Readmission of a 61-Year-Old Patient

Case Report: Readmission of a 61-Year-Old Patient with Worsening Ascites and New-Onset Jaundice

Introduction

Systemic amyloidosis is a rare but serious clinical condition, usually characterized by varied organ involvement that hinders diagnosis and treatment. This case report describes the course of a 61-year-old African-American male with primary (AL) amyloidosis, who initially presented with ascites and nephrotic-range proteinuria, and was later readmitted with worsening ascites and new jaundice. The case illustrates the intracachability of disease monitoring, risk of rapid progression, and the need for timely interventions.

This article presents a thorough case review, buttressed by clinical observations, diagnostic testing, and discussion of management.

Patient Background

The patient was a 61-year-old male who initially presented with:

• Subacute abdominal and lower extremity swelling for two months
• Bilateral lower extremity edema
• Tense ascites
• Jugular venous distension

Initial lab investigations provided the following results:

• Albumin: 2.1 g/dL (hypoalbuminemia)
• Alkaline Phosphatase (AP): 718 IU/L
• AST: 158 IU/L
• ALT: 119 IU/L
• Total Bilirubin (TB): 0.5 mg/dL (normal at the start)
• INR: 1.07
• Creatinine: 0.9 mg/dL
• Urinalysis: Nephrotic range proteinuria

Further workup ruled out viral hepatitis, autoimmune illness, infection, and malignancy.

Imaging and Diagnostics

• Abdominal CT Scan: Ascites with potentially nodular liver; no hepatosplenomegaly
• Serum Ascites Albumin Gradient (SAAG): >1.1, indicative of portal hypertension
• Echocardiogram: Left ventricular hypertrophy, concerning for an infiltrative cardiomyopathy
• Sigmoid Colon Biopsy: Positive for amyloid deposition
• Mass Spectroscopy: Established primary AL amyloidosis

This established that the systemic illness had multi-organ involvement, specifically impacting the liver, kidneys, and potentially the heart.

Readmission and Clinical Deterioration

About one month following hospital discharge, the patient was readmitted with:

• Increased ascites (indicating persistent or deteriorating portal hypertension and hepatic dysfunction)
• New-onset jaundice (not originally present)

Implications of New-Onset Jaundice

The occurrence of jaundice in amyloidosis signifies:

1. Hepatic involvement: Progressive amyloid infiltration of the liver resulting in cholestasis or hepatocellular damage.
2. Worsening prognosis: Jaundice in systemic amyloidosis is linked to poor outcomes and decreased survival rates.
3. Complicated management: Ascites control, hepatic function, and systemic therapy become increasingly difficult.

Clinical Discussion

1. Pathophysiology of Ascites in Amyloidosis

• Amyloid deposition in the hepatic sinusoids results in deranged blood flow and portal hypertension.
• Hypoalbuminemia is a contributor to decreased oncotic pressure, which further aggravates ascites.

2. Significance of New-Onset Jaundice

• Points to cholestatic dysfunction or diffuse hepatic amyloid deposition.
• Implies that disease evolution has reached a critical phase.

3. Prognostic Considerations

• Individuals with hepatic amyloidosis and jaundice frequently have a survival interval of weeks to months.
• Treatment usually centers on supportive therapy and management of symptoms.

**4. Differential Diagnosis for Jaundice in This Setting

• Progression of hepatic amyloidosis (most probable)
• Obstruction of bile (unlikely since CT revealed no obstruction)
• Drug-induced liver injury (second consideration)
• Viral hepatitis (already excluded)

Management Strategies

During Readmission

The primary treatment goals would have been:

• Relief of symptoms: Repeated paracentesis for tense ascites
• Nutritional support: Management of hypoalbuminemia and poor oral intake
• Support of liver function: Monitoring bilirubin and coagulation factors
• Systemic therapy: If the patient’s condition permitted, considering chemotherapy (e.g., bortezomib-containing regimens)

Challenges in Treatment

• Advanced liver dysfunction usually restricts chemotherapy.
• The risk of complications (hepatic encephalopathy, bleeding varices, renal failure) is greatly enhanced.
• Many patients need palliative measures aimed at comfort and quality of life.

Literature Review on Similar Cases

Published reports have identified the evolution from ascites to jaundice in systemic amyloidosis:

• Gertz MA et al. discussed how hepatic amyloidosis with jaundice has a very poor prognosis, frequently less than six months.
• Kyle RA’s work revealed that once bilirubin becomes elevated greatly, patients do not have much tolerance for treatment.
• Comparing cases reveals that portal hypertension and jaundice are late presentations of systemic amyloidosis.

Key Learning Points from This Case

1. Rapid progression: Amyloidosis can progress rapidly even after stabilization at the beginning.
2. Jaundice as a red flag: Jaundice indicates severe hepatic impairment and unfavorable prognosis.
3. Multidisciplinary care: Involves hepatology, nephrology, cardiology, and oncology consultation.
4. Need for early diagnosis: Early detection of amyloidosis enhances survival prospects with focused treatment.
5. Patient education: Families must be educated regarding disease course and palliative measures.

Conclusion

This case illustrates the complex clinical course of systemic AL amyloidosis, as a patient initially presented with ascites and then was readmitted with worsening ascites and new-onset jaundice. The transition reflects the advancement in hepatic involvement and a reminder of the aggressive nature of amyloidosis.

Early diagnosis, intensive monitoring, and palliative care are still the most important. Sadly, once jaundice appears, the outlook is unfavorable, and treatment tends to shift towards quality of life and symptom relief.