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Autoimmune Liver Disorders and Small-Vessel Vasculitis: Four Case Reports and Review of the Literature

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Autoimmune Liver Disorders and Small-Vessel Vasculitis: Four Case Reports and Review of the Literature CASE REPORT January-February, Vol. 13 No. 1, 2014: 136-141 Autoimmune liver disorders and small-vessel vasculitis: four case reports and review of the literature Francesco Tovoli,* Antonio Vannini,** Marco Fusconi,*** Magda Frisoni,*** Daniela Zauli* * University of Bologna, Department of Medical and Surgical Sciences. Bologna, Italy. ** Azienda Ospedaliero-Universitaria S.Orsola-Malpighi Bologna, Department of Emergency. Bologna, Italy. *** Azienda Ospedaliero-Universitaria S.Orsola-Malpighi Bologna, Department of Internal Medicine and Digestive Diseases. Bologna, Italy. ABSTRACT Autoimmune liver diseases (AILD) are a group of immunologically induced hepatic disorders that can lead to liver cirrhosis and end-stage liver disease. Extra-hepatic involvement and association with rheumatic dis- eases (such as Sjögren’s syndrome, systemic sclerosis and rheumatoid arthritis) are well known, whereas the coexistence of AILD with small-vessel vasculitis in the same patients have been only occasionally repor- ted. In the present paper we report four such cases and an extensive review of the literature. Clinical features of autoimmune-liver diseases associated with small-vessel vasculitis are discussed, as well as possi- ble common pathogenic pathways including HLA genomics, costimulatory molecules and autoantibodies. In conclusion, knowledge about this association can help physicians in recognising and treating an aggressive disease which could otherwise result in severe and multiple organ damage, compromising the overall prog- nosis and the indication to liver transplantation. Key words. Primary biliary cirrhosis. Autoimmune hepatitis. Anti-neutrophil cytoplasmic antibodies. Systemic vasculitis. INTRODUCTION CASE REPORT Autoimmune liver disorders (AILD) are a group Case 1 of immunologically induced hepatic disorders includ- ing autoimmune hepatitis (AIH), primary biliary A 75-year-old woman was admitted to the hospital cirrhosis (PBC) and primary sclerosing cholangitis after a bilateral mastectomy for tumor-like lesions. (PSC).1 Though hepatobiliary involvement repre- The histopathological examination of the surgical sents the main feature of AILD, association with sample had shown granulomatous lesions with focal rheumatic diseases such as Sjögren’s syndrome, sys- components of granulocyte-dominated vasculitis and temic sclerosis, rheumatoid arthritis has been fre- microabscesses. Her past medical history included quently reported.2-3 chronic sinusitis, nasal polyps and dacryocystitis. On the contrary, the association of small-vessel The patient complained of difficulty in nasal vasculitis and AILD has been seldom reported. breathing due to crusty secretions, xerostomia and We report four cases of this rare association. We xerophthalmia. The general physical examination also report an extensive review of the literature was unremarkable. about the topic. Laboratory investigation showed mild eosinophilia (908/µL) and moderately elevated alkaline phospha- tase (ALP - 1.4x) and gamma-glutaryl transferase (γ-GT - 2.5x), increased IgM (410 mg/dL) and slight- ly elevated total cholesterol (241 mg/dL). Kidney Correspondence and reprint request: Francesco Tovoli function tests, urine analysis and inflammatory Department of Medical and Surgical Sciences, University of Bologna. markers were normal. The sputum culture was posi- Via Massarenti 9, 40138 Bologna, Italy. tive for Staph aureus. E-mail: [email protected] Anti-mitochondrial (AMA) and anti-SS-A autoan- Manuscript received: January 23, 2013. tibodies were present, while antinuclear antibodies Manuscript accepted: March 15, 2013. (ANA) and cryoglobulins were absent. Antineutro- 137 Autoimmune liver disorders and vasculitis. , 2014; 13 (1): 136-141 phil cytoplasmic antibodies (ANCA) tests, performed cal history was positive for type 1 AIH (diagnosed at both by indirect immunofluorescence (IIF) on etha- the age of 16), chronic sinusitis, non-atopic asthma nol-fixed neutrophils and ELISA assay for antibo- (skin prick tests negative), hypereosinophilia. dies to myeloperoxidase (MPO) and proteinase-3 Diagnosis of AIH had been made on the basis of (PR3) were negative as well. marked elevation of aminotransferases, hypergam- Ultrasound examination of the liver showed no maglobulinemia, positivity for ANA and anti-smooth biliary obstruction. muscle antibodies (SMA), liver biopsy showing A percutaneous needle liver biopsy showed a mode- piecemeal necrosis with plasmacellular infiltrate. rately active chronic hepatitis with intralobular and He was being treated with azathioprine (50 mg/day) portal granulomas, consistent with PBC (stage 3). and low-dose methylprednisolone (4 mg/day). Nasal endoscopy revealed the presence of crusty On admission he was febrile; abdominal tender- lesions and the biopsy showed lesions similar to the ness was present in the epigastric and right hypo- previous post-mastectomy breast tissue biopsy sug- chondriac regions. Murphy’s sign was positive. gestive of granulomatosis with polyangiitis (GPA, He had hypereosinophilia (1,518/µL) with normal formerly known as Wegener granulomatosis). levels of total serum IgE. Aspartate aminotransfe- High resolution computed tomography (HRCT) of rase (AST), alanine aminotrasferase (ALT) and cho- the chest showed several nodules located around the lestatic liver enzymes were elevated (AST 1.2x, ALT bronchial vessels and in the subpleural space in both 1.5x, gGT 3.4x, ALP 1.3x). C-reactive protein (CRP) lung fields, which were suggestive of vasculitic lesions. levels were also increased (5x). There were also some small pulmonary nodules ANA and SMA were positive at low titre. ANCA located in the upper lung fields due to post-TB scar- tests, performed both by IIF on ethanol-fixed neu- ring processes. The Mantoux test was positive but no trophils and ELISA assay for antibodies to MPO acid fast bacilli were found in the sputum culture. and PR3, were negative. The salivary gland scintigram, the Schirmer test Ultrasound examination of abdomen revealed and the Break Up Time test were all compatible with thickening of the gallbladder wall (6 mm) without Sicca syndrome.4 evidence of gallstones. Absence of renal involvement and inflammation Following the diagnosis of acute cholecystitis the markers on one hand and coexisting pathologies on patient was proposed for cholecystectomy and an the other (severe osteoporosis and post-TB scarring open liver biopsy to evaluate the progression of AIH. processes) suggested a non-aggressive therapeutic ma- Histopathological examination of the gallbladder nagement of the patient. The usual therapeutic regi- wall revealed a marked inflammatory cell infiltrate men consisting in the combination of cyclophospamide (mainly eosinophils). No gallstone were found in the with methylprednisolone was therefore excluded. Ins- gallbladder. tead, we prescribed antibiotics. This decision was ba- Liver biopsy specimen showed chronic hepatitis sed on clinical evidence that has shown that nasal of minimal activity with mild portal fibrosis and colonization with Staph aureus is an independent risk eosinophilic portal vasculitis, the latter being factor for relapse of GPA. Furthermore, prophylactic absent in the previous biopsy performed 11 years treatment with cotrimoxazole can reduce the incidence before. of disease relapses and has proven effective for the in- Our patient was diagnosed as suffering from duction of remission in early or limited forms of GPA. Churg-Strauss syndrome (CSS) and he was treated Consistently with these observations, after two with azathioprine (50 mg/day) and high-dose me- weeks of antibiotic treatment (cotrimoxazole 800 thylprednisolone (40 mg/day). A month after his dis- mg/160 mg one tablet twice daily) our patient was charge he was well and without symptoms of asymptomatic. A HRCT of the chest performed two asthma, his eosinophil count was normal. months later showed complete resolution of the vas- culitic lesions. Case 3 Currently, three years after the diagnosis of GPA, no vasculitic relapses have occurred. A 72-year-old man was admitted to the hospital to investigate a previous diagnosis of ANCA-positive Case 2 vasculitis established two months earlier on the ba- sis of fever, livedo reticularis, Raynaud phenome- A 27-year-old man was admitted to the hospital non, positivity for ANCA (ELISA was positive for complaining of upper abdominal pain. His past medi- anti lactoferrin detected with a non-specified kit in a 138 Tovoli F, et al. , 2014; 13 (1): 136-141 different laboratory) and a dramatic response to ste- Unit was necessary. Despite aggressive immunosup- roid treatment. pressive therapy (methylprednisolone 1 g/day for 9 On admission he was asymptomatic and his phy- days) and invasive ventilation the patient died two sical examination was unremarkable. weeks later for respiratory failure. Inflammatory indexes were elevated – erythrocyte Post-mortem examination revealed diffuse pulmo- sedimentation rate (ESR) 76 mm/h, CRP 3x - and la- nary consolidation due to inhalation pneumonia as boratory tests were compatible with cholestasis the cause of death and confirmed the diagnosis of (γGT 6x, ALP 1.2x). ALT, AST and bilirubin were PBC and MPA. normal. His medication at the time included predni- sone 5 mg/day. Case 4 Autoantibody profile showed medium titre positi- vity for ANA with a multiple nuclear dots pattern A 36-year-old woman came to our attention as (MND); AMA were negative. outpatient in order to investigate two episodes of an- We performed an immunoblot assay to
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