Sjoegren's Syndrome with Multiple Cystic Lesions and Pulmonary Arteriovenous Fistulae

Taniguchi, Hirokazu; Miwa, Atsuo; Abo, Hitoshi; Doi, Hiroshi; Izumi, Saburo

doi:10.2169/internalmedicine.47.1529

Cited by 4 publications

(2 citation statements)

References 6 publications

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“…The etiology of the AVM could not be confirmed from histological findings. [ 14 ] Gupta et al also described the eccentric vessel location of multiple cysts in patients with SS although they could not find any AVM. [ 13 ] Our case is the second case of SS-associated pulmonary AVM reported globally and the first case of recurrence of pulmonary AVMs with aggravating multiple lung cysts in a patient with SS.…”

Section: Discussionmentioning

confidence: 99%

Recurrent pulmonary arteriovenous malformation in a patient with Sjögren syndrome: A case report

et al. 2022

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Rationale: Pulmonary manifestations of Sjögren syndrome (SS) are variable and may involve the airway or lung parenchyma and increase the risk of vascular and malignant disease. However, to date, only one case of pulmonary arteriovenous malformation (AVM) has been reported in a patient with SS. Here, we report a rare case of recurrent pulmonary AVMs with aggravating multiple cysts in a patient with SS during a period of 14 years. Patient concerns: A 45-year-old woman was diagnosed with SS and pulmonary AVM in the right lung. Her AVMs were embolized successfully and she was followed up annually for 14 years. Eleven years after the initial treatment, her chest computed tomography showed new pulmonary AVMs in the left lung with aggravating multiple cysts. Diagnosis: We diagnosed her with SS according to the American–European consensus group criteria of 2010. Chest computed tomography and angiographic findings confirmed the recurrence of pulmonary AVMs. Interventions: The patient’s recurrent pulmonary AVMs were successfully treated by embolization. Outcomes: Although her multiple cystic lung lesions had been aggravating during 14 years, she received embolization for the pulmonary AVMs twice and developed no complication related to these procedures. Currently, the patient is 56 years old and still alive with good performance state. Lessons: To date, only one case of pulmonary AVM has been reported in a patient with SS. The patient died 2.5 years after the diagnosis without recurrence of AVM. Here, we present a rare case of recurrent pulmonary AVMs associated with aggravating multiple cysts in both lungs, which were observed during long-term follow-up, in a patient with SS.

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Section: Discussionmentioning

confidence: 99%

Recurrent pulmonary arteriovenous malformation in a patient with Sjögren syndrome: A case report

et al. 2022

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“…It was reported that Sjögren's syndrome could be associated with pulmonary arteriovenous fistula 6 and vaginoperineal fistula after perianal surgery 7 . However, no case of Sjögren's syndrome associated with the development of lacrimal fistula has been reported to date.…”

Section: Introductionmentioning

confidence: 99%

A case with acquired lacrimal fistula due to Sjögren's syndrome

Ishikawa

Shoji

Nishiyama

et al. 2019

American Journal of Ophthalmology Case Reports

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Purpose To report a case with lacrimal fistula presumably associated with Sjögren's syndrome. Observations A patient with Sjögren's syndrome was referred to our hospital for fistula from the inferior lacrimal canaliculus to the palpebral conjunctiva of her left lower eyelid. She also revealed severe dry eye in both eyes. She had no history of trauma or congenital lacrimal fistula. She was administering 0.3% purified sodium hyaluronate and 3% diquafosol sodium solution 6 times a day for dry eye. The Schirmer-1 test indicated that tear secretion was 0 mm for 5 min for both eyes. She was diagnosed with Sjögren's syndrome based on increased levels of blood Sjögren's-syndrome-related antigen A and Sjögren's-syndrome-related antigen B antibodies, decreased saliva volume, and lip biopsy. We performed silicone tube intubation and patched the fistula with conjunctiva. We observed the lacrimal sac and nasolacrimal duct under lacrimal micro-endoscopy; there was no bacterial concretion, obstruction, and inflammation of lacrimal mucosa. After the operation, her symptoms improved and lacrimal perforation healed after the removal of the silicone tube. Conclusions and importance Sjögren's syndrome can cause not only corneal perforation but also mucosal perforation, which may lead to a lacrimal fistula. Sjögren's syndrome patients with severe dry eye should be managed carefully.

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Can We Differentiate Between Primary Sjögren Syndrome and Idiopathic Multicentric Castleman Disease Based on the Characteristics of Pulmonary Cysts?

Zhou,

Zhang,

Liu

et al. 2024

Journal of Thoracic Imaging

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Purpose: To identify radiological characteristics that could help differentiate cystic lung diseases between primary Sjögren syndrome (pSS) and idiopathic multicentric Castleman disease (iMCD). Patients and Methods: Patients with pSS or iMCD who had cysts were enrolled. Cyst characteristics (number, size, morphology, and distribution) and other accompanying manifestations (nodules, ground-glass opacities, calcification, and thickening of the bronchovascular bundles and interlobular septa) were compared between them. Results: Eleven patients with pSS and 25 patients with iMCD were eligible for our study. Eleven patients with pSS (100.0%) and 23 patients with iMCD (92.0%) had round or oval cysts. None of the patients with pSS had irregular cysts, but 21 (84.0%) patients with iMCD had irregular cysts (P = 0.005). Smooth-walled cysts were present in 11 patients with pSS (100.0%) and 18 patients with iMCD (72.0%). Only 1 patient with pSS (9.1%) exhibited non-smooth-walled cysts, whereas 23 patients with iMCD (92.0%) had non-smooth-walled cysts (P = 0.003). The presence of nodules was common in both groups (P = 1.000). However, the nodules were more likely to be larger and more numerous in patients with iMCD (P < 0.001). Cysts with mural nodules (52.2%) and central nodules (47.8%) were only observed in iMCD (P = 0.007). Conclusion: Although regular and smooth-walled cysts were common in the 2 diseases, irregular and non-smooth-walled cysts were more often associated with iMCD than pSS. Nodules in iMCD tended to be larger and more numerous, and a close positional relationship between nodules and cysts was only observed in iMCD.

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Sjoegren's Syndrome with Multiple Cystic Lesions and Pulmonary Arteriovenous Fistulae

Abstract: Abstract

Cited by 4 publications

References 6 publications

Recurrent pulmonary arteriovenous malformation in a patient with Sjögren syndrome: A case report

Recurrent pulmonary arteriovenous malformation in a patient with Sjögren syndrome: A case report

A case with acquired lacrimal fistula due to Sjögren's syndrome

Can We Differentiate Between Primary Sjögren Syndrome and Idiopathic Multicentric Castleman Disease Based on the Characteristics of Pulmonary Cysts?

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