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Bilateral interstitial keratitis, erythema nodosum and atrial fibrillation as presenting signs of polyarteritis nodosa.

ABSTRACT: Purpose:To report a case of Polyarteritis Nodosa (PAN) presenting as bilateral episcleritis and interstitial keratitis along with erythema nodosum and atrial fibrillation and to review the ophthalmic literature on PAN with anterior segment findings. Observations:A 35-year old man presented with a two-month history of bilateral episcleritis, skin lesions consistent with erythema nodosum, joint effusions and episodes of prolonged diarrhea and atrial fibrillation. Ophthalmic examination was significant for bilateral diffuse episcleral injection and nummular corneal stromal infiltrates. The patient underwent an extensive infectious and inflammatory work-up that was negative except for a very elevated erythrocyte sedimentation rate (123 mm/h, normal < 20 mm/h) and C-reactive protein (51 mg/L, normal < 5 mg/L). In order to rule out inflammatory bowel disease upper endoscopy and colonoscopy were performed. Biopsies of the gastrointestinal mucosa were positive for a small- and medium-vessel necrotizing vasculitis consistent with polyarteritis nodosa. Disease control was achieved with systemic prednisone and azathioprine. Upon self-tapering both medications the patient developed hearing loss and interstitial keratitis recurred, hence the diagnosis of Cogan's syndrome/PAN was made. Intravenous pulse steroids were administered with resolution of his symptoms. The patient continues to be on azathioprine without disease recurrence for 1.5 years. ? review of the ophthalmic literature on PAN with anterior segment findings revealed only 10 cases; of these, 6 had originally presented with ocular manifestations alone (scleritis, peripheral ulcerative keratitis, episcleritis, dacryoadenitis) and 4 of these 6 were lethal due to delay in diagnosis. Conclusion and importance:Early diagnosis of PAN is crucial, as the five-year mortality rate is close to 90%; upon initiation of systemic immunosuppression the mortality rate drops to 20%. Though PAN manifestations in the anterior segment are rare, a high index of suspicion is warranted in cases of bilateral episcleritis and interstitial keratitis.

SUBMITTER: Vingopoulos F

PROVIDER: S-EPMC7044707 | biostudies-literature | 2020 Jun

REPOSITORIES: biostudies-literature

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Bilateral interstitial keratitis, erythema nodosum and atrial fibrillation as presenting signs of polyarteritis nodosa.

Vingopoulos Filippos F Karagiotis Theodoros T Palioura Sotiria S

American journal of ophthalmology case reports 20200221

<h4>Purpose</h4>To report a case of Polyarteritis Nodosa (PAN) presenting as bilateral episcleritis and interstitial keratitis along with erythema nodosum and atrial fibrillation and to review the ophthalmic literature on PAN with anterior segment findings.<h4>Observations</h4>A 35-year old man presented with a two-month history of bilateral episcleritis, skin lesions consistent with erythema nodosum, joint effusions and episodes of prolonged diarrhea and atrial fibrillation. Ophthalmic examinat ...[more]

PMID: 32140612

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Project description:Leprosy is a disease caused by Mycobacterium leprae where the clinical spectrum correlates with the patient immune response. Erythema Nodosum Leprosum (ENL) is an immune-mediated inflammatory complication, which causes significant morbidity in affected leprosy patients. The underlying cause of ENL is not conclusively known. However, immune-complexes and cell-mediated immunity have been suggested in the pathogenesis of ENL. The aim of this study was to investigate the regulatory T-cells in patients with ENL. Forty-six untreated patients with ENL and 31 non-reactional lepromatous leprosy (LL) patient controls visiting ALERT Hospital, Ethiopia were enrolled to the study. Blood samples were obtained before, during and after prednisolone treatment of ENL cases. Peripheral blood mononuclear cells (PBMCs) were isolated and used for immunophenotyping of regulatory T-cells by flow cytometry. Five markers: CD3, CD4 or CD8, CD25, CD27 and FoxP3 were used to define CD4+ and CD8+ regulatory T-cells. Clinical and histopathological data were obtained as supplementary information. All patients had been followed for 28 weeks. Patients with ENL reactions had a lower percentage of CD4+ regulatory T-cells (1.7%) than LL patient controls (3.8%) at diagnosis of ENL before treatment. After treatment, the percentage of CD4+regulatory T-cells was not significantly different between the two groups. The percentage of CD8+ regulatory T-cells was not significantly different in ENL and LL controls before and after treatment. Furthermore, patients with ENL had higher percentage of CD4+ T-ells and CD4+/CD8+ T-cells ratio than LL patient controls before treatment. The expression of CD25 on CD4+ and CD8+ T-cells was not significantly different in ENL and LL controls suggesting that CD25 expression is not associated with ENL reactions while FoxP3 expression on CD4+ T-cells was significantly lower in patients with ENL than in LL controls. We also found that prednisolone treatment of patients with ENL reactions suppresses CD4+ T-cell but not CD8+ T-cell frequencies. Hence, ENL is associated with lower levels of T regulatory cells and higher CD4+/CD8+ T-cell ratio. We suggest that this loss of regulation is one of the causes of ENL.

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Dataset Information

Bilateral interstitial keratitis, erythema nodosum and atrial fibrillation as presenting signs of polyarteritis nodosa.

Publications

Bilateral interstitial keratitis, erythema nodosum and atrial fibrillation as presenting signs of polyarteritis nodosa.

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