Successful Induction Treatment With Rituximab of Isolated Pauci-Immune Pulmonary Capillaritis Presenting as Diffuse Alveolar Hemorrhage in a Pediatric Patient

Katherine E Lewinter; Michal Cidon; Mikako Warren; Manvi Bansal

doi:10.1016/j.chest.2020.06.022

Successful Induction Treatment With Rituximab of Isolated Pauci-Immune Pulmonary Capillaritis Presenting as Diffuse Alveolar Hemorrhage in a Pediatric Patient

Chest. 2020 Nov;158(5):e225-e227. doi: 10.1016/j.chest.2020.06.022.

Authors

Katherine E Lewinter¹, Michal Cidon², Mikako Warren³, Manvi Bansal⁴

Affiliations

¹ Divisions of Pediatric Pulmonology and Sleep Medicine, Children's Hospital Los Angeles, University of Southern California School of Medicine, Los Angeles, CA. Electronic address: klewinter@chla.usc.edu.
² Department of Pediatric Rheumatology, Children's Hospital Los Angeles, University of Southern California School of Medicine, Los Angeles, CA.
³ Department of Pathology, Children's Hospital Los Angeles, University of Southern California School of Medicine, Los Angeles, CA.
⁴ Department of Pediatric Pulmonology and Sleep Medicine, Children's Hospital Los Angeles, University of Southern California School of Medicine, Los Angeles, CA.

PMID: 33160541
DOI: 10.1016/j.chest.2020.06.022

Abstract

Diffuse alveolar hemorrhage often presents as dyspnea, cough, or hemoptysis, and it is mediated by both immune and nonimmune processes. Isolated pauci-immune capillaritis (IPPC) is a rare diagnosis in which capillaritis, small-vessel vasculitis of the lung, is found on biopsy in the absence of an underlying systemic disorder. Traditionally, IPPC has been treated similarly to anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis with cyclophosphamide and glucocorticoids. However, few cases describing management options are available in the literature, especially among pediatric patients. Our report of successful induction of remission in an adolescent girl suggests that the combination of IV rituximab and pulse methylprednisolone may be a viable option for disease control in pediatric patients with IPPC.

Keywords: diffuse alveolar hemorrhage; interstitial lung disease; pediatric pulmonology.

Publication types

Case Reports

MeSH terms

Adolescent
Capillaries / pathology
Drug Therapy, Combination
Dyspnea / diagnosis
Dyspnea / etiology
Female
Hemoptysis* / diagnosis
Hemoptysis* / etiology
Hemoptysis* / therapy
Humans
Immunologic Factors / administration & dosage
Lung / diagnostic imaging
Lung Diseases, Interstitial* / diagnosis
Lung Diseases, Interstitial* / etiology
Lung Diseases, Interstitial* / physiopathology
Lung Diseases, Interstitial* / therapy
Methylprednisolone / administration & dosage*
Pulse Therapy, Drug / methods
Remission Induction / methods
Rituximab / administration & dosage*
Tomography, X-Ray Computed / methods
Treatment Outcome
Vasculitis* / complications
Vasculitis* / diagnosis
Vasculitis* / physiopathology
Vasculitis* / therapy

Substances

Immunologic Factors
Rituximab
Methylprednisolone