A Rare Case of Kussmaul Disease (Sialodochitis Fibrinosa)

Ray, Amitava; Burgin, Sarah J.; Spector, Matthew E.

doi:10.4303/jcrm/235894

Cited by 9 publications

(16 citation statements)

References 4 publications

(3 reference statements)

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“…We defined 'eosinophilic sialodochitis' as recurrent painful major salivary gland swelling associated with eosinophil-rich mucus plugs or the histopathologic finding of sialodochitis with a periductal eosinophilic infiltrate. This definition excluded cases of recurrent salivary gland swelling associated with fibrinous mucus plugs and ductal abnormalities, but lacking evidence for eosinophilic infiltration of the major salivary glands (Londe and Pelz, 1933;Ammar-Khodja, 1971;Laudenbach et al, 1985;Ericson et al, 1991;Ray et al, 2015;Hayashi et al, 2016). These cases have been described in children, often in association with atopic illness, and in adults without atopy (Laudenbach et al, 1985).…”

Section: Literature Review and Case Definitionmentioning

confidence: 99%

“…Since 1971, the diagnosis ‘sialodochitis fibrinosa of Kussmaul’ has been applied to cases of recurrent salivary gland swelling with fibrinous mucus plugs (Ammar‐Khodja, ). There are only three reports of this entity in the English medical literature (as judged by a PubMed search) (Chikamatsu et al , ; Shirasaki et al , ; Ray et al , ). However, it has been analyzed in a series of over 35 publications in Japan since 1975.…”

Section: Introductionmentioning

confidence: 99%

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Eosinophilic sialodochitis: redefinition of ‘allergic parotitis’ and ‘sialodochitis fibrinosa’

et al. 2016

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Sialodochitis fibrinosa and allergic parotitis have described rare patients with recurrent salivary gland swelling and mucus plugs, often with atopy. We have evaluated three patients with atopic disease, recurrent salivary gland swelling and an eosinophilic sialodochitis. Two had eosinophil-rich mucus plugs. 56 additional cases were identified in a medical literature database search, each defined by recurrent salivary gland swelling associated with eosinophil-rich mucus plugs or sialodochitis with periductal eosinophilic infiltration. The majority (78%) were reported from Japan. Females were predominantly affected (F:M=2.3) with a median age of 47 years at evaluation. The parotid and submandibular glands were involved, respectively, in 71% and 46%. Allergic symptoms were present in 66%, atopic disease in 63% of those with reported allergy testing, and blood eosinophilia in 71%. Contrast sialography and other imaging modalities documented ductal dilatation in 82%. Treatments included anti-allergic medications (58%), systemic glucocorticoids (25%), duct cannulation with irrigation, steroid injection, and/or duct dilatation (36%), and glandular resection (19%). We recommend the diagnosis “eosinophilic sialodochitis” be applied to patients who meet this case definition. The disease is a unique cause of chronic recurrent salivary gland swelling. Its likely allergic etiology may be amenable to current or future biologic therapies.

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Section: Literature Review and Case Definitionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Eosinophilic sialodochitis: redefinition of ‘allergic parotitis’ and ‘sialodochitis fibrinosa’

et al. 2016

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“…Most reports indicate allergy as an etiology of ES and emphasize eosinophilia in both mucus plugs and peripheral blood, as well as elevated serum IgE. Histopathological studies show eosinophilic infiltration around the large salivary gland ducts 4,8–10 . Treatment for ES includes the abovementioned conventional options as well as anti‐anaphylactic treatment 4,7 …”

Section: Introductionmentioning

confidence: 99%

Eosinophilic Sialodochitis: A Type of Chronic Obstructive Sialadenitis Related to Allergy

et al. 2020

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Objectives To investigate the clinical, laboratory, radiological, histopathological, and immunohistochemical features, and the expression of allergy‐related cytokines in eosinophilic sialodochitis (ES). Methods Thirty‐eight patients diagnosed with chronic obstructive sialadenitis (COS) who had undergone glandular excision or incisional biopsy were enrolled. Seventeen patients with comorbid atopic disease and increased ductal tissue eosinophils comprised the ES group, while 21 patients comprised the COS group. The clinicopathological features and allergy‐related cytokine expression were compared between groups. Results The ES group frequently involved multiple, bilateral major salivary glands, and the number of glands was significantly greater than the COS group (2.8 ± 1.1 vs. 1.2 ± 0.4, P < .001). Serum immunoglobulin (Ig) E was elevated in 91% of patients in ES group (419 ± 357 kU/L) and peripheral blood eosinophil was significantly greater compared with the COS group (7.6% ± 4.6% vs. 2.5% ± 1.4%, P < .001). Histologically, eosinophil infiltration in ES group was observed around the main and interlobular ducts (50 ± 39/high power field [HPF]). Follicular hyperplasia (76%), epithelial mucous metaplasia (82%), and mucus plugs with eosinophils (41%) were observed. IgE‐positive cell count was 20.7 ± 18.3/HPF and tryptase‐positive mast cell count was 23.5 ± 15.1/HPF, which was significantly greater than the respective cell counts in COS group, which mainly infiltrated around the ducts. The levels of interleukin‐4, interleukin‐13, and eotaxin in tissue were significantly greater in ES than the COS group. Conclusions The clinicopathological characteristics of ES are significantly different from COS and ES might have an allergy‐related pathogenesis. Level of Evidence 4 Laryngoscope, 131:E800–E806, 2021

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“…Diagnosis is difficult and may be confused with other clinical entities such as Sjögren syndrome or infectious sialadenitis. [2] Although diagnostic criteria has not been defined, the typical findings of described cases are: (a) recurring episodes of parotid/submandibular gland swelling, (b) discharge of mucofibrinous plugs with a high content of eosinophils, (c) elevated levels of IgE and/or eosinophils in blood, (d) presence of concomitant allergic processes such as asthma or allergic rhinitis, (e) irregular dilation of the main salivary ducts as seen by imaging studies (sialography or MRI), (f) glandular biopsy showing lymphocytic infiltrates and abundant eosinophils within the interstium surrounding the salivary ducts. [3,4] The etiology of this disease remains unknown although the most accepted hypothesis is that it is an allergic process.…”

Section: Introductionmentioning

confidence: 99%

Sialodochitis fibrinosa (kussmaul disease) report of 3 cases and literature review

Robles¹,

Álvarez

Sanchinel

et al. 2016

Medicine

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Background:Sialodochitis fibrinosa is a rare disease which is characterized by recurring episodes of pain and swelling of the salivary glands due to the formation of mucofibrinous plugs. Analytic studies ascertain elevated levels of eosinophils and immunoglobulin E (IgE). Imaging studies such as magnetic resonance imaging (MRI) and sialography reveal dilation of the main salivary duct (duct ectasia). Treatment is initially supportive, consisting of compressive massages, and use of antihistamines and/or corticosteroids.Material and methods:In the following, 3 cases of sialodochitis fibrinosa are presented which were diagnosed in a third level hospital during the period of 2008 and 2016, as well as a literature review of all cases reported to our knowledge.Results:Of the 41 cases found, including the 3 of this article, 66% were women with an average age of 45 years old. However, 75% of reported cases were of Japanese heritage. Involvement of the parotid glands was more frequent than the submandibular glands. In more than half of all cases treatment with compressive massages, antihistamines and/or corticosteroids was effective.Conclusion:Clinicians should consider sialodochitis fibrinosa as a diagnostic possibility when presented with cases of recurring parotid and submandibular gland tumescence.

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A Rare Case of Kussmaul Disease (Sialodochitis Fibrinosa)

Cited by 9 publications

References 4 publications

Eosinophilic sialodochitis: redefinition of ‘allergic parotitis’ and ‘sialodochitis fibrinosa’

Eosinophilic sialodochitis: redefinition of ‘allergic parotitis’ and ‘sialodochitis fibrinosa’

Eosinophilic Sialodochitis: A Type of Chronic Obstructive Sialadenitis Related to Allergy

Sialodochitis fibrinosa (kussmaul disease) report of 3 cases and literature review

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