Severe autoimmune neutropenia associated with acute autoimmune hepatitis

Wehbe, Ahmad Mouhamad; Johannsson, Birgir; Raife, Thomas J.; Bleile, Michelle; Bell, Adam M.; Curtis, Brian R.; Halfdanarson, Thorvardur R.

doi:10.1007/s12185-010-0557-1

Cited by 5 publications

(7 citation statements)

References 26 publications

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“…Our case represents a female patient with a flare of AIH-1 on a backdrop of possible chronic unresolved liver inflammation, complicated by severe AIN. In concordance with the existing literature, she was also positive for atypical pANCA [ 1 - 3 ]. The autoimmune origin of her secondary agranulocytosis can be advocated by the absence of alternative causes, the association with the AIH flare and the sustained response to treatment with corticosteroids and MMF [ 3 , 9 ].…”

Section: Discussionsupporting

confidence: 89%

“…AIN associated with autoimmune liver diseases at the time of diagnosis is very rare. We located only three cases of AIN in the context of AIH [ 1 - 3 ] with positive atypical pANCA. Our case represents a female patient with a flare of AIH-1 on a backdrop of possible chronic unresolved liver inflammation, complicated by severe AIN.…”

Section: Discussionmentioning

confidence: 99%

“…The autoimmune origin of her secondary agranulocytosis can be advocated by the absence of alternative causes, the association with the AIH flare and the sustained response to treatment with corticosteroids and MMF [ 3 , 9 ]. It is not yet clear whether the detection of atypical pANCA plays an active pathogenic role in the development of AIN, or whether their presence is a signifier of autoimmune activity [ 3 , 7 , 9 ].…”

Section: Discussionmentioning

confidence: 99%

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" Type 1 autoimmune hepatitis presenting with severe autoimmune neutropenia"

Doumtsis

Oikonomou

Goulis

et al. 2017

aog

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Autoimmune hepatitis (AIH) is a progressive, chronic liver disease characterized by unresolving hepatocellular inflammation of autoimmune origin. The clinical spectrum may vary from asymptomatic presentation, to non-specific symptoms such as fatigue, arthralgias, nausea and abdominal pain, to acute severe liver disease. AIH is characterized by the presence of interface hepatitis and portal plasma cell infiltration on histological examination, hypergammaglobulinemia, and positive autoantibodies. AIH is associated with other autoimmune diseases and its course is often accompanied by various non-specific hematological disorders. However, the coexistence of autoimmune neutropenia (AIN) is infrequent. We present a case of a female patient diagnosed with type 1 AIH and agranulocytosis on presentation. A diagnosis of AIN was established, based on the patient’s sex, the underlying liver disease, the absence of alternative causes, the presence of atypical anti-neutrophil cytoplasmic antibodies in patient’s serum and the favorable and dose-dependent treatment of both pathologic entities with corticosteroids and mycophenolate mofetil.

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Section: Discussionsupporting

confidence: 89%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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" Type 1 autoimmune hepatitis presenting with severe autoimmune neutropenia"

Doumtsis

Oikonomou

Goulis

et al. 2017

aog

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“…Evidence supporting one or more of these hypotheses was present in the current case and many of the previously reported cases. Maturation arrest of myeloid precursors was seen on bone marrow biopsy in the present case and several other cases (12, 24–25, 27–28) suggesting possible cellular or immune-mediated central marrow suppression. Cases in which splenomegaly was found suggest the potential for neutrophil sequestration (9, 20–22, 27, 36).…”

Section: Discussionsupporting

confidence: 65%

“…Autoimmune liver diseases, including autoimmune hepatitis and primary sclerosis cholangitis, have been associated with non-specific ANCA, presence of antineutrophil membrane antibodies, and severe neutropenia in the absence of vasculitis (24–26). ANCA and severe neutropenia has also been described in three cases of Sjögren's syndrome, and in one case, cutaneous vasculitis and antineutrophil membrane antibodies were reported (27–28).…”

Section: Literature Reviewmentioning

confidence: 99%

Antineutrophil Cytoplasmic Antibodies, Autoimmune Neutropenia, and Vasculitis

Grayson

Sloan

Niles

et al. 2011

Seminars in Arthritis and Rheumatism

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Objectives Reports of an association between antineutrophil cytoplasmic antibodies (ANCA) and autoimmune neutropenia have rarely included cases of proven vasculitis. A case of ANCA-associated vasculitis (AAV) with recurrent neutropenia is described and relevant literature on the association between ANCA, neutropenia, and vasculitis is reviewed. Methods Longitudinal clinical assessments and laboratory findings are described in a patient with AAV and recurrent episodes of profound neutropenia from December 2008 – October 2010. A PubMed database search of the medical literature was performed for papers published from 1960 through October 2010 to identify all reported cases of ANCA and neutropenia. Results A 49 year-old man developed recurrent neutropenia, periodic fevers, arthritis, biopsy-proven cutaneous vasculitis, sensorineural hearing loss, epididymitis, and positive tests for ANCA with specificity for antibodies to both proteinase 3 and myeloperoxidase. Antineutrophil membrane antibodies were detected during an acute neutropenic phase and were not detectable in a post-recovery sample, whereas ANCA titers did not seem to correlate with neutropenia. An association between ANCA and neutropenia has been reported in 74 cases from 24 studies in the context of drug/toxin exposure, underlying autoimmune disease, or chronic neutropenia without underlying autoimmune disease. In these cases, the presence of atypical ANCA patterns and other antibodies were common; however, vasculitis was uncommon and when it occurred was usually limited to the skin and in cases of underlying toxin exposure. Conclusions ANCA is associated with autoimmune neutropenia, but systemic vasculitis rarely occurs in association with ANCA and neutropenia. The interaction between neutrophils and ANCA may provide insight into understanding both autoimmune neutropenia and AAV.

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Acute and Acute Severe (Fulminant) Autoimmune Hepatitis

Czaja

2012

Dig Dis Sci

122

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Autoimmune hepatitis frequently has an abrupt onset of symptoms, and it can present with acute liver failure. The abrupt presentation can indicate spontaneous exacerbation of a pre-existent chronic disease, newly created disease, a superimposed infectious or toxic injury, or new disease after viral infection, drug therapy, or liver transplantation. Deficiencies in the classical phenotype may include a low serum immunoglobulin G level and low or absent titers of the conventional autoantibodies. The original revised diagnostic scoring system of the International Autoimmune Hepatitis Group can guide the diagnostic evaluation, but low scores do not preclude the diagnosis. Liver tissue examination is valuable to exclude viral-related or drug-induced liver injury and support the diagnosis by demonstrating centrilobular necrosis (usually with interface hepatitis), lymphoplasmacytic infiltration, hepatocyte rosettes, and fibrosis. Conventional therapy with prednisone and azathioprine induces clinical and laboratory improvement in 68-75 % of patients with acute presentations, and high dose prednisone or prednisolone (preferred drug) is effective in 20-100 % of patients with acute severe (fulminant) presentations. Failure to improve or worsening of any clinical or laboratory feature within 2 weeks of treatment or worsening of a mathematical model of end-stage liver disease within 7 days justifies liver transplantation in acute liver failure. Liver transplantation for acute severe (fulminant) autoimmune hepatitis is as successful as liver transplantation for autoimmune hepatitis with a chronic presentation and other types of acute liver failure (patient survival >1 year, 80-94 %). Liver transplantation should not be delayed or superseded by protracted corticosteroid therapy or the empiric institution of nonstandard medications.

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Severe autoimmune neutropenia associated with acute autoimmune hepatitis

Cited by 5 publications

References 26 publications

" Type 1 autoimmune hepatitis presenting with severe autoimmune neutropenia"

" Type 1 autoimmune hepatitis presenting with severe autoimmune neutropenia"

Antineutrophil Cytoplasmic Antibodies, Autoimmune Neutropenia, and Vasculitis

Acute and Acute Severe (Fulminant) Autoimmune Hepatitis

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