Liver involvement in sarcoidosis: a case report

Abstracts

A sarcoidose representa uma doença inflamatória cuja etiologia permanece desconhecida. Acomete principalmente o interstício pulmonar, com formação de granulomas não caseosos e adenomegalia. Trata-se de enfermidade sistêmica, portanto, pode atingir qualquer órgão ou sistema, devendo ser lembrada no diagnóstico diferencial das doenças infecciosas, neoplásicas ou auto-imunes, isto porque, se diagnosticada corretamente, evita medicações ou cirurgias desnecessárias. Os autores relatam um caso de sarcoidose pulmonar com envolvimento hepático, no qual o paciente desenvolveu cirrose e trombose de veia porta.

Fígado; Sarcoidose; Ultra-sonografia

CASE REPORT

Liver involvement in sarcoidosis: a case report^*

André Albernaz^I; Ana Cláudia Ferreira Rosa^II; Márcio Martins Machado^III; Nestor de Barros^IV; Orlando Milhomem da Mota^V; Jales Benevides Santana Filho^VI; Rodrigo Alvarenga Nunes^VII; Huber Martins Vasconcelos Júnior^VIII; Patrícia Tavares Pereira de Souza^VIII; Thaís de Toledo Lima^VIII; Leonardo Medeiros Milhomem^IX; Carlos Alberto Ximenes Filho^II; Carlos Alberto Ximenes^X; Giovanni Guido Cerri^XI

^IMD-Resident, Department of Radiology of Faculty of Medicine, Universidade Federal de Goiás

^IIMD-Radiologists at the Faculty of Medicine, Universidade Federal de Goiás Clinics Hospital

^IIIInvited Professor, Department of Radiology at Faculty of Medicine, Universidade Federal de Goiás

^IVDoctor Professor, Department of Radiology at Faculty of Medicine, Universidade de São Paulo

^VHead of the Department of Digestive Tract Diseases Hospital Araújo Jorge of the Associação de Combate ao Câncer em Goiás

^VISurgeon at the Department of Digestive Diseases, Hospital Araújo Jorge of the Associação de Combate ao Câncer em Goiás

^VIIAcademic of Medicine, Faculty of Medical Sciences, Universidade do Vale do Sapucaí

^VIIIAcademics of Medicine, Faculty of Medicine, Universidade Federal de Goiás

^IXAcademic of Medicine, Faculty of Medicine, Universidade de Ribeirão Preto

^XHead of the Department of Radiology, Faculty of Medicine, Universidade Federal de Goiás

^XITitular Professor at Department of Radiology, Faculty of Medicine, Universidade de São Paulo, Director of the Diagnosis Center of the Hospital Sírio Libanês

^{Mailing address}

ABSTRACT

Sarcoidosis is an inflammatory disease whose etiology remains unknown. It is characterized by the formation of non-caseating granulomas and adenopathy, mainly in the pulmonary interstice. This is a systemic disease that may affect any organ or system and should be included in the differential diagnosis of infectious, neoplastic or autoimmune diseases as a correct diagnosis avoids unnecessary surgeries or administration of drugs. The authors report a case of pulmonary sarcoidosis with hepatic involvement in which the patient has developed cirrhosis and portal vein thrombosis.

Keywords: Liver; Sarcoidosis; Ultrasonography.

INTRODUCTION

Sarcoidosis is a multisystem inflammatory disease of unknown etiology and is characterized by the formation of non-caseating granulomas. Sarcoidosis has a higher prevalence between the third and fifth decades of life, predominating in blacks and with low prevalence in the South America compared to other regions. The lung is the organ most commonly affected, but the abdominal occurrence without evidence of pulmonary alterations at chest x-ray may be found in about 25%–38% of the cases⁽¹⁾.

In this paper, the authors report a case of sarcoidosis with pulmonary involvement associated with liver and spleen alterations.

CASE REPORT

A 47 year old, patient, with a recent diagnosis of pulmonary sarcoidosis, has developed upper digestive hemorrhage. Upper digestive endoscopy has revealed large caliber varicose cords with signs of recent bleeding.

Ultrasound (US) detected the heterogeneous aspect of the liver with rhomb edges and irregular surface, small relative decrease of volume in the right hepatic lobe when compared to the left one. At the Doppler study, portal vein thrombosis was identified (Figure 1). Additionally, the spleen was increased with homogeneous echotexture (Figure 2).

Hepatitis B and C serologies were made with negative results and the patient didn't present an alcoholism history. Hepatic biopsy revealed cirrhosis associated with non-caseating granulomas.

DISCUSSION

In the abdomen, the sarcoidosis may mimic different infectious, neoplastic or autoimmune diseases; presenting itself as asymptomatic or with non-specific symptoms (fever, fatigue, ponderal loss and diarrhea)⁽¹⁾.

The liver and spleen are the most affected organs (40%–60%), with or without granulomas formation⁽¹⁾. The involvement of the periportal regions is more characteristic than the hepatic compromising itself⁽²⁾. As a result, there is a possibility of portal hypertension development. Occasionally, portal vein thrombosis with signs of cavernomatous transformation may be found⁽²⁾. Even so, the portal hypertension is not usual⁽³⁾. In the initial phases of the hepatic involvement in sarcoidosis, the patients can be succesfully treated with corticosteroids⁽¹⁾. However, without an effective treatment, a periportal fibrosis may develop with irreversible portal hypertension^(2,3). As the disease progresses, the process may reach the hepatic parenchyma and lead to the development of cirrhosis^(1,3). In the present case, the patient has developed portal hypertension probably secondary to the chronic hepatopathy (cirrhosis), as demonstrated by the hepatic biopsy, also being affected by portal vein thrombosis, presenting signs of cavernomatous transformation.

Radiologically, the most common finding is hepatomegaly (29%) with a homogeneous or heterogeneous liver. Focal nodules may be observed in up to 19% of cases⁽¹⁾. These, in its turn, most of time are followed by hepatomegaly, adenopathy and splenic nodules⁽¹⁾. The use of corticoids can make the nodules disappear⁽²⁾, but the hepatomegaly remains⁽¹⁾. In the patient studied, hepatomegaly, hepatic or splenic nodules, or lymphadenomegaly were not ultrasonographic findings.

Although the liver may be normal at the ultrasound in the cases sarcoidosis with hepatic involvement, diffuse textural alterations also have been described in these cases. As previously mentioned, the granulomas usually are too small to be ultrasonographically identified^(2,3). Additionally, at ultrasonography, we could observe an increase in the hepatic echogenicity⁽³⁾. The nodules, when present, usually appear as hypoechoic in relation to the parenchyma, although hyperechoid cases have been described⁽¹⁾. In computed tomography (CT) after endovenous contrast administration, the nodules were seen as hypoattenuating with hypocaptation. The magnetic resonance imaging (MRI) shows hypointenses lesions in all the precontrast sequences, more conspicuous in early post-gadolinium T1 weighted images⁽¹⁾.

The splenomegaly is found in 25% to 60% of the patients with abdominal sarcoidosis and is associated with the presence of granulomas which are too small to be seen in US. CT or MRI⁽¹⁾. Calcifications as a sequel are unusual, both in the liver and spleen, but they may be found⁽³⁾. In the patient studied, we have found only homogeneous splenomegaly.

Although most usually sarcoidosis affects the liver, spleen and lymphonodus, any abdominal organ may be affected. Additionally, in radiologic studies it may mimic neoplastic, infectious or autoimmune disases, so it should be included in the differential diagnosis of abdominal organomegalies⁽¹⁾.

CONCLUSION

The authors conclude that the hepatic involvement in sarcoidosis should be taken into consideration in the follow-up of patients with pulmonary sarcoidosis, presenting with portal hypertension and upper digestive hemorrhage, understood in the possible context of a chronic hepatopathy (cirrhosis) possibly associated with sarcoidosis.

REFERENCES

Received October 28, 2004.

Accepted after revision March 15, 2005.

• 1. Warshauer DM, Lee JKT. Imaging manifestations of abdominal sarcoidosis. AJR 2004;182:1528.
• 2. Machado MM, Rosa ACF, Cerri GG. Doenças hepáticas difusas, hipertensão portal e transplante de fígado. In: Cerri GG, Oliveira IRS, editores. Ultra-sonografia abdominal. Rio de Janeiro, RJ: Revinter, 2002;55124.
• 3. Mergo PJ, Ros PR. Imaging of diffuse liver disease. Radiol Clin North Am 1998;36:365375.

*

Study developed at the Diagnosis Center of the Hospital Sírio Libanês, São Paulo, SP, at the Department of Radiology of the Faculty of Medicine, Universidade Federal de Goiás, and at the Department of Digestive Tract Diseases of Hospital Araújo Jorge of the Associação de Combate ao Câncer em Goiás, Goiânia, GO.

Publication Dates

• Publication in this collection
  25 May 2006
• Date of issue
  Apr 2006

History

• Accepted
  15 Mar 2005
• Received
  28 Oct 2004