Unique Features of Posterior Ocular Involvement of Whipple’s Disease in a Patient with No Gastrointestinal Symptoms

Bosello, Francesca; Casalino, Giuseppe; Neri, Enrico; Alfano, Alessandro; Bonora, Adriana; Marchini, Giorgio

doi:10.1080/09273948.2020.1859548

“…It concerns up to 6% of patients with WD, and usually regresses after antibiotic therapy (12). The most common images include uveitis, vitritis, retinitis, and optic neuritis (4,5). Super cial ocular and corneal involvements are less common and include super cial punctuate keratitis, crystalline keratopathy, peripheral corneal opacities, neovascularization and brovascular pannus, and scleral nodules (13)(14)(15).…”

Section: Discussion Conclusionmentioning

confidence: 99%

“…Subsequently, central nervous manifestations and other severe complications can occur. Less frequent manifestations, such as spondyloarthritis or ocular involvement as uveitis have been reported, which can mimic in ammatory rheumatic disease (2)(3)(4)(5). Treatment options include an initial phase of intravenous ceftriaxone, trimethoprim/sulfamethoxazole, or a combination of doxycycline and hydroxychloroquine for at least one year (1).…”

Section: Introductionmentioning

confidence: 99%

Uveitis secondary to Whipple’s disease immune reconstitution inflammatory syndrome: a new entity ?

LEQUAIN

¹

,

BRUNET

²

,

SEILLER

³

et al. 2022

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0

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Background: Whipple's disease is a rare infectious systemic condition caused by Tropheryma whipplei which, can involve several organs such as the gastrointestinal tract, joins, skin, central nervous system, and eyes. Because of its non-specific symptoms and frequent join involvement preceding other Whipple's disease symptoms, a relevant percentage of patients are treated as inflammatory arthritis and received immunosuppressive treatment such as tumor necrosis factor α inhibitors, associated with Immune Reconstitution Inflammatory Syndrome (IRIS), complicating antibiotic therapy. Case presentation: A 53-year-old male presented with bilateral knee arthritis, a weight loss of 30 kg in 6 months without diarrhea, a chronic febricula at 38°C, and cognitive disorders. He was under treatment with tumor necrosis factor α for a misdiagnosis of presumed post-viral spondyloarthritis. Given the unusual clinical presentation for spondyloarthritis, further tests were performed as Polymerase Chain Reaction (PCR) in blood, saliva, stools, joint fluid of the left knee, and cerebrospinal fluid and revealing the presence of the T. whipplei genome, confirming the diagnosis of WD and antibiotic treatment was started. In addition, an ophthalmic examination revealed that the patient presented bilateral posterior uveitis and an aqueous humor sample confirmed the presence of T.whippley. Thus, the patient was treated with classical Whipple’s disease therapy and subconjunctival corticosteroid injections. At three months, he presented persistent ocular posterior segment inflammation, leading to repeated PCR tests in blood, saliva, cerebrospinal fluid, stools, and aqueous humor, which were negatives. Therefore, an ocular IRIS was considered in the context of posterior uveitis recurrence after the effectiveness of antibiotic therapy and negative samples. Thus, the patient was treated with systemic corticosteroid therapy, allowing ocular inflammatory signs to disappear in both eyes. Conclusions: This case revealed the existence of IRIS-induced uveitis complicating Whipple’s disease. Therefore, ophthalmologists, rheumatologists, and internists should be aware of this rare complication, particularly in the context of previous immunosuppressive therapy.

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“…Ocular involvement in WD is found in up to 6% of patients, and it is usually found in association with systemic manifestations [ 2 ]. Frequent ocular findings include chronic uveitis (vitritis, retinitis, choroiditis, panuveitis), optic neuritis, and optic nerve edema [ 3 , 4 ]. Ocular surface (crystalline keratopathy, scleral nodules) [ 5 – 7 ], and neuro-ophthalmological involvement (oculomasticatory myorhythmia, supranuclear ophthalmoplegia) is less common [ 8 , 9 ].…”

Section: Introductionmentioning

confidence: 99%

“…In an unlucky twist, atypical presentations of WD can sometimes mimic rheumatic disease such as spondyloarthritis, and immunosuppressive therapy is therefore sometimes inadequately used in these situations [ 3 , 4 , 10 , 11 ]. This can predispose to the development of immune reconstitution inflammatory syndrome (IRIS) when patients are ultimately weaned off immunosuppressive drugs and given adequate antibiotic treatment [ 1 , 10 , 12 ].…”

Section: Introductionmentioning

confidence: 99%

Immune recovery uveitis in Whipple’s disease: an unusual ocular presentation

Lequain,

Abramowicz,

Seiller

et al. 2024

J Ophthal Inflamm Infect

2

0

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Purpose To describe an unusual case of Whipple’s disease (WD) complicated by uveitis, and subsequent paradoxical worsening after effective antibiotic treatment targeting Tropheryma whipplei (TW). Methods Case report. Results A 53-year-old male presented with bilateral knee arthritis, weight loss, chronic low-grade fever, and cognitive disorders. He was under treatment with tumor necrosis factor α inhibitors (TNFi) for seronegative spondyloarthritis. Given this unusual clinical presentation, further investigations were performed and revealed blood, saliva, stool, synovial fluid and cerebrospinal fluid positivity for TW, confirming the diagnosis of systemic WD. Ophthalmologic examination revealed bilateral posterior uveitis and an aqueous humor sample confirmed the presence of intraocular TW. TNFi were stopped, and the patient was subsequently treated with adequate antibiotics (ceftriaxone, followed by doxycycline and hydroxychloroquine), and subconjunctival corticosteroid injections. After a transient improvement of the ocular symptoms, he presented a recurrence of posterior segment inflammation, leading to repeated PCR testing for TW which were negative. Therefore, paradoxical worsening of the inflammation in the context of immune recovery uveitis (IRU) was thought to be the culprit. The patient was treated with systemic corticosteroid therapy, allowing for rapid improvement of the ocular findings. Conclusions This case underlines the possibility of IRU complicating WD. Ophthalmologists, rheumatologists, and internists should be aware of this rare complication, particularly in the context of previous immunosuppressive therapy.

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“…2 Frequent ocular ndings include chronic uveitis (vitritis, retinitis, choroiditis, panuveitis), optic neuritis, and optic nerve edema. 3,4 Ocular surface (crystalline keratopathy, scleral nodules), [5][6][7] and neuro-ophthalmological involvement (oculomasticatory myorhythmia, supranuclear ophthalmoplegia) is less common. 8,9 In an unlucky twist, atypical presentations of WD can sometimes mimic rheumatic disease such as spondyloarthritis, and immunosuppressive therapy is therefore sometimes inadequately used in these situations.…”

Section: Introductionmentioning

confidence: 99%

Immune recovery uveitis in Whipple’s disease: an unusual ocular presentation

LEQUAIN,

ABRAMOWICZ,

SEILLER

et al. 2023

Preprint

0

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Purpose To describe an unusual case of Whipple’s disease (WD) complicated by uveitis, and subsequent paradoxical worsening after effective antibiotic treatment targeting Tropheryma whipplei (TW). Methods Case report. Results A 53-year-old male presented with bilateral knee arthritis, weight loss, chronic low-grade fever, and cognitive disorders. He was under treatment with tumor necrosis factor α inhibitors (TNFi) for seronegative spondyloarthritis. Given this unusual clinical presentation, further investigations were performed and revealed blood, saliva, stool, synovial fluid and cerebrospinal fluid positivity for TW, confirming the diagnosis of systemic WD. Ophthalmologic examination revealed bilateral posterior uveitis and an aqueous humor sample confirmed the presence of intraocular TW. TNFi were stopped, and the patient was subsequently treated with adequate antibiotics (ceftriaxone, followed by doxycycline and hydroxychloroquine), and subconjunctival corticosteroid injections. After a transient improvement of the ocular symptoms, he presented a recurrence of posterior segment inflammation, leading to repeated PCR testing for TW which were negative. Therefore, paradoxical worsening of the inflammation in the context of immune recovery uveitis (IRU) was thought to be the culprit. The patient was treated with systemic corticosteroid therapy, allowing for rapid improvement of the ocular findings. Conclusions This case underlines the possibility of IRU complicating WD. Ophthalmologists, rheumatologists, and internists should be aware of this rare complication, particularly in the context of previous immunosuppressive therapy.

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Unique Features of Posterior Ocular Involvement of Whipple’s Disease in a Patient with No Gastrointestinal Symptoms

Cited by 5 publications

References 9 publications

Uveitis secondary to Whipple’s disease immune reconstitution inflammatory syndrome: a new entity ?

Uveitis secondary to Whipple’s disease immune reconstitution inflammatory syndrome: a new entity ?

Immune recovery uveitis in Whipple’s disease: an unusual ocular presentation

Immune recovery uveitis in Whipple’s disease: an unusual ocular presentation

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