Proteinase 3 (PR3)-antineutrophil cytoplasmic antibody (ANCA)-associated vasculitic neuropathy in diffuse cutaneous systemic sclerosis: a rare duo

Radwan, Yasser; Berini, Sarah E.; Ernste, Floranne C.; Makol, Ashima

doi:10.1136/bcr-2019-232987

Cited by 4 publications

(3 citation statements)

References 13 publications

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“…Another case of SSc and ANCA associated vasculitis reported by Radwan et al of a 65-year-old man with diffuse cutaneous SSc presenting with new-onset peripheral neuropathy. The patient had a positive PR3 and cytoplasmic ANCA, and histopathology confirmed an inflammatory vasculitic neuropathy [12] . On the other hand, overlap between PAN and SSc was very rarely reported in SSc.…”

Section: Discussionmentioning

confidence: 92%

Overlap between systemic sclerosis and polyarteritis nodosa: A case report

Mahmoud

Elsaid

Zayed

2021

The Egyptian Rheumatologist

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Background Systemic sclerosis (SSc) is a multiorgan connective tissue disease characterized by vasculopathy, inflammation, autoimmunity, and fibrosis in the skin, lungs and other organs. The occurrence of frank vasculitis is uncommon. Case presentation A 36-year old male patient with limited cutaneous SSc developed multiple necrotic ulcers on both legs and feet and gangrene of several toes, followed by an acute onset of axonal sensorimotor neuropathy affecting both radial and peroneal nerves, severe testicular pain with gangrenous patches over the scrotum. The hepatitis B virus (HBV) core antibody was positive while HB surface antigen and surface antibody, HAV and HCV antibodies were negative. The polymerase chain reaction for HBV and HCV showed no detectable viraemia. Antineutrophil cytoplasmic antibodies, cryoblobulins, anticardiolipin antibodies, lupus anticoagulant, antimitochondrial and anti- liver-kidney microsomal antibodies were negative. Pelvi-abdominal ultrasound and portal vein Doppler study showed a coarse and heterogeneous echo-texture of the liver, splenomegaly, moderate ascites and an enlarged, patent portal vein. Fibroscan revealed grade III liver fibrosis. He had an attack of haematemesis with elevation of the liver enzymes and low serum albumin and prothrombin concentrations. He was diagnosed as a case of polyarteritis nodosa. He was successfully treated by methylprednisolone intravenous pulses, followed by oral prednisone 40 mg/day. Plasmapheresis and six monthly doses of 1000 mg intravenous cyclophosphamide. Prednisone was gradually tapered to 5 mg/day with addition of azathioprine 100 mg/day. Conclusion The association between systemic sclerosis and polyarteritis nodosa is very rare. The co-existence of SSc and vasculitis necessitates modification of the treatment plan.

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Section: Discussionmentioning

confidence: 92%

Overlap between systemic sclerosis and polyarteritis nodosa: A case report

Mahmoud

Elsaid

Zayed

2021

The Egyptian Rheumatologist

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“…We identified six papers describing the clinical features of 12 SSc patients with ANCA‐associated neuropathy (Table 1). 2,3,5–8 In all the reported cases, the SSc diagnosis preceded the ANCA‐associated neuropathy diagnosis. Positive results for anti‐Scl‐70 antibody were observed in five patients (5/12).…”

Section: Discussionmentioning

confidence: 98%

ANCA‐associated neuropathy in systemic sclerosis: A case report and review of literature

Takenaka

Takeichi

Nishi

et al. 2021

J Cutaneous Imm & Allergy

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Systemic sclerosis (SSc) is a multi‐system autoimmune disease. Anti‐neutrophil cytoplasmic antibodies (ANCA) are autoantibodies directed against enzymes found within primary granules of neutrophils and lysosomes in monocytes. Although up to 12% of SSc patients have ANCA, only a minority of these patients develop an overlap syndrome with ANCA‐associated vasculitis. We summarize previous reports on SSc patients with ANCA‐associated neuropathy. In all the reported cases, the SSc diagnosis preceded the ANCA‐associated neuropathy diagnosis. Seven of the eight patients with limited cutaneous SSc had interstitial lung disease (ILD). Thus, patients with ANCA‐associated neuropathy in lSSc may be prone to complication with ILD.

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“…Second, ANCA positivity was found to be more closely related to ILD in SSc patients, regardless of AAV classification [ 6 ]. Third, proteinase 3 (PR3)-ANCA was shown to be associated with neuropathy in SSc patients, although it was not classified as having SSc-AAV-OS [ 7 ]. In addition, our previous study on ANCA positivity in Korean patients with SSc revealed that the detection rate of ANCA was 20.3%, and 1.7% of study subjects could finally be reclassified as having SSc-AAV-OS [ 8 ].…”

Section: Introductionmentioning

confidence: 99%

Overlap syndrome of systemic sclerosis with antineutrophil cytoplasmic antibody-associated vasculitis according to 2022 ACR/EULAR criteria

Ha,

Pyo,

Ahn

et al. 2024

Korean J Intern Med

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Background/Aims: This study applied the 2022 American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) criteria for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) to patients with systemic sclerosis (SSc) and investigated the frequency of overlap syndrome of SSc and AAV (SSc-AAV-OS).Methods: Among the 232 patients diagnosed with SSc, 105 with signs suggestive of small- or medium-vessel vasculitis, which were defined as the present of interstitial lung disease (ILD), peripheral neuropathy, or suspected renal vasculitis, were included in this study and analyzed.Results: Among the 105 SSc patients, the detection rate of ANCA was 19.0%. When the 2022 ACR/EULAR criteria were applied, the frequency of SSc-AAV-OS was 20.0%, which was much higher than 1.7% reported with previous criteria for AAV. ANCA positivity contributed to the reclassification of SSc-AAV-OS more than ANCA negativity in SSc patients with signs suggestive of small- or medium-vessel vasculitis.Conclusions: The frequency of SSc-AAV-OS in SSc patients with signs suggestive of small- or medium-vessel vasculitis at diagnosis was 20.0%. Therefore, we suggest that physicians should perform ANCA tests in SSc patients exhibiting signs suggestive of small- or medium-vessel vasculitis and apply the new criteria for AAV.

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Proteinase 3 (PR3)-antineutrophil cytoplasmic antibody (ANCA)-associated vasculitic neuropathy in diffuse cutaneous systemic sclerosis: a rare duo

Cited by 4 publications

References 13 publications

Overlap between systemic sclerosis and polyarteritis nodosa: A case report

Overlap between systemic sclerosis and polyarteritis nodosa: A case report

ANCA‐associated neuropathy in systemic sclerosis: A case report and review of literature

Overlap syndrome of systemic sclerosis with antineutrophil cytoplasmic antibody-associated vasculitis according to 2022 ACR/EULAR criteria

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