Microscopic polyangiitis presenting with persistent cough and hemoptysis
in pediatrics: A case report and review
Abstract
Background:Microscopic polyangiitis (MPA) is a necrotizing vasculitis
which involves small- and medium-sized vessels. It is associated with
the presence of anti-neutrophil cytoplasmic antibodies with a
perinuclear staining pattern (p-ANCA). The kidney and lung are the
organs primarily affected. MPA is rare in children, and easily
misdiagnosed. In this work we describe a complete course of this
disease. Case presentation: An 11-year-old girl with a one-month cough
and hemoptysis, showed no improvement after imipenem-cilastatin
treatment. P-ANCA, and microscopic haematuria and proteinuria, were
positive, and a chest CT revealed an area of shadow in bilateral lower
lobes of the lungs. Renal biopsies revealed crescentic
glomerulonephritis. MPA was diagnosed based on these observations. The
patient showed dramatic clinical and imaging improvement after
immunosuppressive treatment. Conclusion: The organs and systems most
affected by MPA in children, are the lungs (about 80%), kidneys (about
80%), cutaneous involvement (about 20%), nervous system involvement
(about 16%), and gastrointestinal involvement (about 16%). Reasonable
care should be taken in examining those patients, while biopsies of the
kidney or any other of the organs remain the gold standard for
diagnostic purposes. Pulmonary involvement may be the initial symptom of
MPA, which is often misdiagnosed as pneumonia and antibiotics are used
at the beginning of the disease. Patients with hemoptysis should undergo
a comprehensive examination, including urine routine, to determine
whether there is renal involvement, and an immunological index detection
to determine whether it is an autoimmune disease.