Two diseases one remedy? Systemic amyloidosis secondary to hidradenitis suppurativa: Treatment with infliximab

Özer, İlkay; Karaçin, Cengiz; Adışen, Esra; Güz, Galip; Gürer, Mehmet Ali

doi:10.1111/dth.12445

Cited by 14 publications

(23 citation statements)

References 10 publications

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“…44 In several cases of amyloidosis accompanying HS, the efficacy of TNF-a blockade on kidney function was reported. 45,46 A limitation in our study was that we did not compare the MEFV variants in the complex HS group with those in the noncomplex HS group. Additional analysis of genes related to AIS, such as IL1RN, NLRP3 or NOD2, would be advantageous in revealing other possible genes associated with HS.…”

Section: Discussionmentioning

confidence: 99%

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Association of pyrin mutations and autoinflammation with complex phenotype hidradenitis suppurativa: a case–control study

Vural¹,

Gündoğdu²,

İli³

et al. 2019

Br J Dermatol

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Summary Background Hidradenitis suppurativa (HS) is a rare, debilitating neutrophilic dermatosis characterized by chronic inflammation of hair follicles. Many inflammatory conditions may accompany HS. Objectives To investigate the association of variants of the MEFV gene with a complex HS phenotype. Methods Firstly, we identified the clinical characteristics of 119 patients with HS with a complex phenotype (Hurley stage III disease and/or additional inflammatory symptoms). Then, we searched for MEFV variants among these patients. The odds ratios (ORs) for pathogenic MEFV mutations were calculated using data from these patients with HS and 191 healthy controls. Results The male/female ratio was higher, and the mean age of onset was earlier, in our complex HS group compared with patients with HS in general. Five of the patients with HS (4·2%) had a diagnosis of familial Mediterranean fever (FMF) with a standardized morbidity ratio of 45 [95% confidence interval (CI) 16·50–99·84, P < 0·001] when compared with the frequency of FMF in the general Turkish population. Of the patients with complex HS, 38% were positive for pathogenic variants of MEFV. The OR for carrying a pathogenic MEFV allele was 2·80 (95% CI 1·31–5·97, P < 0·001). Conclusions The frequency of MEFV mutations in the group of patients with complex HS was higher than that in healthy controls, suggesting that MEFV mutations may contribute to the pathogenesis of HS. Understanding the role of autoinflammation in HS is of fundamental importance for the development of novel therapies.

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Section: Discussionmentioning

confidence: 99%

“…Genetic analysis of the MEFV gene was provided in a report from Turkey revealing a heterozygous M680I mutation in one patient with HS, amyloidosis and renal failure . In several cases of amyloidosis accompanying HS, the efficacy of TNF‐α blockade on kidney function was reported …”

Section: Discussionmentioning

confidence: 99%

Association of pyrin mutations and autoinflammation with complex phenotype hidradenitis suppurativa: a case–control study

Vural¹,

Gündoğdu²,

İli³

et al. 2019

Br J Dermatol

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“…The clinical course of amyloidosis concluded in renal insufficiency in four patients (44.4%), 12–15 whereas five (56.6%) experienced a clinical improvement with infliximab, 16,17 adalimumab, 4 fludrocortisone 4 and other agents 10 . Table 4 summarizes the main characteristics of patients with a dual diagnosis of HS and amyloidosis.…”

Section: Resultsmentioning

confidence: 99%

“…These observations may account for the high efficacy attributed to adalimumab and, to a lesser extent, infliximab and anakinra in the management of HS 24-26 . Similarly, adalimumab and infliximab have led to favourable outcomes in patients with HS‐related AA amyloidosis 4,16,17 .…”

Section: Discussionmentioning

confidence: 97%

Amyloidosis in hidradenitis suppurativa: a cross‐sectional study and review of the literature

et al. 2020

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Summary Background Coexistence of hidradenitis suppurativa (HS) and amyloidosis has been anecdotally described, but the association between these conditions is yet to be firmly established. Aim To study the association between HS and amyloidosis using a large‐scale computerized database, and to perform a literature review to characterize all reported patients with coexistent HS and amyloidosis. Methods A cross‐sectional study was conducted comparing the prevalence of amyloidosis among patients with HS and age‐, sex‐ and ethnicity‐matched healthy controls (HCs). Additionally, a review of literature was performed to summarize all reported cases with a dual diagnosis of both conditions. Results In total, 4417 patients with HS and 22 085 controls were included in the study. The prevalence of amyloidosis was increased in patients with HS compared with the HC group [n = 7 (0.2%) vs. n = 2 (0.0%), respectively; OR = 17.5; 95% CI 3.6–84.4; P < 0.001]. In a multivariate analysis, HS was still associated with amyloidosis (OR = 11.2; 95% CI 1.3–94.5; P = 0.03). The literature review identified nine patients who developed amyloidosis during the course of HS, with 44.4% eventually having renal failure. ‬Favourable outcomes were reported in patients managed by tumour necrosis factor (TNF)‐α inhibitors. Conclusion This study establishes the association between HS and amyloidosis. Screening for amyloidosis may be considered in patients with HS with a relevant clinical picture, mainly proteinuria. TNF‐α inhibitors may be preferred in patients with a dual diagnosis of these conditions.

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“…Because of its chronic inflammatory condition, HS can be suggested as a possible cause of secondary systemic amyloid A amyloidosis. However, only a few cases of severe HS with complications of the colon and the kidney have been reported so far. As the present case was histologically proven to be HS complicated by secondary amyloidosis throughout the GI tract, early therapeutic intervention is necessary to prevent the complication, especially in severe HS.…”

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confidence: 99%

Secondary gastrointestinal amyloid A amyloidosis possibly caused by hidradenitis suppurativa

Kochi

Esaki

Kurahara

2018

Digestive Endoscopy

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A 54-year-old man was referred to our hospital (Matsuyama Red Cross Hospital) because of 2-year duration of extended multiple nodules with abscess and fistula with pus discharge in the gluteal and perianal regions (Fig. 1a). Laboratory data identified inflammatory reaction (white blood cells [WBC], 11 430/lL, and C-reactive protein, 4.5 mg/dL) and anemia (hemoglobin levels, 8.6 g/dL). Serum amyloid A protein was elevated (50.2 lg/mL). Ileocolonoscopy showed diffuse coarse mucosa with numerous diminutive nodules throughout the colorectum (Fig. 1b). Esophagogastroduodenoscopy (EGD) identified whitish coarse mucosa with spotted hemorrhage and reddish spots in the gastric antrum (Fig. 1c) and granular mucosa in the descending duodenum (Fig. 1d). Histological examination taken from the colon, ileum, stomach, and duodenum showed massive amyloid deposits, which were positive for anti-serum amyloid A (SAA) antibody staining, in the lamina propria and around the submucosal vessels (Fig. 2). We diagnosed the patient as having secondary amyloid A amyloidosis of the gastrointestinal (GI) tract caused by severe hidradenitis suppurativa (HS). His HS was resected surgically with reconstruction by split-thickness skin grafting from his back. SSA protein at 5 months after surgery decreased to 12.2 lg/mL. EGD and ileocolonoscopy at 3 years after surgery indicated indistinctive gastric, duodenal, and colorectal mucosa; however, amyloid deposits in the GI tract remained positive under bioptic examination.

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Two diseases one remedy? Systemic amyloidosis secondary to hidradenitis suppurativa: Treatment with infliximab

Cited by 14 publications

References 10 publications

Association of pyrin mutations and autoinflammation with complex phenotype hidradenitis suppurativa: a case–control study

Association of pyrin mutations and autoinflammation with complex phenotype hidradenitis suppurativa: a case–control study

Amyloidosis in hidradenitis suppurativa: a cross‐sectional study and review of the literature

Secondary gastrointestinal amyloid A amyloidosis possibly caused by hidradenitis suppurativa

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