The following are some of the conclusions and proposals made at the Chapel Hill Consensus Conference on the Nomenclature of Systemic Vasculitis. 1. Although not a prerequisite component of the definitions, patient age is recognized as a useful discriminator between Takayasu arteritis and giant cell (temporal) arteritis. 2. The name "polyarteritis nodosa," or alternatively, the name "classic polyarteritis nodosa," is restricted to disease in which there is arteritis in medium-sized and small arteries without involvement of smaller vessels. Therefore, patients with vasculitis affecting arterioles, venules, or capillaries, including glomerular capillaries (i.e., with glomerulonephritis), are excluded from this diagnostic category. 3. The name "Wegener's granulomatosis" is restricted to patients with granulomatous inflammation. Patients with exclusively nongranulomatous small vessel vasculitis involving the upper or lower respiratory tract (e.g., alveolar capillaritis) fall into the category of microscopic polyangiitis (microscopic polyarteritis). 4. The term "hypersensitivity vasculitis" is not used. Most patients who would have been given this diagnosis fall into the category of microscopic polyangiitis (microscopic polyarteritis) or cutaneous leukocytoclastic angiitis. 5. The name "microscopic polyangiitis," or alternatively, "microscopic polyarteritis," connotes pauci-immune (i.e., few or no immune deposits) necrotizing vasculitis affecting small vessels, with or without involvement of medium-sized arteries. Cryoglobulinemic vasculitis, Henoch-Schonlein purpura, and other forms of immune complex-mediated small vessel vasculitis must be ruled out to make this diagnosis. 6. The name "cutaneous leukocytoclastic angiitis" is restricted to vasculitis in the skin without involvement of vessels in any other organ. 7. Mucocutaneous lymph node syndrome must be present to make a diagnosis of Kawasaki disease.(ABSTRACT TRUNCATED AT 250 WORDS)

https://www.onlinelibrary.wiley.com/doi/pdf/10.1002/art.1780370206

Nomenclature of systemic vasculitides. Proposal of an international consensus conference

Churg-Strauss Syndrome. Clinical Study and Long-Term Follow-Up of 96 Patients

https://www.kidney-international.org/article/S0085-2538(15)60452-5/pdf

Diagnostic value of standardized assays for anti-neutrophil cytoplasmic antibodies in idiopathic systemic vasculitis

Objective: To examine possible risk factors for relapse, including chronic nasal carriage of Staphylococcus aureus and serial antineutrophil cytoplasmic antibody (ANCA) determinations in patients w...

Association of Chronic Nasal Carriage of Staphylococcus aureus and Higher Relapse Rates in Wegener Granulomatosis

Objective

Churg-Strauss syndrome (CSS) is classified among the so-called antineutrophil cytoplasmic antibody–associated systemic vasculitides (AASVs) because of its clinicopathologic features that overlap with the other AASVs. However, while antineutrophil cytoplasmic antibodies (ANCAs) are consistently found in 75–95% of patients with Wegener's granulomatosis or microscopic polyangiitis, their prevalence in CSS varies widely and their clinical significance remains uncertain. We undertook this study to examine the prevalence and antigen specificity of ANCAs in a large cohort of patients with CSS. Moreover, we evaluated the relationship between ANCA positivity and clinicopathologic features.



Methods

Immunofluorescence and enzyme-linked immunosorbent assay were used to determine the presence or absence of ANCAs in 93 consecutive patients at the time of diagnosis. The main clinical and pathologic data, obtained by retrospective analysis, were correlated with ANCA status.



Results

ANCAs were present by immunofluorescence in 35 of 93 patients (37.6%). A perinuclear ANCA (pANCA) pattern was found in 26 of 35 patients (74.3%), with specificity for myeloperoxidase (MPO) in 24 patients, while a cytoplasmic ANCA pattern, with specificity for proteinase 3, was found in 3 of 35 patients (8.6%). Atypical patterns were found in 6 of 30 patients with anti-MPO antibodies (20.0%). ANCA positivity was associated with higher prevalences of renal disease (51.4% versus 12.1%; P < 0.001) and pulmonary hemorrhage (20.0% versus 0.0%; P = 0.001) and, to a lesser extent, with other organ system manifestations (purpura and mononeuritis multiplex), but with lower frequencies of lung disease (34.3% versus 60.3%; P = 0.019) and heart disease (5.7% versus 22.4%; P = 0.042).



Conclusion

ANCAs are present in ∼40% of patients with CSS. A pANCA pattern with specificity for MPO is found in most ANCA-positive patients. ANCA positivity is mainly associated with glomerular and alveolar capillaritis.

https://onlinelibrary.wiley.com/doi/pdf/10.1002/art.21250

Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome

https://www.kidney-international.org/article/S0085-2538(15)34810-9/pdf

Autoantibodies against myeloid lysosomal-enzymes in crescentic glomerulonephritis

Antineutrophil cytoplasmic autoantibodies with specificity for myeloperoxidase (MPO) were found in 53 patient sera that were routinely submitted for antineutrophil cytoplasmic antibody determination. Based on clinical and histologic criteria, 15 of these 53 patients were classified as having systemic necrotizing vasculitis of the polyarteritis group, 11 patients were classified as having Wegener's granulomatosis (WG), and 14 were classified as having idiopathic crescentic glomerulonephritis. The remaining 13 patients did not fulfill the diagnostic criteria for these disorders, although most of these patients had clinical symptoms compatible with these disorders. While all patients with WG had renal involvement, only 4 of the 15 patients with systemic necrotizing vasculitis of the polyarteritis group had glomerulonephritis. The sensitivity of autoantibodies to MPO for either systemic necrotizing vasculitis of the polyarteritis group, WG, or idiopathic crescentic glomerulonephritis was further tested in all our patients with these disorders (n = 104). Twenty-seven of 104 patients had autoantibodies to MPO. Furthermore, 69 of the remaining 77 patients had autoantibodies specific for the 29-kd serine protease, which has been reported to be specifically associated with WG. Sera from 8 patients were negative for either of these antibodies (92% sensitivity of autoantibodies to MPO and/or the 29-kd serine protease). The specificity of autoantibodies to MPO for either systemic necrotizing vasculitis of the polyarteritis group, WG, or idiopathic crescentic glomerulonephritis was also tested in selected groups of patients who had closely related diseases. Two of 144 patients had autoantibodies to MPO (specificity 99%). We conclude that these antibodies are not only associated with idiopathic and vasculitis-associated crescentic glomerulonephritis, but also with classic polyarteritis nodosa, Churg-Strauss syndrome, and the polyangiitis overlap syndrome without renal involvement. The presence of autoantibodies to MPO or the 29-kd serine protease is highly sensitive and specific for systemic necrotizing vasculitis of the polyarteritis group, WG, and idiopathic crescentic glomerulonephritis.

https://onlinelibrary.wiley.com/doi/pdf/10.1002/art.1780330829

Association of autoantibodies to myeloperoxidase with different forms of vasculitis

https://www.amjmed.com/article/0002-9343(91)90074-8/pdf

Detection of autoantibodies against myeloid lysosomal enzymes: A useful adjunct to classification of patients with biopsy-proven necrotizing arteritis

Antibodies to myeloperoxidase were detected in the serum of three patients with the Churg-Strauss syndrome.

https://thorax.bmj.com/content/thoraxjnl/46/1/70.full.pdf

J. D. Elema

Papers

Autoantibodies against myeloid lysosomal-enzymes in crescentic glomerulonephritis

Association of autoantibodies to myeloperoxidase with different forms of vasculitis

Detection of autoantibodies against myeloid lysosomal enzymes: A useful adjunct to classification of patients with biopsy-proven necrotizing arteritis

Antimyeloperoxidase antibodies in the Churg-Strauss syndrome

Steroid-Responsive Interstitial Pulmonary Disease in Systemic Sclerosis: Monitoring by Bronchoalveolar Lavage