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Fulltext Antibodies in systemic lupus antineutrophil cytoplasmic erythematosus: prevalence, disease activity correlations and organ system associations

Fauzi AR, Kong NCT, Chua MK, Jeyabalan V, Idris MN, Azizah R

Med J Malaysia, 2004 Aug;59(3):372-7.
PMID: 15727383

Systemic Lupus Erythematosus (SLE) is a disease with multiorgan involvement and multiple autoantibody production including antineutrophil cytoplasmic antibodies (ANCA). Despite its reported prevalence in more than one third of SLE patients, the role of ANCA in the pathogenesis or otherwise in SLE remains unresolved. 131 SLE patients had been previously studied for various serologic parameters of disease activity. Their cumulative organ involvement in the course of their disease had also been determined and the Lupus Activity Index (LAI) calculated. Their stored sera were then screened for the presence of ANCA by two methods viz Indirect immunofluorescence (IIF) and also enzyme-linked immunosorbent assay (ELISA). ANCA was present in 24.8% of these SLE patients. The atypical ANCA pattern was predominant and accounted for an overall of 20.6%. Anti-MPO and anti-PR3 were detected in 1.5% of patients respectively. No association was found between ANCA positivity and disease activity. There was also no association of ANCA with specific organ involvement. Despite the high prevalence of ANCA especially the atypical variant in SLE, they probably represent only one of the wide repertoire of autoantibodies found in this disease. Routine testing for ANCA in lupus patients is therefore not recommended.

Matched MeSH terms: Antibodies, Antineutrophil Cytoplasmic/blood*
Fulltext ANCA-associated vasculitis - revisited, and its association with autoimmune thyroid disease

Lai Y, Nik Yahya NH, Ong SG

Med J Malaysia, 2014 Apr;69(2):98-100.
PMID: 25241822

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are a group of uncommon diseases. Given its rarity and multisystem clinical presentation which are usually serious and potentially fatal, prompt recognition and early treatment are of utmost importance. We report a case of AAV that presented with digital infarcts, cutaneous vasculitis, rapidly progressive glomerulonephritis, mononeuritis multiplex, eosinophilia and positive myeloperoxidase (MPO)-ANCA antibody. Apart from renal damage, there was complete recovery in other systems following intravenous cyclophosphamide and high-dose glucocorticoids albeit the response was delayed. This response obviates the need for second-line therapy with newer agent such as rituximab (anti-CD20 monoclonal antibody). We would also like to highlight that this is the first case of AAV that is associated with autoimmune thyroid disease to be reported from Malaysia.

Matched MeSH terms: Antibodies, Antineutrophil Cytoplasmic
Fulltext Cutaneous Vasculitis as Early Presentation of Eosinophilic Granulomatosis with Polyangiitis without Systemic Symptoms

Sulaiman W, Abdullah AC, Chung SF, Karim N, Tang JJ

Oman Med J, 2019 Jul;34(4):345-349.
PMID: 31360325 DOI: 10.5001/omj.2019.67

Eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome) is a rare vasculitis in children. It commonly presents with respiratory symptoms, especially asthma, allergic rhinitis, and peripheral eosinophilia. Involvement of other systems, such as renal and cardiac, may carry a poor prognosis. Anti-neutrophil cytoplasmic antibodies have been found less frequently in children with this condition. We report a case of a 15-year-old male who presented with indurated and pruritic papules on both legs and peripheral eosinophilia without other system involvement. Histopathological findings from a skin biopsy were suggestive of eosinophilic granulomatosis with polyangiitis. Anti-neutrophil cytoplasmic antibodies were negative.

Matched MeSH terms: Antibodies, Antineutrophil Cytoplasmic
Fulltext Upper respiratory tract symptoms, renal involvement and vasculitis: a case report and review of wegener granulomatosis

Said MS

J Clin Med Res, 2010 Aug 18;2(4):189-93.
PMID: 21629538 DOI: 10.4021/jocmr412w

Wegeners Granulomatosis is a condition associated with systemic vasculitis which can present with upper respiratory tract symptoms initially. On September 2001, a 15-year-old girl presented with symptoms of nasal block for 3 weeks. She later developed joint pains and worsening renal status requiring dialysis. A renal biopsy was performed which showed pauci-immune cresentric glomerulonephritis. Her cANCA levels were positive. She was treated with oral cyclophosphamide and steroids and later responded.
KEYWORDS: Wegener granulomatosis; Young girl; Cyclophophamide; cANCA.

Matched MeSH terms: Antibodies, Antineutrophil Cytoplasmic
Clinical and imaging features predictive of orbital granulomatosis with polyangiitis and the risk of systemic involvement

Tan LT, Davagnanam I, Isa H, Taylor SR, Rose GE, Verity DH, et al.

Ophthalmology, 2014 Jun;121(6):1304-9.
PMID: 24560566 DOI: 10.1016/j.ophtha.2013.12.003

Granulomatosis with polyangiitis (GPA), previously Wegener's granulomatosis, requires prompt diagnosis and systemic review to exclude life-threatening disease. However, early diagnosis of orbital GPA may be difficult because anti-neutrophil cytoplasmic antibody (ANCA) and anti-PR3 antibody screening can be negative at presentation and orbital biopsies taken for diagnosis may not show the classic features of GPA. This study was designed to compare GPA with other causes of orbital inflammation and to identify the presenting clinical and imaging features most likely to predict GPA and its systemic spread.

Matched MeSH terms: Antibodies, Antineutrophil Cytoplasmic/blood
Clinical and Imaging Features of Lacrimal Gland Involvement in Granulomatosis with Polyangiitis

Tan LT, Davagnanam I, Isa H, Rose GE, Verity DH, Pusey CD, et al.

Ophthalmology, 2015 Oct;122(10):2125-9.
PMID: 26233627 DOI: 10.1016/j.ophtha.2015.06.026

Lacrimal gland involvement in granulomatosis with polyangiitis (GPA) commonly accompanies orbital disease, but occasionally may be the sole presentation preceding any other organ manifestation or systemic disease. Diagnosis of orbital GPA, especially in patients with lacrimal involvement as the initial presentation, can be difficult because of nonspecific clinical features and lack of diagnostic specificity on histologic and antineutrophilic cytoplasmic antibody (ANCA) testing. Orbital GPA can be associated with a high morbidity from potential visual loss or rapid progression of latent systemic disease, making early diagnosis important. The purpose of this study was to describe the clinical and imaging features of patients with lacrimal gland involvement secondary to GPA and to compare them with those of other orbital inflammatory conditions in the lacrimal gland fossa.

Matched MeSH terms: Antibodies, Antineutrophil Cytoplasmic/blood
Fulltext The atypical presentation of rheumatoid arthritis in an elderly woman: a case report

Devaraj NK

Ethiop J Health Sci, 2019 Jan;29(1):957-958.
PMID: 30700964 DOI: 10.4314/ejhs.v29i1.18

The diagnosis of rheumatologic problem can be difficult, especially if not all the diagnostic criteria or typical clinical features are seen. This includes conditions such as rheumatoid arthritis which needs early diagnosis to start disease modifying drugs (DMARDs) which can improve the prognosis and prevent further joint erosion and organ damage. This case report focused on a similar scenario in an elderly woman initially thought to have osteoarthritis but was diagnosed later with rheumatoid arthritis which brought much relief to her current predicament.

Matched MeSH terms: Antibodies, Antineutrophil Cytoplasmic/blood
"Fleshy" Skin Cellulitis: A Triggering Factor for ANCA Associated Vasculitis

Yang SC, Mustafar R, Kamaruzaman L, Wei Yen K, Mohd R, Cader R

Acta Med Indones, 2019 Oct;51(4):338-343.
PMID: 32041918

A 59-year-old lady with underlying hypothyroidism presented with acute contact dermatitis progressed to cellulitis with superimposed bacterial infection and acute kidney injury. She responded to initial management with antibiotics, but a week later, she had cutaneous and systemic vasculitis. Her skin biopsy consistent with immune-mediated leuko-cytoclastic vasculitis and her blood test was positive for cytoplasmic-anti-neutrophil cytoplasmic antibody (c-ANCA). A diagnosis of ANCA-associated vasculitis was made and she was treated with immunosuppressant with plasmapheresis and hemodialysis support for her kidney failure. Despite aggressive measures, the patient succumbed to her illness. This case report demonstrates that soft tissue infection could trigger the development of ANCA-associated vasculitis whilst a background of hypothyroidism serves as a predisposing factor as both condition were reported separately in a couple of case studies before.

Matched MeSH terms: Antibodies, Antineutrophil Cytoplasmic/blood
Fulltext Immunofluorescence (IF) pattern, autoantigens of anti-neutrophil cytoplasmic antibodies (ANCA) and their clinical associations

Masita Arip, Marlyn Mohammad, Salbiah Nawi, Shahnaz Murad

Jurnal Sains Kesihatan Malaysia, 2010;8(2):37-41.
MyJurnal

Antineutrophil cytoplasmic antibodies (ANCA) are autoantibodies directed against primary granules of neutrophils and monocytes' lysosomes. In general, c-ANCA is strongly associated with vasculitic disorders mainly in ANCA-associated systemic vasculitis (AASV). p-ANCA have been identified in several diseases such as primary (AASV) and secondary vasculitis such as collagen vascular diseases, rheumatoid arthritis and inflammatory bowel diseases given the term 'ANCA-associated disease.' The objective of this study was to determine the rate of ANCA positivity by indirect immunofluorescent (IF) and enzyme linked immunosorbent assay (ELISA) and its association with AASV and ANCA associated diseases. Serum from patients with history suspicion of systemic vasculitis were tested for ANCA by IF. Those samples positive for ANCA by IF were further tested for antibodies against myeloperoxidase (MPO) and proteinase 3 (PR3) using the ELISA. Clinical data from medical records were obtained and analyzed. Of 468 samples, a total of 110 were positive for ANCA by IF. IF results showed a p-ANCA pattern in 96 patients and c-ANCA in 14. Of 110 IF positive ANCA, 45 patients were positive by ELISA. Seventeen were positive for MPO-ANCA, 9 were PR3-ANCA positive and 19 were both MPO and PR3 positive. Only 2 patients were classified AASV ie Wegener granulomatosis and the other with microscopic polyangiitis. The remaining patients (n = 108) may be classified as ANCA associated diseases. Our study showed that pANCA (87.3%) was the more common ANCA pattern and 40.9% of IF positive samples were positive for PR3- and MPOANCA.

Matched MeSH terms: Antibodies, Antineutrophil Cytoplasmic
Fulltext Deep vein thrombosis as a rare presentation in a female with anti-neutrophil cytoplasmic antibodies-associated vasculitis

Wan Ghazali WS, Mohammad N, Ismail AM

Arch Rheumatol, 2017 Jun;32(2):171-174.
PMID: 30375559 DOI: 10.5606/ArchRheumatol.2017.6108

This article aims to report a case of a young female patient with anti-neutrophil cytoplasmic antibodies-associated vasculitis complicated with pulmonary renal syndrome, multiple relapses, and who later developed venous thromboembolism. Pulmonary renal syndrome is a well- recognized and lethal complication; however, incidence of venous thromboembolism has not been well-described. In this article, we described a 38-year-old Malay female patient admitted in 2008 with three-month history of peripheral neuropathy of lower limbs and right ankle ulcers. Initial inflammatory markers were high and perinuclear Anti-Neutrophil Cytoplasmic Antibodies were positive. She was diagnosed as anti-neutrophil cytoplasmic antibodies-associated vasculitis and started on intravenous methylprednisolone with methotrexate. She presented with relapse of skin vasculitis complicated with pulmonary renal syndrome after being stable for one year. She was intubated and proceeded with plasmapheresis and hemodialysis. She completed six cycles of cyclophosphamide. Renal biopsy revealed chronic changes consistent with end stage renal disease. She further relapsed in 2011 with nasal blockage, epistaxis, and nasal deviation. Chest X-ray revealed lung nodules. Prednisolone was increased, her symptoms settled, and she was discharged with azathioprine. She was readmitted at the end of the same year due to two-day history of right deep vein thrombosis and she later succumbed to methicillin-resistant Staphylococcus aureus sepsis.

Matched MeSH terms: Antibodies, Antineutrophil Cytoplasmic