed based on clinical and pathological features, with radiological changes in the lungs being a key diagnostic indicator. Despite extensive research, the exact cause remains unclear, and the condition is considered a separate entity with unique pathological and clinical features. Over 56 cases have been documented, with ongoing research aiming to better understand its pathogenesis and improve treatment outcomes.Wegener's granulomatosis is a rare disease characterized by granulomatous inflammation in the respiratory tract and other organs, with widespread vascular lesions resembling those in polyarteritis nodosa. The disease typically presents with progressive ulceration in the respiratory tract, along with signs of systemic inflammation. It is named after Fritz Wegener, who first described it in 1936. The condition is associated with granulomatous ulceration in the respiratory tract, widespread granulomatous and vascular lesions in the lungs, spleen, and kidneys, and necrotizing lesions in small vessels. The disease progresses rapidly, often leading to death within five months, though some patients may have a more chronic course with periods of remission. The clinical features include chronic cough, hemoptysis, and respiratory symptoms, along with systemic manifestations such as fever, malaise, and skin rashes. Histologically, the disease is marked by granulomatous inflammation with giant cells, necrosis, and vascular involvement. The pathogenesis is not fully understood, but it is believed to involve an immune-mediated hypersensitivity reaction. The disease is distinct from other granulomatous and vascular conditions, such as polyarteritis nodosa and malignant granuloma, though there is some overlap in clinical and pathological features. Treatment options include radiotherapy for localized lesions and corticosteroids for widespread involvement. Radiotherapy has shown success in healing respiratory tract lesions, while corticosteroids are effective in managing systemic symptoms. The disease is often diagnosed based on clinical and pathological features, with radiological changes in the lungs being a key diagnostic indicator. Despite extensive research, the exact cause remains unclear, and the condition is considered a separate entity with unique pathological and clinical features. Over 56 cases have been documented, with ongoing research aiming to better understand its pathogenesis and improve treatment outcomes.