Presentation
Protease proteinase 3 (PR3) and myeloperoxidase (MPO) ANCA are present in almost all patients with MPA and GPA in the active generalized stage of the disease; in EGPA, ANCA are present in less than 40 percent and their presence is associated with typical [thermofisher.com]
CNS involvement is uncommon in RA and presents vasculitis-like symptoms, such as stroke, seizures, and meningitis (probably due to rheumatoid nodules). [ncbi.nlm.nih.gov]
Entire Body System
- Malaise
The most common and initial symptom is migraine (33%) localized in the temporal area, accompanied by fever, malaise, myalgia and anorexia. The temporal artery is often swollen and sensitive to palpation. [ncbi.nlm.nih.gov]
- Lymphadenopathy
The most frequent radiological features are pulmonary infiltrates, hemorrhage, and hilar lymphadenopathy. [thermofisher.com]
Respiratoric
- Nasal Ulcer
Nasal disease presents with obstruction, nasal ulcers and septal perforation, serosanguinous discharge, or epistaxis. Destruction of the nasal septum results in the typical saddle-nose deformity. [thermofisher.com]
- Saddle Nose
Destruction of the nasal septum results in the typical saddle-nose deformity. Renal disease from severe renal failure to proteinuria or haematuria with no impairment of renal function. [thermofisher.com]
Skin
- Papule
Cutaneous manifestations including ulcers, palpable purpura, papules, and nodules. Late-onset asthma. Peripheral blood and tissue eosinophilia. Cardiac disease. [thermofisher.com]
- Cutaneous Manifestation
Cutaneous manifestations including ulcers, palpable purpura, papules, and nodules. Late-onset asthma. Peripheral blood and tissue eosinophilia. Cardiac disease. [thermofisher.com]
Musculoskeletal
- Arthralgia
Musculoskeletal symptoms are common, with most patients experiencing arthralgias and/or myalgias. A true synovitis can be seen in 25 percent of patients. Mononeuritis multiplex. [thermofisher.com]
Face, Head & Neck
- Epistaxis
Nasal disease presents with obstruction, nasal ulcers and septal perforation, serosanguinous discharge, or epistaxis. Destruction of the nasal septum results in the typical saddle-nose deformity. [thermofisher.com]
Workup
X-Ray
- Pulmonary Infiltrate
The most frequent radiological features are pulmonary infiltrates, hemorrhage, and hilar lymphadenopathy. [thermofisher.com]
Treatment
[…] skin Vasculitis diagnosis: Testing for differential diagnosis Prompt recognition of vasculitis either as a new presentation or a clinical relapse is key to optimizing management and preventing organ damage.6 The good news is that prompt diagnosis and treatment [thermofisher.com]
Treatment PubMed The neurologic manifestations of systemic lupus erythematosus Greenberg BM (2009) Review Treatment PubMed The involvement of the peripheral nervous system in biopsy proven active giant cell arteritis Pfadenhauer K, Roesler A and Golling [ncbi.nlm.nih.gov]
Prognosis
A combination of steroids and pulse cyclophosphamide is recommended for patients with a poor prognosis. ( 24, 25 ) With a relapse rate of 25% and reduced survival rate, a close follow-up of suspected PACNS is mandatory. [ncbi.nlm.nih.gov]
Etiology
Variable vessel vasculitis Behçet disease and Cogan's syndrome Single-organ vasculitis Primary central nervous system vasculitis Vasculitis associated with systemic disease Lupus vasculitis Rheumatoid vasculitis Vasculitis associated with probable etiology [ncbi.nlm.nih.gov]
Pathophysiology
CONCLUSION The vasculitides and connective tissue diseases associated to them provide an avenue for investigating the pathophysiology of immune system among the vasculature of the central and peripheral nervous system. [ncbi.nlm.nih.gov]
Prevention
Prompt recognition of vasculitis either as a new presentation or a clinical relapse is key to optimizing management and preventing organ damage.6 ANCA-associated vasculitides Vasculitis is a condition that causes inflammation of vessel walls.3 ANCA-associated [thermofisher.com]
A randomized controlled trial of 44 patients showed that maintenance therapy with infliximab failed to show more efficacy in disease control than that of steroids, and it did not allow a reduction in the dose of steroids required to prevent relapse. [ncbi.nlm.nih.gov]