Bioethical and medico-social dilemmas in the management of granulomatosis with polyangiitis in a child: A case report

Abstract

Granulomatosis with polyangiitis (GPA) is the most common ANCA-associated vasculitis in the pediatric population. The disease course is characterized by an extremely high level of unfavorable clinical outcomes and depends not only on timely diagnosis and protocol compliance but also on interaction with patients and their relatives.

The aim of the study was to highlight the GPA course in a child, burdened by social circumstances and caregiver-dependent features of the therapeutic management.

Case presentation. The disease onset in a 12-year-old boy was characterized by damage to the upper respiratory tract (sinusitis), the protocol treatment of which during 4 months did not contribute to the elimination of symptoms. Timely performed CT scan with GPA signs was not considered from the point of presence of a rare disease. New symptoms (weakness, deformation of the nose dorsum, hemorrhagic rash, hemoptysis, deep veins thrombosis of the lower limb) joined and prompted an in-depth examination that resulted the multisystem lesions (severe anemia, multiple lungs foci, nephrotic proteinuria, hematuria, impaired kidney function) with the high positive anti-proteinase-3 antibodies. Presentation features of GPA with the progressive involvement of other systems and the symptoms increasing, simultaneously unpreparedness of clinicians for a rare diagnosis and lack of a multidisciplinary approach led to a delay in starting immunosuppressive therapy. Burdens of social circumstances (family unpreparedness for diagnosis and clinical decision) tragically worsened the disease scenario.

Conclusions. Ambiguous situations in pediatric practice during the treatment of a rare disease require discussion of a wide range of ethical issues in the interaction between the doctor and the patient and his family. The inclusion of bioethical, psychosocial and legal fragments in the clinical guidelines (especially for diseases with an unfavorable prognosis) for patient’s protection and support throughout the follow-up period should become a mandatory addition to standard therapeutic management.