Disappearance of multiple hyperechoic liver nodules in sporadic porphyria cutanea tarda after treatment with ledipasvir/sofosbuvir for hepatitis C

Takata, Kazuhide; Shakado, Satoshi; Sakamoto, Keiko; Fukuda, Hiromi; Yamauchi, Ryo; Fukuda, Sho; Kunimoto, Hideo; Umeda, Kaoru; Tanaka, Takashi; Yokoyama, Keiji; Morihara, Daisuke; Takeyama, Yoshifumi; Irie, Makoto; Sakisaka, Shotaro

doi:10.1007/s12328-017-0772-x

Cited by 5 publications

(8 citation statements)

References 22 publications

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“…Multiple hyperechoic nodules due to focal steatosis are important as diagnostic triggers for PCT and for differential disease from cancer. Many reports have shown that focal fatty changes can present as multiple hyperechoic nodules in the liver in patients with PCT 5,6,9–25 . However, the incidence of liver nodules in patients with PCT is unknown, and it is possible that some patients with PCT may not have been diagnosed.…”

Section: Discussionmentioning

confidence: 99%

“…PCT. 5,6,[9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25] However, the incidence of liver nodules in patients with PCT is unknown, and it is possible that some patients with PCT may not have been diagnosed. In all seven cases, urinary porphyrin levels were measured for the first time following the detection of multiple hyperechoic lesions, leading to the diagnosis of PCT.…”

Section: Casementioning

confidence: 99%

“…Twenty-five cases were selected from 18 previous reports for the analysis. 5,[9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25] Some of the previous reports indicated a diagnosis as chronic hepatic porphyria instead of PCT; however, on confirming the diagnostic criteria of each case, they were judged to have PCT. We reviewed these 32 cases, including seven of our cases, and have summarized their findings in Table 3.…”

Section: Casementioning

confidence: 99%

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Multiple focal fatty changes in the liver in patients with porphyria cutanea tarda: A case series and review of the literature

Takata

Fukunaga

Nishizawa

et al. 2021

J of Clinical Ultrasound

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Porphyria cutanea tarda (PCT) is commonly diagnosed in cases where multiple hyperechoic nodules are observed in the liver. Pathologically, these nodules associated with PCT are focal fatty deposits. We report here, seven cases of PCT with fatty changes over multiple foci in the liver. Furthermore, the characteristics of ultrasonography (US) findings of 32 previously reported cases are summarized. The US features of these nodules showed a homogenous hyperechoic or hyperechoic rim pattern, partial confluence, and no mass effect in the vascular structures. Because multiple hyperechoic liver nodules occasionally mimic malignancies, and because their diagnosis can be challenging, clinicians should consider checking urine porphyrin levels to rule out PCT when such nodules are observed on US.

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Section: Discussionmentioning

confidence: 99%

Section: Casementioning

confidence: 99%

Section: Casementioning

confidence: 99%

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Multiple focal fatty changes in the liver in patients with porphyria cutanea tarda: A case series and review of the literature

Takata

Fukunaga

Nishizawa

et al. 2021

J of Clinical Ultrasound

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“…The skin becomes very fragile, with superficial erosions covered by crust following minimal trauma. 43 …”

Section: Cutaneous Porphyriasmentioning

confidence: 99%

“…In hepatitis C virus (HCV)-positive patients, antiviral treatment may cure both the hepatitis and the PCT. 43 …”

Section: Cutaneous Porphyriasmentioning

confidence: 99%

Porphyria: What Is It and Who Should Be Evaluated?

Edel

Mamet

2018

Rambam Maimonides Med J

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The porphyrias are a group of rare metabolic disorders, inherited or acquired, along the heme biosynthetic pathway, which could manifest with neurovisceral and/or cutaneous symptoms, depending on the defective enzyme. Neurovisceral porphyrias are characterized by acute attacks, in which excessive heme production is induced following exposure to a trigger. An acute attack usually presents with severe abdominal pain, vomiting, and tachycardia. Other symptoms which could appear include hypertension, hyponatremia, peripheral neuropathy, and mild mental symptoms. In severe attacks there could be severe symptoms including seizures and psychosis. If untreated, the attack might become very severe, affecting the peripheral, central, and autonomic nervous system, leading to paralysis, respiratory failure, hyponatremia, coma, and even death. From the biochemical point of view, acute attacks are involved with increased levels of precursors in the heme biosynthetic pathway, up to the deficient step. Of these precursors, aminolevulinic acid (ALA) is considered to be neurotoxic. Treatment is directed to reduce ALA production by reducing the activity of the enzyme aminolevulinate synthase (ALAS)—most effectively by heme therapy. Cutaneous symptoms are a consequence of elevated porphyrins in the blood stream. These porphyrins react to light; therefore sun-exposed areas are affected, producing fragile erosive skin lesions in porphyria cutanea tarda (PCT) or non-scarring stinging and burning symptoms in erythropoietic protoporphyria (EPP). Unlike the most common neurovisceral porphyria, acute intermittent porphyria (AIP), variegate porphyria (VP), and hereditary coproporphyria (HCP) can have cutaneous symptoms as well. Differentiating them from other cutaneous porphyrias is essential for accurate diagnosis, treatment, and patient recommendations.

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Ledipasvir/Sofosbuvir Is Effective as Sole Treatment of Porphyria Cutanea Tarda with Chronic Hepatitis C

et al. 2023

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Background and Aims Chronic hepatitis C [CHC] is a risk factor for porphyria cutanea tarda [PCT]. To assess whether ledipasvir/sofosbuvir is effective for treating both PCT and CHC, we treated patients with CHC + PCT solely with ledipasvir/sofosbuvir and followed them for at least 1 year to assess cure of CHC and remission of PCT. Methods Between September 2017 and May 2020, 15 of 23 screened PCT + CHC patients were eligible and enrolled. All were treated with ledipasvir/sofosbuvir at recommended doses and durations, according to their stage of liver disease. We measured plasma and urinary porphyrins at baseline and monthly for the first 12 months and at 16, 20, and 24 mos. We measured serum HCV RNA at baseline, 8–12, and 20–24 mos. Cure of HCV was defined as no detectable serum HCV RNA ≥ 12 weeks after the end of treatment (EOT). Remission of PCT was defined clinically as no new blisters or bullae and biochemically as urinary uro- plus hepta-carboxyl porphyrins ≤ 100 mcg/g creatinine. Results All 15 patients, 13 of whom were men, were infected with HCV genotype 1. 2/15 withdrew or were lost to follow-up. Of the remaining 13, 12 achieved cure of CHC; 1 had complete virological response, followed by relapse of HCV after ledipasvir/sofosbuvir but was subsequently cured by treatment with sofosbuvir/velpatasvir. Of the 12 cured of CHC, all achieved sustained clinical remission of PCT. Conclusions Ledipasvir/sofosbuvir [and likely other direct-acting antivirals] is an effective treatment for HCV in the presence of PCT and leads to clinical remission of PCT without additional phlebotomy or low-dose hydroxychloroquine treatment. Trial Registration ClinicalTrials.gov NCT03118674. Supplementary Information The online version contains supplementary material available at 10.1007/s10620-023-07859-8.

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Disappearance of multiple hyperechoic liver nodules in sporadic porphyria cutanea tarda after treatment with ledipasvir/sofosbuvir for hepatitis C

Cited by 5 publications

References 22 publications

Multiple focal fatty changes in the liver in patients with porphyria cutanea tarda: A case series and review of the literature

Multiple focal fatty changes in the liver in patients with porphyria cutanea tarda: A case series and review of the literature

Porphyria: What Is It and Who Should Be Evaluated?

Ledipasvir/Sofosbuvir Is Effective as Sole Treatment of Porphyria Cutanea Tarda with Chronic Hepatitis C

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