Diagnostic and Therapeutic Meanders in a Wegener Disease - Case Report and Therapeutic Challenges

Abstract Granulomatosis with polyangiitis (GPA), previously known as Wegener’s granulomatosis, is a vasculitis that primarily affects the respiratory tract and kidneys (1) . Known as a rare disease, predominantly affects Caucasian adults aged between 45 and 60 years (2) . While the specific cause remains unclear, it is believed that environmental factors play a role in triggering the condition in individuals who are genetically predisposed (3) . There is a wide range of clinical manifestations, comprising both general nonspecific symptoms (4) and organ-specific symptoms, with lung involvement in nearly 90% of patients (5) . The diagnosis relies on clinical and imaging criteria, along with histopathological confirmation (2) . Treatment consists of an induction phase, followed by a maintenance phase, utilizing glucocorticoids and immunosuppressants, which has greatly enhanced the prognosis for these patients (6) . The objective of this paper is to illustrate the complexity of GPA through the presentation of a clinical case. The patient is a 59-year-old man with a professional background involving exposure to respiratory toxins who presented to the clinic with intermittent mucopurulent cough, episodes of hemoptysis, nonspecific chest pain, bilateral knee pain, weight loss, and excessive nocturnal sweating. The imaging findings indicated significant polymorphic lesions in the lungs, and the diagnosis of GPA was confirmed via transthoracic biopsy. Using a therapeutic strategy of immunosuppression and oral corticosteroid therapy, the patient’s clinical condition showed a modest improvement. The particularity of this case lies in the need to revise the treatment strategy for a patient identified as a non-responder to cyclophosphamide, given the imaging, functional and biological decline observed under the initial immunosuppressive therapy.