Wegener’s granulomatosis

It is a granulomatous vasculitis that affects the upper and lower respiratory tract and the renal glomeruli.

It is a rare entity that has its maximum incidence at 40 years of age with a clear predominance in males and whites.

How is it produced?

The cause is unknown, but it is accepted that it is a foreign antigen that enters the body by inhalation, producing an abnormal response of the host's immune system, causing an inflammatory response in the respiratory tract and at the systemic level.

Symptoms

It initially comprises a general symptomatology with fever, fatigue, weakness, loss of appetite, weight loss and myalgia along with mainly respiratory symptoms, to which renal, skin, neurological and ocular symptoms can later be added.

Upper respiratory symptoms include chronic sinusitis, chronic otitis with hearing loss, and rhinitis, up to ulcerations of the nasal mucosa, saddle nose deformity, and nasal septum perforations.

At the pulmonary level, respiratory distress, cough, chest pain and hemoptysis (emission of blood through the mouth) are observed.

Renal involvement appears in up to 80% of patients, late with a characteristic lesion such as glomerulonephritis that initially manifests with a loss of protein and blood through the urine, until progressing to end-stage renal failure, this being a more frequent cause. frequent mortality.

The ocular organs are affected in the form of scleritis, episcleritis, dacryocystitis, uveitis, and proptosis ("bulging eye" due to involvement of inflammatory tissue in the orbital cavity).

Diagnosis

It is carried out through the typical symptoms of the disease at the respiratory and renal level mainly. Complementary tests support the diagnosis.

blood tests can show characteristically high titers of cytoplasmic antibodies against neutrophils (ANCA-C), appearing in up to 70% of patients, thus confirming the diagnosis.

The chest radiograph shows abnormalities in most patients in the form of multiple pulmonary infiltrates with or without often cavitated nodules.

The biopsy is the one who confirms the diagnosis. Lung biopsy is the most cost-effective, since the lesions typical of vasculitis can be confirmed in the lung tissue and granulomas can be identified.

Treatment

Without treatment, life expectancy is less than 6 months due to the progression to end-stage renal failure.

At present, with adequate treatment, improvement is achieved in 90% of patients. The drug of choice is cyclophosphamide, an immunosuppressive drug associated with corticosteroids during the first months, and maintained for at least one year after achieving clinical remission.

Up to half of patients who go into remission have one or more relapses, needing the aforementioned medication again.

Irreversible sequelae may remain, such as "saddle" deformity, deafness, and / or kidney failure.