Cholestasis and disseminated histoplasmosis in a psoriatic patient on infliximab: case report and review of literature.

BACKGROUND: Histoplasma capsulatum is the most common endemic mycosis in the United States and frequently presents as an opportunistic infection in immunocompromised hosts. Though liver involvement is common in disseminated histoplasmosis, primary gastrointestinal histoplasmosis of the liver in absence of lung involvement is rare. Similarly, cholestatic granulomatous hepatitis in liver histoplasmosis is rarely seen. CASE PRESENTATION: We present a rare case of primary gastrointestinal histoplasmosis manifesting with acute granulomatous hepatitis and cholestasis in a 48-year-old female with psoriatic arthritis, receiving methotrexate and infliximab. The epidemiology, risk factors, clinical presentation, diagnosis, and treatment of histoplasmosis is discussed. Furthermore, we review the published cases of biopsy-proven disseminated histoplasmosis with cholestatic jaundice to highlight histoplasmosis involvement in the liver. CONCLUSION: Histoplasmosis should be considered in immunosuppressed patients with fever, chills, abdominal pain and cholestasis with progressive jaundice, particularly in subjects without evidence of biliary obstruction. Future studies are needed to accurately assess the risk of this fungal infection, specifically in patients on immunomodulatory therapy for autoimmune disease.

Isolation of Burkholderia pseudomallei from a Pet Green Iguana, Belgium.

We isolated Burkholderia pseudomallei, the causative agent of melioidosis, from liver granulomas of a pet green iguana (Iguana iguana) in Belgium. This case highlights a risk for imported green iguanas acting as a reservoir for introduction of this high-threat, zoonotic pathogen into nonendemic regions.

X-linked Inhibitor of Apoptosis Complicated by Granulomatous Lymphocytic Interstitial Lung Disease (GLILD) and Granulomatous Hepatitis.

The X-linked inhibitor of apoptosis (XIAP) deficiency is a primary immunodeficiency characterized by Epstein-Barr virus (EBV)-driven hemophagocytic lymphohistiocytosis (HLH), splenomegaly, and colitis. Here, we present, for the first time, granulomatous hepatitis and granulomatous and lymphocytic interstitial lung disease (GLILD) as manifestations of XIAP deficiency. We report successful treatment of GLILD in XIAP deficiency with rituximab and azathioprine and discuss the role of XIAP deficiency in immune dysregulation.

[Q fever : A rare differential diagnosis of granulomatous disease]. / Q-Fieber : Eine seltene Differenzialdiagnose granulomatöser Erkrankungen.

Q fever is a worldwide distributed zoonotic disease with a mostly benign course, which regularly reoccurs in Germany. This report is about a patient with sporadic serologically proven Q fever, which also showed typical histopathological findings with nonspecific granulomatous hepatitis, usually seen in acute disease. The bone marrow biopsy revealed so-called doughnut granulomas, which are not pathognomonic but a typical finding in Q fever. This case report impressively underlines that the histomorphological findings can make a decisive contribution to the clarification by extended differential diagnostics, even though it plays a subordinate role in the routine diagnostics of disseminated Q fever.

Case report: granulomatous hepatitis due to Mycobacterium avium in an Atlantic yellow-nosed albatross (Thalassarche chlororhynchos) from Southern Brazilian coast.

This study reports a granulomatous hepatitis caused by Mycobacterium avium in an Atlantic yellow-nosed albatross (Thalassarche chlororhynchos) found dead on Brazil southern coast. At necropsy, the albatross was cachectic and the liver was severely enlarged with multifocal to coalescing white nodules. Histopathological evaluation revealed multifocal to coalescing granulomas with caseous necrosis, surrounded by an infiltrate of macrophages and multinucleated giant cells, and a thin capsule of fibrous connective tissue. The Fite-Faraco staining technique revealed multiple acid-fast bacilli (AFB) stained in magenta, predominantly in the areas of necrosis. Bacterial culture and polymerase chain reaction (PCR) analysis confirmed the presence of Mycobacterium avium in liver samples. This case underscores the importance of wildlife surveillance in coastal regions. Pelagic birds like the Atlantic yellow-nosed albatross can harbor pathogenic agents that represent a threat to wildlife and domestic animals. Enhanced monitoring and research are essential to understand the epidemiology and potential risks associated with such infections in coastal ecosystems.

Unusual coexistence of low-grade mucinous cystic neoplasm and idiopathic granulomatous hepatitis.

INTRODUCTION AND IMPORTANCE: Mucinous cystic neoplasms (MCNs) of the liver are rare precancerous lesions, accounting for less than 5 % of all hepatic cysts. The coexistence of MCNs with idiopathic granulomatous hepatitis is exceedingly uncommon and lacks documentation in the existing literature. CASE PRESENTATION: A 43-year-old Tunisian woman with no significant medical history presented with persistent right hypochondrium pain. Clinical examination revealed a palpable mass in the right hypochondrium. Laboratory tests indicated elevated liver enzymes and cholestasis. Imaging studies identified an 18 × 11 cm cystic formation in the right liver lobe, initially suspected to be a type I hydatid cyst. The patient underwent a right hepatectomy, and histological examination confirmed the presence of a low-grade MCN of the liver coexisting with idiopathic granulomatous hepatitis. The patient experienced an uncomplicated postoperative recovery. CLINICAL DISCUSSION: In our case, the concomitant presence of MCN and idiopathic granulomatous hepatitis was incidental, without any causal link. The definitive diagnosis of these two conditions relies on histopathological examination. It is essential to surgically remove the MCN and identify the cause of granulomatous hepatitis to effectively manage the patient. CONCLUSION: This case underscores the uncommon occurrence of both MCN and idiopathic granulomatous hepatitis in the liver, highlighting the diagnostic challenges associated with the latter. Accurate diagnosis through thorough evaluation is essential for effective management.

Granulomatous hepatitis with Crohn's disease: a case report.

A 45-year-old man who was regularly followed up for Crohn's disease (CD) and maintained clinical remission with vedolizumab (VDZ). At 37 years old, he was diagnosed CD from longitudinal ulcers in the distal ileum by balloon-assisted enteroscopy (BAE). During the follow-up, liver enzyme elevation, splenomegaly and thrombocytopenia were in progress. Esophagogastric varices suggested chronic liver disease and portal hypertension. Magnetic resonance elastography (MRE) showed liver stiffness of 3.4 kPa and proton density fat fraction (PDFF) of 1.86%. He was diagnosed with granulomatous hepatitis based on a liver biopsy. The hepatic venous pressure gradient (HVPG) was mildly elevated at 7 mmHg, consistent with the pre-sinusoidal portal hypertension due to granulomatous hepatitis. We report a rare case with granulomatous hepatitis diagnosed from liver injury and portal hypertension, despite the stable intestinal symptoms of CD.

Granulomatous Hepatitis Treated With Certolizumab Pegol.

Idiopathic granulomatous hepatitis is a rare condition characterized by hepatic granulomas with constitutional symptoms such as recurrent fevers, myalgias, and hepatosplenomegaly in the absence of infection or inflammatory disorder. Typical treatment and course of this disease consist of a course of steroids with rapid symptom resolution. However, symptoms may recur when steroids are tapered. In these circumstances, azathioprine, methotrexate, infliximab, and adalimumab have demonstrated good response. In this case, we present a patient who developed antidrug antibodies to infliximab and adalimumab and was the first documented case of this disease to be treated with certolizumab pegol. Our case highlights the novel efficacy of certolizumab pegol for idiopathic granulomatous hepatitis and its role in treating idiopathic granulomatous hepatitis with antidrug antibodies.

Turner syndrome with isochromosome Xq as a cause of granulomatous hepatitis: a case report.

Introduction: Turner syndrome (TS) is the most common sex chromosome abnormality in women, caused by a complete or partial absence of the second sex chromosome. The karyotype 46, X,i(Xq) is the underlying cause in about 10% of the cases of TS. Hepatic abnormalities are frequent in TS. Granulomas are relatively common in liver samples but are very rarely reported in TS. Case presentation: A 15-year-old female with TS attended a consultation for evaluation of elevated liver enzymes. Her chromosomal analysis showed mosaicism 46, X (iso xq)100%. There were no stigmata of chronic liver disease. A liver biopsy showed granulomatous hepatitis. Other causes of hepatic granulomas have been excluded. Ursodeoxycholic acid (UDCA) therapy leads to the normalization of transaminases. Clinical discussion: Although Hepatic involvement is common and mostly asymptomatic in TS, the mechanism of liver injury is not well understood. The hepatic histological changes in these cases are variable and range from minimal abnormalities to nonalcoholic steatohepatitis (NASH), liver architectural changes, and biliary lesions. Hepatic granulomas are associated with a wide range of systemic disorders but are very rarely reported in tuner syndrome. Normalization of liver enzymes after treatment with UDCA was previously reported, but the importance of this approach is to be determined. Conclusion: Granulomatous hepatitis may be associated with TS and may be added to the histological patterns encountered in this disorder.

Bacillus Calmette-Guérin (BCG)-Induced Pneumonitis: A Case Report.

Bladder cancer is the second most common genitourinary (GU) malignancy worldwide. Treatment involves early cystectomy and intravesical Bacillus Calmette-Guérin (BCG), which is effective for T1 high-grade tumors and carcinoma in situ (CIS) but can cause significant side effects, including chemical and bacterial cystitis, hematuria, incontinence, pneumonitis, malaise, fever, and sepsis. We present the case of a 47-year-old male with transitional cell carcinoma (TCC, G3 pTa) treated with transurethral resection of bladder tumor (TURBT) who developed a fever and non-productive cough after BCG injections. Initially discharged, he returned with worsened symptoms. His vital signs showed a fever of 38.2°C, a heart rate of 104 beats per minute (bpm), and a saturation of 93% on room air. Blood tests indicated inflammation and liver dysfunction. Imaging revealed lung micronodularity, and further CT imaging showed bilateral miliary nodules indicative of BCG pneumonitis. MRI ruled out disseminated tuberculosis, identifying a hepatic cyst. Cultures from blood, urine, sputum, and broncho-alveolar lavage were negative, but granulomatous inflammation was confirmed on liver biopsy. The patient was treated with oral glucocorticoids and anti-tuberculosis medications (rifampicin, isoniazid, and ethambutol), and clinical improvement was shown. The patient was discharged, and a follow-up at the respiratory clinic was scheduled. BCG pneumonitis, a severe BCG therapy complication, necessitates early diagnosis and management to reduce morbidity and mortality.

A Rare Case Report of Disseminated Nocardia Farcinica Granulomatous Hepatitis and Clinical Management Experience.

Background: Nocardiosis is primarily an opportunistic infection affecting immunocompromised individuals, with a predilection for the lungs, brain, or skin in those with compromised immune function. Granulomatous hepatitis caused by Nocardia is a rare clinical manifestation. This study aims to provide a systematic overview of the clinical features of Nocardiosis caused by Nocardia farcinica, enhancing our understanding of this disease. Methods: We report a case of a 75-year-old male with no underlying diseases presenting with a history of "recurrent fever for more than 4 months", along with fatigue, poor appetite, and pleural and abdominal effusion. Despite treatment at multiple hospitals, the patient showed little improvement. Chest CT revealed chronic inflammation, small nodules, bilateral pleural effusion, and pleural thickening. Abdominal CT indicated multiple low-density lesions in the liver, multiple small calcifications, and abdominal effusion. Results: Liver biopsy suggested inflammatory changes, with focal granuloma formation. Metagenomic next-generation sequencing (mNGS) of liver tissue indicated Nocardia farcinica, leading to the final diagnosis of disseminated Nocardia farcinica granulomatous hepatitis. Conclusion: Nocardia infection is a rare disease primarily observed in immunocompromised patients but can also occur in those with normal immune function. The clinical and radiological features lack specificity; however, the utilization of mNGS technology enables rapid identification of the pathogenic microorganism. Nocardia farcinica is generally susceptible to sulfonamide drugs and amikacin, offering viable treatment options.

A Case of Disseminated Histoplasmosis Presenting As Adrenal Insufficiency With Granulomatous Hepatitis.

Histoplasmosis is a fungal infection caused by Histoplasma capsulatum. In the United States, histoplasmosis is endemic in the Mississippi River Valley and Ohio. Histoplasmosis is often asymptomatic in immunocompetent individuals, and severe disseminated cases are more often seen in immunosuppressed patients. Disseminated histoplasmosis often affects the reticuloendothelial system, invading specific visceral organs such as the liver, spleen, and pancreas. The current study presents a unique case of disseminated histoplasmosis in a 64-year-old immunocompetent male. The patient's presentation included a 40-lb weight loss over a year, bilateral adrenal nodules, abnormal liver enzymes, and granulomatous hepatitis, which initially raised suspicion of a malignant etiology. An adrenal mass biopsy showed fungal morphology that confirmed an H. capsulatum infection. Further history showed that the patient recently traveled to Bangladesh, which is thought to be a region endemic to histoplasmosis. This case is noteworthy because disseminated histoplasmosis rarely affects immunocompetent individuals, and an infectious etiology for adrenal insufficiency is exceedingly rare, especially in the United States. The treatment regimen included a 14-day induction therapy of IV amphotericin B followed by outpatient itraconazole, leading to symptom resolution. This case highlights the need to consider an infectious etiology for adrenal insufficiency, especially among immunocompetent individuals who may be at risk after traveling to endemic areas.

Facing the Unknown: Idiopathic Giant Cell Hepatitis.

Giant cell hepatitis is a rare infiltrative disease associated with several viruses, drugs, malignancies, and autoimmune conditions. To date, treatment aims at controlling the underlying etiology, and there are limited data on the clinical course and treatment of idiopathic cases. We present a case of idiopathic giant cell hepatitis in an otherwise healthy adult man and review the literature regarding treatment and outcomes in this population.

Disseminated Histoplasmosis in Central California Seen in an Immunocompromised Patient.

Histoplasma capsulatum is a dimorphic fungus found in certain parts of North, Central, and South America. Transmission is primarily through airborne inoculation from inhaled fungal microconidia. Histoplasmosis is typically a self-limited mycosis; however, in patients with immunodeficiency, disseminated disease can occur and may lead to high disease burden. This report studies a case of disseminated histoplasmosis in a patient newly diagnosed with human immunodeficiency virus. His presentation on admission was consistent with infectious pulmonary granulomatous disease, and further imaging and laboratory results showed evidence of multi-organ involvement. It is likely his presentation in Central California was a reactivation infection after inoculation in Central America many years ago.

Disseminated Mycobacterium bovis Infection: A Rare Complication of Bacillus Calmette-Guérin Immunotherapy.

Intravesical instillation of bacillus Calmette-Guérin (BCG) is a crucial adjunctive therapy in the treatment of bladder carcinoma. Its systemic complications are rare but include disseminated Mycobacterium bovis (M. bovis) infections, the diagnosis of which is a challenging task that requires keen clinical awareness. We report the case of an adult male treated with BCG who presented with fever, nonspecific constitutional symptoms, hepatic cytolysis, and cholestasis. After a detailed workup, the diagnosis was made of disseminated M. bovis infection with hepatic, pulmonary, renal, and ureteral involvement. Prompt anti-tuberculosis treatment resulted in clinical and analytical improvement. This case highlights the importance of early recognition of this serious complication in patients with BCG exposure, as well as the difficulty in confirming the diagnosis for proper treatment.

The Diagnostic Dilemma of Acute Granulomatous Hepatitis in a Patient With Crohn's Disease: A Case Report and Review of Literature.

Liver involvement is not an uncommon extraintestinal manifestation of inflammatory bowel disease (IBD). IBD-associated liver diseases may have a variety of etiopathogenetic origins (including shared autoimmune pathogenesis, the effect of chronic inflammatory status, and adverse effects of drugs). Nevertheless, acute granulomatous hepatitis in the setting of Crohn's disease (CD) is a rare clinical entity. It warrants, however, a careful assessment as both clinical and pathological features of Crohn's-associated granulomatous hepatitis closely mimic extrapulmonary hepatic sarcoidosis, with considerable overlaps between the 2 diseases, which certainly makes a definitive diagnosis quite challenging. It is crucial to exclude infectious etiologies during the evaluation of acute granulomatous hepatitis, as inappropriate immunosuppressive treatment may cause a systemic flare-up of an underlying liver infection. We report a rare case of a 35-year-old female with a history of CD who presented with recurrent fevers, acute abdominal pain, and cholestasis. She was found to have acute hepatitis with noncaseating granulomas on liver biopsy. A comprehensive diagnostic workup did not ultimately prove a specific etiological culprit. The patient was treated with oral corticosteroids, and she demonstrated a positive clinical and laboratory response to the treatment. Our case highlights the diagnostic dilemma of acute granulomatous hepatitis in the setting of co-existent CD with a multisystemic syndrome. Granulomatous hepatitis represents a relatively rare manifestation of both extraintestinal CD and extrapulmonary sarcoidosis, with potential difficulties discriminating between the 2 entities on many occasions. The case also demonstrates the value of an interdisciplinary approach in the context of multisystemic disease to achieve the best outcome.

Granulomatous Hepatitis Following Intra-Vesical Instillation of Bacillus Calmette-Guérin for Treatment of Bladder Cancer.

Intra-vesical instillation of bacillus Calmette-Guérin (BCG) is an important treatment modality of superficial bladder cancer. It is usually well tolerated, although some adverse reactions can occur. One possible yet rare complication is granulomatous hepatitis, that is thought to be caused either by BCG infection or a hypersensitivity reaction to the bacillus. We present a case of a 79-year-old apparently immunocompetent patient who developed granulomatous hepatitis a few months after BCG administration for bladder cancer immunotherapy. It is important to notice that acid-fast smears and cultures are often negative, and these should not exclude diagnosis nor delay treatment. Our case highlights the importance of clinical suspicion and prompt initiation of appropriate treatment.

Granulomatous Hepatitis Secondary to Histoplasmosis in an Immunocompetent Patient.

Histoplasma capsulatum is the most common endemic mycosis in the United States and usually occurs in certain geographic areas, such as the Mississippi or Ohio River valleys. Histoplasmosis usually causes a mild disease in the immunocompetent but can progress to disseminated disease in patients with impaired immunity. Granulomatous hepatitis as a manifestation of disseminated histoplasmosis in immunocompetent patients is extremely rare. We report the case of a 62-year-old immunocompetent gentleman with a history of histoplasmosis who presented with abdominal pain, elevated liver enzymes, who was diagnosed with granulomatous hepatitis secondary to histoplasmosis.