Autoimmune hepatitis and agranulocytosis

Cuadrado, Abel Santamaría; Aresti, S.; Cortés, Montserrat; Gomez-Ortega, Jose M.; Salcines, J.R.

doi:10.1016/j.dld.2008.01.006

Cited by 6 publications

(7 citation statements)

References 6 publications

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“…AIN was suggested based on the temporal relationship with a flare of the hepatitis, presence of periplasmic-ANCA and response to corticosteroid treatment [23]. Our patient differs from the patient described by Cuadrado et al in several important ways.…”

Section: Discussioncontrasting

confidence: 52%

Severe autoimmune neutropenia associated with acute autoimmune hepatitis

et al. 2010

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A 49-year-old previously healthy female presented with acute hepatitis and severe neutropenia. A diagnosis of type 1 autoimmune hepatitis was made based on the histological appearance of a liver core biopsy, positive anti-smooth muscle antibodies, and positive anti-neutrophil cytoplasmic antibody (atypical ANCA). Hemogram revealed mild leukopenia with severe neutropenia (absolute neutrophil count 256/mm 3 ), normal hemoglobin and mild thrombocytopenia (115000/mm 3 ). A bone marrow biopsy and aspirate had a normal karyotype, increase in granulopoiesis, prominence of promyelocytes (31%) and absence of mature granulocytes. Anti-neutrophil antibodies were detected in the patient's blood. Therapy was directed at the underlying hepatitis with resolution of neutropenia without the use of colony-stimulating factors.

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Section: Discussioncontrasting

confidence: 52%

Severe autoimmune neutropenia associated with acute autoimmune hepatitis

et al. 2010

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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%

" 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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“…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%

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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Autoimmune hepatitis and agranulocytosis

Cited by 6 publications

References 6 publications

Severe autoimmune neutropenia associated with acute autoimmune hepatitis

Severe autoimmune neutropenia associated with acute autoimmune hepatitis

" Type 1 autoimmune hepatitis presenting with severe autoimmune neutropenia"

Antineutrophil Cytoplasmic Antibodies, Autoimmune Neutropenia, and Vasculitis

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