Relationship of ocular presentation in granulomatosis with polyangiitis to autoantibodies and disease activity

Zusammenhang zwischen okulärer Manifestation bei Granulomatose mit Polyangiitis und Autoantikörpern sowie Krankheitsaktivität

Abstract

Objective

The aim of this study was to assess disease characteristics, autoantibodies, and disease activity in granulomatosis with polyangiitis (GPA) patients with ocular manifestations.

Methods

The cohort included 46 GPA patients visiting the ophthalmology clinic. Ocular manifestations were recorded, clinical and slit lamp examinations were performed. The Birmingham Vasculitis Activity Score (BVAS) was recorded. Laboratory investigations and the antineutrophil cytoplasmic antibody (ANCA) assay were performed.

Results

Median age of the 22 male and 24 female patients was 44.5 (32–63) years, median disease duration 6.5 (1–16) years. Ocular manifestations were present in all patients: 12 (26.1%) had proptosis; 40 (87%) had scleritis/episcleritis, with perforation in 3 (6.5%); 33 (71.7%) had keratoconjunctivitis (KC), with acute infiltrative stromal keratitis in 11, peripheral ulcerative keratitis in 15, and sclerosing keratitis in 11 patients. Uveitis was present in 11 (23.9%) and retinal changes including vasculitis, exudates, and hemorrhage were present in 7 (15.2%). Blurred vision was present in 43 (93.5%) patients and 2 (4.3%) had vision loss. Glaucoma was present in 4 (8.7%) and hypotony in 2 (4.3%) patients. Involvement was bilateral in 32 (69.6%) patients. Rheumatoid factor (RF) was positive in 56.5% and significantly associated with uveitis (p = 0.04), while antinuclear antibody (ANA) was positive in 45.7% and significantly associated with KC (p = 0.04). BVAS tended to be higher in patients with uveitis (p = 0.49).

Conclusion

Ocular involvement must be considered in all GPA patients and referral to an experienced ophthalmologist is mandatory for proper management and improved outcome of such a rare systemic disease. ANA and RF positivity may raise suspicion for KC or uveitis, respectively. There was a remarkable association between uveitis and disease activity.

Zusammenfassung

Ziel

Ziel der vorliegenden Studie war es, Krankheitsmerkmale, Autoantikörper und Krankheitsaktivität bei Patienten mit Granulomatose und Polyangiitis (GPA) bei Vorliegen okulärer Manifestationen zu untersuchen.

Methoden

Die Kohorte umfasste 46 GPA-Patienten der ophthalmologischen Klinik. Die okulären Manifestationen wurden dokumentiert, es erfolgte eine klinische und eine Spaltlampenuntersuchung. Außerdem wurde der Birmingham Vasculitis Activity Score (BVAS) ermittelt. Neben anderen Laboruntersuchungen wurde die Bestimmung der antineutrophilen zytoplasmatischen Antikörper (ANCA) durchgeführt.

Ergebnisse

Das Durchschnittsalter der 22 männlichen und 24 weiblichen Patienten betrug 44,5 (32–63) Jahre, die mittlere Krankheitsdauer lag bei 6,5 (1–16) Jahren. Bei allen Patienten lagen okuläre Manifestationen vor: Bei 12 (26,1 %) eine Proptosis bulbi; 40 (87 %) wiesen eine Skleritis/Episkleritis auf, 3 mit Perforation 3 (6,5 %); bei 33 (71,7 %) bestand eine Keratokonjunktivitis (KC), mit akuter infiltrativer stromaler Keratitis in 11 Fällen, mit peripherer ulzerativer Keratitis bei 15 und mit sklerosierender Keratitis bei 11 Patienten. Eine Uveitis lag bei 11 Patienten vor (23,9 %), und Retinaveränderungen einschließlich Vaskulitis, Exudaten und Blutungen bestanden in 7 Fällen (15,2 %). Verschwommensehen bestand bei 43 (93,5 %) Patienten, und in 2 Fällen (4,3 %) lag eine Einschränkung der Sehkraft vor. Ein Glaukom bestand in 4 (8,7 %) und eine Hypotonie in 2 (4,3 %) Fällen. Bei 32 (69,6 %) Patienten war die Beteiligung bilateral. Der Rheumafaktor (RF) war in 56,5 % der Fälle positiv und in signifikanter Weise mit einer Uveitis assoziiert (p = 0,04), während antinukleäre Antikörper (ANA) in 45,7 % der Fälle positiv und signifikant mit einer KC assoziiert waren (p = 0,04). Der BVAS war bei Patienten mit Uveitis tendenziell höher (p = 0,49).

Schlussfolgerung

Die okuläre Beteiligung muss bei allen GPA-Patienten berücksichtigt werden, eine Überweisung an einen erfahrenen Ophthalmologen ist zwingend für die angemessene Versorgung und einen besseren Verlauf bei einer solchen seltenen systemischen Erkrankung. ANA- und RF-Positivität können den Verdacht auf eine KC oder Uveitis wecken. Es bestand eine beachtliche Assoziation zwischen einer Uveitis und der Krankheitsaktivität.

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Correspondence to Prof. T. A. Gheita MD.

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T. A. Gheita and E. M. Abd El Latif declare that they have no competing interests.

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. Informed consent was obtained from all individual participants included in the study. Additional informed consent was obtained from all individual participants from whom identifying information is included in this article.

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Gheita, T.A., Abd El Latif, E.M. Relationship of ocular presentation in granulomatosis with polyangiitis to autoantibodies and disease activity. Z Rheumatol 78, 281–286 (2019). https://doi.org/10.1007/s00393-018-0495-5

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Keywords

  • Anti-neutrophil cytoplasmic antibody-associated vasculitis
  • Rheumatoid factor
  • Uveitis
  • Keratoconjunctivitis
  • Ophthalmologists

Schlüsselwörter

  • Mit antineutrophilen zytoplasmatischen Antikörpern assoziierte Vaskulitis
  • Rheumafaktor
  • Uveitis
  • Keratokonjunktivitis
  • Ophthalmologen