Renal Involvement and Its Treatment in Pediatric Patients With Proteinase-3 Anti-Neutrophil Cytoplasmic Antibody Positive Granulomatosis With Polyangiitis: A Case Series

Abstract

We describe three pediatric patients between the ages of 10 and 14 years old who were diagnosed with granulomatosis with polyangiitis (GPA) between 2014 and 2019. Each case involves variations in presentation, symptomatology, diagnostics, and induction and maintenance therapy regimens. Patient 1 presented with significant renal involvement, hypertensive emergency, and focal alveolar hemorrhage, a rare presentation of GPA that causes up to 60% mortality. Patient 2 presented with minimal renal involvement and a diffuse petechial rash, which is the most common cutaneous presentation of GPA. Finally, patient 3 presented with significant renal involvement and later on with symptoms of idiopathic intracranial hypertension (IIH), a unique and rare presentation associated with GPA. Despite the heterogeneity of these cases, the similar therapy regimens used in each case successfully achieved induction and maintenance of disease remission, providing an evidentiary basis for these treatment regimens even in severe and unusual pediatric GPA cases.

The Efficacy and Safety of Rituximab in ANCA-Associated Vasculitis: A Systematic Review

“…Following the first report of the successful treatment of AAV with RTX, different rare clinical manifestations were reported as having been successfully treated with RTX. Some of the most important rare clinical presentations in GPA patients, which resolved following RTX, include retrobulbar granuloma [ 78 ], refractory ophthalmic [ 126 ], pachymeningitis [ 146 ], the presence of orbital inflammation [ 122 ], long-established end-stage renal disease [ 147 ], post-kidney-transplantation glomerulonephritis-related AAV [ 148 ], pituitary GPA [ 149 ], renal mass [ 150 ], gastric ulcer [ 151 ], pancreatitis [ 152 ], ophthalmoplegia [ 153 ], pyoderma gangrenosum [ 154 , 155 , 156 ], prostatitis [ 157 ] aortic inflammation [ 158 ], intestinal involvement [ 159 ], mastitis [ 160 ], aortitis [ 161 ], gingivitis [ 162 ], Isolated Pauci-Immune Pulmonary Capillaritis [ 163 ], isolated orbital GPA [ 164 ], cranial neuropathy [ 165 ], severe necrotizing scleritis [ 166 ], genital necrosis and inflammation [ 167 ], GPA-associated subcutaneous cheek odule [ 168 ], pseudo-tumoral digital nodules [ 169 ], hypertrophic pachymeningitis [ 170 , 171 ], acute myocarditis [ 172 ], palpable purpura [ 173 ], central nervous system vasculitis [ 174 ], intra-cranial hypertension [ 175 , 176 ], tracheobronchial stenosis [ 142 ], acute aortic valve regurgitation [ 177 ], GPA-mimicking meningeal tuberculosis [ 178 ], gynecological involvement [ 179 ], GPA-mimicking lung mal...…”