MPO-ANCA associated vasculitis with mononeuritis multiplex following influenza vaccination

Background:ANCA-associated vasculitis sometimes presents mononeuritis multiplex which worsens the prognosis and activity of daily living in patients.Objectives:This study aimed to determine the clinical feature of mononeuritis multiplex associated with AAV.Methods:Consecutive patients with AAV who visited Tokyo Medical Center between April 2006 and December 2019 were included in this study. We examined the following clinical features: prevalence of neuropathy, age of onset, sex, the worst blood test values before the initial therapy (white blood cell count (WBC), eosinophil count (Eo), MPO-ANCA, PR3-ANCA, and C-reactive protein(CRP) levels), manual muscle testing (MMT)score of 20 muscles (Max 100 points), and time (days) from the initial symptoms to the initial induction therapies.Results:A total of 89 patients with AAV were identified. Among them, 19 patients had eosinophilic granulomatosis with polyangiitis (EGPA) (8 males and 11 females, mean age 63.3 ± 2.7), 9 patients had granulomatosis with polyangiitis (GPA) (0 males, 9 females, mean age 75.6 ± 3.9), and 61 patients had microscopic polyangiitis (MPA) (17 males, 44 females, mean age 78.2 ± 1.5). Of the 89 AAV patients, 26 had sensory neuropathy (15/19 EGPA (78.9%), 11/61 MPA (18.0%), and 0/9 GPA (0%)). Motor neuropathy was observed in 19 patients (EGPA 14/19 (73.7%), MPA 5/61 (8.2%), GPA 0/9 (0%)). 15 patients had both sensory and motor neuropathies (EGPA 12/19 (63.2%), MPA 3/61 (4.9%), GPA 0 (0%)). In patients with both sensory and motor neuropathy, sensory impairment preceded in all cases. Among patients with neuropathy, the time from initial symptoms to initial induction therapy in patients with and without motor neuropathy was 34 ± 10.1 days and 30 ± 10.8 days (p = 0.776), respectively. Also, when comparing those who were treated within 7 days from the onset of movement disorders with those who were treated later, MMT score two weeks after the start of treatment were 92.15 ± 1.47 vs. 91.25 ± 2.65 (p = 0.77).Between the patients with EGPA with and without sensory neuropathy, there were no significant differences in the following: highest WBC(19620.0 ± 2082.6 vs. 19350.0 ± 4033.5 cells/uL (p = 0.953)), highest Eo(10790.6 ± 1774.8 vs 12440.8 ± 3436.9 cells/uL (p = 0.6750)), and highest CRP levels (4.467 ± 0.96 vs 2.70 ± 1.85 mg/dL (p = 0.41)) before the initial therapy. On the other hand, comparing the EGPA patients with and without motor neuron disorder, CRP levels were significantly higher in those with motor impairment than those without(WBC 20978.6 ± 2049.8 vs. 15600.0 ± 3429.9 cells/uL (p = 0.20); Eo 12213.4 ± 1775.5 vs. 8127.0 ± 2971.0 cells/uL (p = 0.25); CRP 5.13 ± 0.89 vs. 1.20 ± 1.48 mg/dL (p = 0.04)). And in patients with motor neuropathy, the decrease in MMT score was significantly correlated with the worst levels of CRP(p = 0.001)while the decrease was not correlated with the other blood tests. ANCA levels were not associated with sensory or motor neuropathy. In similar analyses of patients with MPA and GPA, there were no significant findings.Conclusion:Worst CRP levels before the initial therapy can be a poor prognosis factor for motor neuropathy in patients with EGPA. Therefore, EGPA patients with high CRP levels need to be paid more attention to because of possible development of motor neuropathy.Disclosure of Interests:satoshi hama: None declared, Yutaro Hayashi: None declared, Keisuke Izumi Grant/research support from: Asahi Kasei Pharma, Takeda Pharmaceutical Co., Ltd., Speakers bureau: Asahi Kasei Pharma Corp, Astellas Pharma Inc., Bristol Myers Squibb, Chugai Pharmaceutical Co., Ltd., Eli Lilly Japan K.K., Mitsubishi Tanabe Pharma Co., Misako Higashida-Konishi: None declared, mari ushikubo: None declared, kumiko akiya: None declared, yutaka okano: None declared, Hisaji Oshima: None declared

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26. ANCA-associated vasculitis in a patient with a PET-avid lung lesion

Rheumatology Advances in Practice ◽

10.1093/rap/rkz029.002 ◽

2019 ◽

Vol 3 (Supplement_1) ◽

Author(s):

Shawki El-Ghazali ◽

Michael Naughton

Keyword(s):

Nasal Polyps ◽

Granulomatosis With Polyangiitis ◽

Conflicts Of Interest ◽

Lung Lesion ◽

Nerve Conduction Studies ◽

Mononeuritis Multiplex ◽

Anca Associated Vasculitis ◽

Lung Malignancy ◽

History Of

Abstract Introduction Vasculitis can be a primary or secondary process. Underlying secondary causes include infection, malignancy and other autoimmune conditions. This case describes a patient with findings consistent with ANCA-associated vasculitis. As part of investigations, she was identified as having a PET-avid lung lesion for which she is currently undergoing further investigation via the respiratory team. From a rheumatological perspective she is receiving treatment for her vasculitis, however at present it is unclear whether this is a secondary paraneoplastic phenomenon. This case explores the nature of her presentation and the relationship of primary versus secondary paraneoplastic vasculitis. Case description A 75-year-old lady presented to hospital with a 6-week history of fatigue, pyrexia and myalgia. During this period she additionally noted numbness/neuropathic pains affecting her feet. She had a background of COPD/asthma diagnosed 10 years previously. She had additionally undergone polypectomy for nasal-polyps and had underwent right mastectomy in 1990 for breast cancer. She was noted to have raised inflammatory markers (CRP 256) and eosinophilia. She was deemed to have an infective exacerbation of COPD/asthma although at the time she had limited respiratory symptoms and CXR was reported normal. She was initiated on a 5-day course of prednisolone 40mg and antibiotics. Her symptoms appeared to respond to treatment, as did her CRP and eosinophilia, and she was discharged. Unfortunately she re-presented with similar symptoms within a few days. Further investigation and blood tests demonstrated cANCA antibodies with PR3 of 128 IU. Urine PCR was mildly raised at 30mg/mmol. She was reviewed by the rheumatology team, and likely diagnosis of ANCA-associated vasculitis was made. It was noted however that while her serology was suggestive of granulomatosis with polyangiitis (GPA), her clinical picture of eosinophilia, COPD/asthma and nasal-polyps was more consistent with eosinophilic granulomatosis with polyangiitis (EGPA). She was re-initiated on prednisolone 40mg and demonstrated clinical response. As part of work-up, CT-CAP was arranged. This reported a spiculated lesion of the right-upper lung lobe with appearances concerning for malignancy. Subsequent PET-CT confirmed this be PET-avid, with additional suspicious nodules. She was additionally arranged for nerve-conduction studies regarding her feet symptoms, with findings being consistent with mononeuritis multiplex. From a rheumatological perspective, she has been initiated on azathioprine and prednisolone is gradually being reduced. She has ongoing follow up with respiratory regarding her lung lesion, with initial biopsy being inconclusive. Further attempt at gaining histology is currently awaited. Discussion From a rheumatological perspective, aspects of this case support a diagnosis of ANCA-associated vasculitis. Presence of cANCA with PR3 antibodies are typically associated with GPA, although her eosinophilia, asthma and previous history of nasal polyps would be more consistent with EGPA. The features of mononeuritis multiplex on nerve conduction studies would also support an underlying diagnosis of vasculitis. Although there is a documented relationship between malignancy and vasculitis, this appears to be relatively rare with a 5-8% association. Haematological malignancies such as lymphoma and MDS have a greater association in comparison to solid tumours. Typical manifestation of paraneoplastic vasculitis is that of cutaneous involvement, particularly of leukocytoclastic vasculitis. Reports suggest improvement of secondary vasculitis on treatment of the underlying condition. With regards to the association of ANCA-vasculitis and solid tumour malignancy, cases appear to be less well-described. The majority of case studies exploring the link between these conditions are primarily in the context of secondary malignancy occurring after immunosuppressive therapy (such as cyclophosphamide) for vasculitis. Review of literature outside of this context, especially in regards of specifically lung malignancy is limited – so a consistent established link may be less apparent. Of the limited studies, interestingly there have been reports of patients with biopsy proven lung malignancy and raised MPO/PR3, with levels normalising following treatment of the underlying lesion. As described in this case, our patient currently awaits further investigation regarding her lung lesion and subsequent review of its definitive intervention. Should investigations confirm malignancy and this be treated, then it would be interesting to see whether there is clinical resolution of her systemic vasculitis secondary to this. Key learning points This case demonstrates the presence of 2 conditions of ANCA-associated vasculitis and possible lung malignancy which were both diagnosed during the same admission. Literature suggests possible association between vasculitis and malignancy in a general sense, however specific description of ANCA-vasculitis and lung malignancy has been rarely described. At present, this patient’s vasculitis is being managed whilst definitive intervention is being arranged for her underlying lung lesion. Continued follow up from both the rheumatologists and respiratory team is ongoing and response of her condition will be assessed pending intervention of her underlying lung lesion. Conflicts of interest The authors have declared no conflicts of interest.

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Influenza vaccination does not result in an increase in relapses in patients with ANCA-associated vasculitis

Nephrology Dialysis Transplantation ◽

10.1093/ndt/gfm640 ◽

2007 ◽

Vol 23 (2) ◽

pp. 654-658 ◽

Cited By ~ 38

Author(s):

P. M. Stassen ◽

J.-S. F. Sanders ◽

C. G. M. Kallenberg ◽

C. A. Stegeman

Keyword(s):

Influenza Vaccination ◽

Anca Associated Vasculitis

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Spectrum and Prognosis of Antineutrophil Cytoplasmic Antibody–associated Vasculitis-related Bronchiectasis: Data from 61 Patients

The Journal of Rheumatology ◽

10.3899/jrheum.190313 ◽

2019 ◽

Vol 47 (10) ◽

pp. 1522-1531 ◽

Cited By ~ 1

Author(s):

Raphael Lhote ◽

Marie Chilles ◽

Matthieu Groh ◽

Xavier Puéchal ◽

Philippe Guilpain ◽

...

Keyword(s):

Median Time ◽

Granulomatosis With Polyangiitis ◽

Microscopic Polyangiitis ◽

Fold Increase ◽

Antineutrophil Cytoplasmic Antibody ◽

Mononeuritis Multiplex ◽

Biological Features ◽

Anca Associated Vasculitis ◽

Worse Prognosis

ObjectiveTo report on a large series of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and bronchiectasis, with a specific focus on the timeline of occurrence of both features.MethodsRetrospective nationwide multicenter study of patients diagnosed with both AAV and bronchiectasis.ResultsSixty-one patients were included, among whom 27 (44.25%) had microscopic polyangiitis (MPA), 27 (44.25%) had granulomatosis with polyangiitis (GPA), and 7 (11.5%) had eosinophilic GPA. Thirty-nine (64%) had myeloperoxidase (MPO)-ANCA and 13 (21%) had proteinase 3–ANCA. The diagnosis of bronchiectasis either preceded (n = 25; median time between both diagnoses: 16 yrs, IQR 4–54 yrs), was concomitant to (n = 12), or followed (n = 24; median time between both diagnoses: 1, IQR 0–6 yrs) that of AAV. Patients in whom bronchiectasis precedes the onset of AAV (B-AAV group) have more frequent mononeuritis multiplex, MPA, MPO-ANCA, and a 5-fold increase of death. The occurrence of an AAV relapse tended to be protective against bronchiectasis worsening (HR 0.6, 95% CI 0.4–0.99, P = 0.049), while a diagnosis of bronchiectasis before AAV (HR 5.8, 95% CI 1.2–28.7, P = 0.03) or MPA (HR 18.1, 95% CI 2.2–146.3, P = 0.01) were associated with shorter survival during AAV follow-up.ConclusionThe association of bronchiectasis with AAV is likely not accidental and is mostly associated with MPO-ANCA. Patients in whom bronchiectasis precedes the onset of AAV tend to have distinct clinical and biological features and could carry a worse prognosis.

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A case of ANCA associated vasculitis presented with mononeuritis multiplex

BIRDEM Medical Journal ◽

10.3329/birdem.v9i3.43094 ◽

2019 ◽

Vol 9 (3) ◽

pp. 257-260

Author(s):

Tanbin Rahman ◽

Md Rashedul Islam ◽

Mohammad Sakhawat Hossen Khan ◽

Sharif Mohammad Ruhul Quddus ◽

Dilruba Alam ◽

...

Keyword(s):

Inflammatory Cell ◽

Granulomatosis With Polyangiitis ◽

Microscopic Polyangiitis ◽

Systemic Vasculitis ◽

Antineutrophil Cytoplasmic Antibody ◽

Mononeuritis Multiplex ◽

Good Clinical Response ◽

Standard Regimen ◽

Anca Associated Vasculitis ◽

Common Manifestation

Mononeuritis multiplex is a common manifestation of many illnesses which includes diabetes, leprosy, malignancy and certain types of systemic vasculitis. The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of rare diseases which show typical characteristic inflammatory cell infiltration and blood vessel wall necrosis. AAV syndromes include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilicgranulomatosis with polyangiitis (EGPA). Here we present a patient who presented with mononeuritis multiplex and had features ofEGPA. The patient was treated with standard regimen of steroids and pulsed cyclophosphamide and she achieved good clinical response. Birdem Med J 2019; 9(3): 257-260

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