Wegener’s disease is a very rare disorder which presents with inflammation of the lung and kidneys. The disorder is classified as a vasculitis meaning it causes inflammation of small blood vessels. The disorder tends to affect the nose, throat, lungs and kidney.
It can affect other organ in the body in the later stages. Wegener’s granulomatosis usually occurs in childhood but most people are diagnosed after a long delay. Typical symptons are often present in the 2nd-3rd decade of life.
The classical symptoms are loss of weight, no appetite, face or nose pain, low grade fever, fatigue, blood in the urine, runny or congested nose, nose bleeds, cough, ear infections, wheezing, joint pains or a skin rash.
The disorder is quite difficult to diagnose as many physicians never suspect it or have no clue about it. Testing includes checking for certain antibodies and most people need a biopsy to confirm the diagnosis.
Chest x-rays are always done when Wegener’s is suspected. A classical feature of this disorder is a deformed or a saddle nose appearance.
Wegener’s is life threatening and needs treatment. In most cases long term corticosteroids and immune suppressing drugs are used. The drugs are potent and have many side effects. There is no cure for Wegener’s and relapses are common. Those patients whose disease is controlled by medications do okay but those who have continued relapses do poor.