Association between autoimmune hepatitis and leukocytoclastic vasculitis, a case report

Introduction and Objectives

Autoimmune hepatitis has an incidence that ranges from 0.9-2%. It is usually associated with other liver diseases and other autoimmune disorders, however, there are few cases associated with leukocytoclastic vasculitis. Now we present the case of an association between autoimmune hepatitis and leukocytoclastic vasculitis.

Materials and Patients

This is a female patient who presented constant pain in the right hypochondrium since 2019, intermittent fever with nocturnal presentation.

In the Personal pathological history, she reported that was healthy, had an uncomplicated pregnancy, had no history of traveling outside the country or visiting caves, had no family history of autoimmune, genetic, or infectious diseases, had no history of exposure to chemical substances or people with a diagnosis of tuberculosis. During the years 2019 to 2024, in addition to pain in the hypochondrium and fever, they presented myalgia, arthralgia, morning stiffness that improves with activity with signs of inflammatory pain, facial erythema, and maculopapular skin rashes on the hands and legs on sun exposure. She presented Eye with foreign body sensation, and we referred to rheumatology, considering the possibility of systemic lupus erythematosus and Sjögren's syndrome, complementary studies were performed, and she was sent to ophthalmology where a normal tear breakup time of less than 5 seconds was concluded. and Sjögren's Syndrome is ruled out. Antibodies are performed to rule out systemic lupus erythematosus, such as Anti DNA and Anti SM, being negative.

Results

We performed a diagnostic approach in a patient with constitutional symptoms, skin rashes and constant pain in the right upper quadrant. Complementary imaging studies were requested such as USG of the liver and CT scan of the abdomen, both of which showed signs of cirrhosis, so autoimmune hepatitis began to be suspected. Within the liver studies, the transaminases are normal, alkaline phosphatase elevated, and hyperglobulinemia. Protein electrophoresis with immunofixation was performed, being positive for HyperGammaglobulinemia, subsequently, biopsies of the lip, liver and skin lesions were taken. Amylodosis and Sjögren's syndrome were ruled out with these studies, however the skin lesions demonstrated leukocytoclastic vasculitis and the antibody studies showed positive ANA, with AMA, ANCA, DNA, SM negative, and the liver biopsy showed findings related to autoimmune hepatitis. Therefore, by ruling out connective tissue and associated oncological diseases, the diagnosis of autoimmune hepatitis was established, since when using the simplified diagnostic criteria of the International Autoimmune Hepatitis Group, 8 points were met, thus confirming the diagnosis of this entity. Due to hypergammaglobulinemia, hematological diseases were ruled out when bone marrow aspiration and biopsy were performed.

Conclusions

We did the approach of cirrhosis of unknown origin. We proceeded to look out autoimmune liver diseases, connective tissue and oncological diseases all of that were ruled out. Reaching the diagnosis required the commitment of several specialties: internal medicine, rheumatology, hematology and pathological anatomy.

Ethical statement: The authors declare that the article is unique, it has not been previously published in any other media services and there are informed consents signed by the participants and the patient for their participation in the hepatology congress held by the AMH 2024.

Declaration of interests: None.

Funding: This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

Table 1.

 

Laboratories Studies	Result
HIV, HBV, HCV	NEGATIVE
ANTI SMOOTH MUSCLE ANTIBODY	NEGATIVE.
ANTI MITOCHONDRIAL ANTIBODY
ANCA P & ANCA C
ALPHA FETOPROTEIN	NEGATIVE.
ANTIBODY OF CARCINOEMBRYONIC
CA 19.9
CA 125
ANA	POSITIVE: 1:80, GRANULAR PATTERN.
ANTI RHO	POSITIVE, 27.1
ANTI LA	NEGATIVE.
BETA 2 MYCROGLOBULINE	1123
C3	187
C4	27
TSH Y T4-L	NORMAL.
IGA	916
IGM	223
IGG	3362
ANTI CCP	NEGATIVE
RHEUMATOID FACTOR	5.1
VSG, PCR.	NORMAL.

ANCA P & ANCA C, perinuclear & cytoplasmic anti-neutrophil cytoplasmic antibodies; ANA, antinuclear antibody; ANTI CCP, anti-cyclic citrullinated peptide antibody; ANTI LA, anti-La antibody; ANTI MITOCHONDRIAL ANTIBODY, anti-mitochondrial antibody; ANTI RHO, anti-Ro antibody; ANTI SMOOTH MUSCLE ANTIBODY, anti-smooth muscle antibody; ANTIBODY OF CARCINOEMBRYONIC, carcinoembryonic antigen; BETA 2 MYCROGLOBULINE, beta-2 microglobulin; CA 125, cancer antigen 125; CA 19.9, cancer antigen 19-9; C3, complement component 3; C4, complement component 4; FACTOR REUMATOIDE, rheumatoid factor; HBV, hepatitis B virus; HCV, hepatitis C virus; HIV, human immunodeficiency virus; IGA, immunoglobulin A; IGG, immunoglobulin G; IGM, immunoglobulin M; PCR, polymerase chain reaction; TSH Y T4-L, thyroid-stimulating hormone and free thyroxine; VSG, erythrocyte sedimentation rate.