Azathioprine Hypersensitivity Presenting as Cardiogenic Shock and Sweet's Syndrome in a Patient with Microscopic Polyangiitis

Turow, Arthur; Yong, Tuck Yean; Fok, Jie Shen; Li, Jordan

doi:10.2169/internalmedicine.51.6962

Cited by 15 publications

(9 citation statements)

References 19 publications

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“…Davis et al ., cited in [ 28 ], have suggested that imidazole moiety of AZT causes the allergic symptoms and the 6-mercaptopurine moiety the liver injury. Reintroduction of AZT should be avoided as this may lead to more serious reactions within hours of the re-challenge [ 30 ]. In summary, increased PCT may be induced by AZT-associated hypersensitivity and complicates the differential diagnosis of AAV flare versus infection.…”

Section: Discussionmentioning

confidence: 99%

Diagnostic Value of Procalcitonin in ANCA-Associated Vasculitis (AAV) to Differentiate Between Disease Activity, Infection and Drug Hypersensitivity

Herrmann¹,

Schinke²,

Csernok³

et al. 2015

TORJ

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Objective: Procalcitonin (PCT) is considered to be a specific marker for severe bacterial infections and sepsis. Elevated PCT levels have been reported in active autoimmune diseases without infection.The aim of this study was to assess the diagnostic value of PCT serum levels in ANCA-associated vasculitis (AAV) patients with respect to infection, disease activity and drug fever using a high sensitive PCT detection method.Methods: In 53 AAV patients with elevated C-reactive protein (CRP) PCT was determined by the Thermo Scientific BRAHMS PCT sensitive KRYPTOR assay. Patients underwent standardized diagnostic procedures for evaluation of disease activity and infection.Results: 53 patients with AAV and elevated CRP (7.7±6.9 mg/dl, PCT 0.34±1.02 ng/ml) were assessed, 10 had infection with elevated CRP levels of 11.2±10.2 mg/dl and PCT levels of 1.06±2.07 ng/dl. 43 patients had no evidence of infection, 36 of them were presented with AAV with normal or only slightly positive PCT levels in active disease (n=36) (PCT 0.06±0.06 ng/ml). 7 patients had increased PCT levels due to azathioprine hypersensitivity (0.76±1.01 ng/ml). For discrimination between infection and vasculitis activity PCT was more useful than CRP with the best cut-off at 0.1 ng/ml (sensitivity 60%, specificity 92%).Conclusion: In contrast to previous studies using semiquantitative PCT assays, the KRYPTOR performs better with respect to discrimination of infection from active AAV. In all patients assessed with active AAV (and without infection) PCT levels remained below the PCT reference limit (0.5 ng/ml) for infections. Drug hypersensitivity seems to be an important differential diagnosis in the setting of elevated CRP and PCT in patients who receive azathioprine.

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Section: Discussionmentioning

confidence: 99%

Diagnostic Value of Procalcitonin in ANCA-Associated Vasculitis (AAV) to Differentiate Between Disease Activity, Infection and Drug Hypersensitivity

Herrmann¹,

Schinke²,

Csernok³

et al. 2015

TORJ

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“…Azathioprine (AZA) still corresponds to the mainstay maintenance therapy in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (1,2). This drug is mostly well-tolerated, but numerous side effects are recognized.…”

Section: Introductionmentioning

confidence: 99%

“…This drug is mostly well-tolerated, but numerous side effects are recognized. These can be divided in two categories: dose-dependent toxic side effects and rarer dose-independent hypersensitivity reactions (1,3). The time lag to symptoms in hypersensitivity reactions is wide, ranging from 1 week to 16 months after starting therapy, but occurs more frequently in the first 4 weeks (1,4).…”

Section: Introductionmentioning

confidence: 99%

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Azathioprine-induced interstitial nephritis in an anti-neutrophil cytoplasmic antibody (ANCA) myeloperoxidase (MPO) vasculitis patient

et al. 2018

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Azathioprine (AZA) is used in a wide array of autoimmune diseases, still corresponding to the mainstay maintenance therapy in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides. Although generally well-tolerated, several side effects are recognized. We report the case of a 50-yearold Caucasian man with kidney-limited ANCA myeloperoxidase (MPO) vasculitis who presented with general malaise, fever, worsening renal function, and elevated inflammatory markers 2 weeks after the initiation of therapy with oral AZA. Although a disease relapse was suspected, renal biopsy revealed an eosinophilic infiltrate, suggestive of acute interstitial nephritis. After suspension of AZA, a sustained improvement of renal function and normalization of inflammatory markers was observed. A diagnosis of allergic interstitial nephritis secondary to AZA was established, corresponding to the first biopsy-proven case described in an ANCA MPO vasculitis patient. Although rare, renal toxicity of AZA must be present in the clinician´s mind, avoiding the straightforward assumption of disease relapse in the case of worsening renal function.

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“…1 Most adverse effects of azathioprine, such as myelosuppression and hepatotoxicity, 2 are dose-dependent and related to drug metabolism. Azathioprine-induced Sweet's syndrome (AISS), as described in our case series, is an idiosyncratic hypersensitivity reaction, representing only 2% of the adverse effects caused by azathioprine.…”

Section: Introductionmentioning

confidence: 99%

Azathioprine‐induced Sweet's syndrome: A case series and review of the literature

2015

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We present three patients with azathioprine-induced Sweet's syndrome (AISS) who attended our tertiary institution within a 12-month period. Established associations exist between Sweet's syndrome and some medications; however, to date links to azathioprine are tentative. While there are case reports of AISS, most have occurred in patients with inflammatory bowel disease (IBD), an underlying predisposition for Sweet's syndrome. Our case series adds to the evidence that the entity of AISS truly exists independent of confounding factors such as concurrent IBD.

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Azathioprine Hypersensitivity Presenting as Cardiogenic Shock and Sweet's Syndrome in a Patient with Microscopic Polyangiitis

Cited by 15 publications

References 19 publications

Diagnostic Value of Procalcitonin in ANCA-Associated Vasculitis (AAV) to Differentiate Between Disease Activity, Infection and Drug Hypersensitivity

Diagnostic Value of Procalcitonin in ANCA-Associated Vasculitis (AAV) to Differentiate Between Disease Activity, Infection and Drug Hypersensitivity

Azathioprine-induced interstitial nephritis in an anti-neutrophil cytoplasmic antibody (ANCA) myeloperoxidase (MPO) vasculitis patient

Azathioprine‐induced Sweet's syndrome: A case series and review of the literature

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