Hydralazine is a commonly used blood pressure medication that has been associated with rheumatologic manifestations such as drug-induced lupus. However, it is rarely associated with antineutrophilic cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV). Here we present a case of a 72-year-old female with hemoptysis, dyspnea, and palpable purpura, ultimately diagnosed with hydralazine-induced AAV. The diagnosis was proven via skin biopsy and serologic markers. A bronchoscopy revealed an alveolar hemorrhage. Treatment involved discontinuing hydralazine as well as steroids and rituximab. This case was atypical due to the relative lack of renal involvement. We also emphasize the importance of recognizing this rare diagnosis and discuss guideline-directed medical therapy for managing this condition.
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http://dx.doi.org/10.7759/cureus.76967 | DOI Listing |
Rheumatology (Oxford)
March 2025
Nephrology and Dialysis Unit, Meyer Children's Hospital IRCCS, Florence, Italy.
Hypereosinophilic syndrome (HES) and eosinophilic granulomatosis with polyangiitis (EGPA) both present with hypereosinophilia and organ damage induced by eosinophils. EGPA is also characterized by vasculitis and is associated with ANCA. Yet, discriminating HES from EGPA may be difficult in clinical practice as biomarkers to reliably differentiate between HES and EGPA are still lacking.
View Article and Find Full Text PDFRheumatology (Oxford)
March 2025
Norwich Medical School, University of East Anglia, Norwich, UK.
Polyarteritis nodosa (PAN) was first described in 1852 with the first widely recognised description in 1866 by Kussmaul and Meier. Since then our concepts of the condition have evolved, with recognition of the difference between polyarteritis nodosa and microscopic polyangiitis (MPA). Classification criteria for PAN remain unsatisfactory.
View Article and Find Full Text PDFRheumatology (Oxford)
March 2025
Division of Pulmonary Medicine, Cleveland Clinic, Cleveland, OH, USA.
This mini review explores the association of interstitial lung disease (ILD) with antineutrophil cystoplasmic antibodies (ANCA) and the clinical syndrome of microscopic polyangiitis (MPA). Reports on radiographic and histopathologic findings as well as genetic predispositions are reviewed. Based on this evidence a concept for the pathogenesis of the relationship of ILD, MPO-ANCA and MPA is proposed.
View Article and Find Full Text PDFRheumatology (Oxford)
March 2025
Department of Nephrology, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark.
The use of plasma exchange (PLEX) as adjunct therapy in the initial management of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis remains to be discussed controversially in light of present evidence. In our Viewpoint we highlight scenarios where we think that physicians might still consider the use of PLEX, such as the most severe presentation forms including diffuse alveolar haemorrhage (DAH) with hypoxaemia and acute glomerulonephritis with significantly impaired kidney function. Recent sub-analyses of the PEXIVAS trial have focused on these patient populations and have provided outcomes of these patients.
View Article and Find Full Text PDFClin Respir J
March 2025
Department of Respiratory Medicine, Toho University Graduate School of Medicine, Ōta, Japan.
Introduction: Anti-neutrophil cytoplasmic antibody (ANCA) seropositivity strongly correlates to ANCA-associated vasculitis. Patients with idiopathic interstitial pneumonias (IIPs) without systemic vasculitis are sometimes ANCA-positive. Radiological and pathological differences between patients with myeloperoxidase (MPO)-ANCA-positive and those with proteinase 3 (PR3)-ANCA-positive IIPs remain unclear.
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