PASS Syndrome: An IL-1-Driven Autoinflammatory Disease

Leuenberger, Mathieu; Berner, Jerome J.; J, Di Lucca; Fischer, Louise Arup; Kaparos, N; Conrad, Curdin; Hohl, Daniel; A, So; Gilliet, Michel

doi:10.1159/000443648

Cited by 51 publications

(66 citation statements)

References 14 publications

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“…PASS syndrome combining PG, acne, HS, and seronegative spondyloarthritis was recently added to the spectrum of autoinflammatory syndromes (Table ). PASS syndrome is distinct from PAPA (pyogenic arthritis, acne, and PG) because it involves non‐pyogenic affection of axial joints, and from PASH syndrome, which has no joint involvement.…”

Section: Passmentioning

confidence: 99%

“…PASS syndrome is distinct from PAPA (pyogenic arthritis, acne, and PG) because it involves non‐pyogenic affection of axial joints, and from PASH syndrome, which has no joint involvement. Only two patients with PASS syndrome have been reported in literature so far …”

Section: Passmentioning

confidence: 99%

“…Autoinflammatory diseases are defined by an aberrant innate immune system and the absence of circulating autoantibodies and autoreactive T‐cells . A common pathogenic mechanism involving interleukin (IL)‐1‐driven recurrent episodes of neutrophil‐predominated sterile inflammation are believed to underlie these rare syndromes . Disease symptoms are often severe and can be unresponsive to standard regimens.…”

Section: Introductionmentioning

confidence: 99%

“…If not managed sufficiently, these syndromes can have devastating physical and psychological consequences for the affected patients . Thus, it is highly important for dermatologists to be familiar with symptoms of autoinflammatory syndromic HS and/or acne, including recurrent episodes of fever, severe joint pain, skin lesions typical of PG, HS, and acne, and laboratory findings suggestive of systemic inflammation with leukocytosis and increased acute phase reactants like erythrocyte sedimentation rate (ESR) and C‐reactive protein (CRP) . This review describes the clinical features, underlying pathophysiology, and the current treatment modalities of autoinflammatory syndromes associated with HS and acne based on existing literature.…”

Section: Introductionmentioning

confidence: 99%

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Autoinflammatory syndromes associated with hidradenitis suppurativa and/or acne

2017

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Autoinflammatory syndromes associated with hidradenitis suppurativa (HS) and/or acne are rare but potentially debilitating disorders if not diagnosed and treated correctly. They share a common pathogenesis involving a dysregulated innate immune system with abnormal interleukin (IL)-1 signaling leading to sterile neutrophilic inflammation. The clinical features are recurrent episodes of fever, painful arthritis, and skin lesions consistent with HS, acne, and pyoderma gangrenosum (PG) accompanied by elevated systemic inflammatory markers in blood. So far, several clinically different syndromes have been reported in the literature including pyoderma gangrenosum, acne, and pyogenic arthritis (PAPA), pyoderma gangrenosum, acne, and hidradenitis suppurativa (PASH), pyoderma gangrenosum, acne, and spondyloarthritis (PASS), pyoderma gangrenosum, acne, pyogenic arthritis, and hidradenitis suppurativa (PAPASH), psoriatic arthritis, pyoderma gangrenosum, acne, and hidradenitis suppurativa (PsAPASH), and pyoderma gangrenosum, acne, and ulcerative colitis (PAC). The rarity of the syndromes complicates the establishment of evidence-based treatment guidelines. Furthermore, treatment can be challenging due to lack of response to standard treatment modalities. Therefore, it is important to increase the awareness about these diseases in order to optimize disease management and ultimately improve the quality of life of patients.

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Section: Passmentioning

confidence: 99%

Section: Passmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Autoinflammatory syndromes associated with hidradenitis suppurativa and/or acne

2017

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“…They include PASS (pyoderma gangrenosum, acne, suppurative hidradenitis, and axial spondyloarthritis), PAPASH (pyogenic arthritis, pyoderma gangrenosum, acne, and HS) and PsAPASH (psoriatic arthritis, pyoderma gangrenosum, acne, and HS) (Bruzzese, 2012; Marzano et al, 2013; Saraceno et al, 2015). Recently, a PASS patient treated with 100 mg/day Anakinra and improvement of all symptoms was reported, after Anakinra discontinuation relapse occurred within 3 days (Leuenberger et al, 2016). …”

Section: Skin Diseases With Il-1 Involvementmentioning

confidence: 99%

Potential of IL-1, IL-18 and Inflammasome Inhibition for the Treatment of Inflammatory Skin Diseases

2017

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In 2002, intracellular protein complexes known as the inflammasomes were discovered and were shown to have a crucial role in the sensing of intracellular pathogen- and danger-associated molecular patterns (PAMPs and DAMPs). Activation of the inflammasomes results in the processing and subsequent secretion of the pro-inflammatory cytokines IL-1β and IL-18. Several autoinflammatory disorders such as cryopyrin-associated periodic syndromes and Familial Mediterranean Fever have been associated with mutations of genes encoding inflammasome components. Moreover, the importance of IL-1 has been reported for an increasing number of autoinflammatory skin diseases including but not limited to deficiency of IL-1 receptor antagonist, mevalonate kinase deficiency and PAPA syndrome. Recent findings have revealed that excessive IL-1 release induced by harmful stimuli likely contributes to the pathogenesis of common dermatological diseases such as acne vulgaris or seborrheic dermatitis. A key pathogenic feature of these diseases is IL-1β-induced neutrophil recruitment to the skin. IL-1β blockade may therefore represent a promising therapeutic approach. Several case reports and clinical trials have demonstrated the efficacy of IL-1 inhibition in the treatment of these skin disorders. Next to the recombinant IL-1 receptor antagonist (IL-1Ra) Anakinra and the soluble decoy Rilonacept, the anti-IL-1α monoclonal antibody MABp1 and anti-IL-1β Canakinumab but also Gevokizumab, LY2189102 and P2D7KK, offer valid alternatives to target IL-1. Although less thoroughly investigated, an involvement of IL-18 in the development of cutaneous inflammatory disorders is also suspected. The present review describes the role of IL-1 in diseases with skin involvement and gives an overview of the relevant studies discussing the therapeutic potential of modulating the secretion and activity of IL-1 and IL-18 in such diseases.

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Comorbidities and Complex Syndromes

Fabbrocini

Vita

2017

Hidradenitis Suppurativa

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PASS Syndrome: An IL-1-Driven Autoinflammatory Disease

Cited by 51 publications

References 14 publications

Autoinflammatory syndromes associated with hidradenitis suppurativa and/or acne

Autoinflammatory syndromes associated with hidradenitis suppurativa and/or acne

Potential of IL-1, IL-18 and Inflammasome Inhibition for the Treatment of Inflammatory Skin Diseases

Comorbidities and Complex Syndromes

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