Endomyocardial fibrosis

Chief Complaint: Shortness of breath

History of Present Illness: This 46-year-old African-American man, with a past medical history significant for end-stage renal disease, first noted increasing dyspnea 2 weeks earlier. After several days of progressive symptoms, he was admitted to the Miriam Hospital, where he was treated for community-acquired pneumonia and volume overload. He was discharged 5 days later, but did not fill prescriptions for furosemide or levofloxacin.

Four days after discharge, the patient again noted dyspnea. Onset was acute and occurred both at rest and with exertion. By the following morning, he had developed a productive cough, chest pain, fever, and chills. The patient called rescue. Pulse oximetry revealed an oxygen saturation of 71%. He was transported to the Miriam Hospital emergency department.

Review of Symptoms: Positive for sweats, subjective fevers and chills, and mild nausea with abdominal bloating. He denied vomiting, rigors, weight change, rash, and lower extremity edema.

Past Medical History: End-stage renal disease secondary to focal segmental glomerulosclerosis, on dialysis for 1 year. Hypertension, hepatitis C with cirrhosis, gout, hypercholesterolemia, pericarditis. 2D echocardiogram 9 months prior showed ejection fraction of 55% with normal left ventricular systolic function.

Medications: Labetolol 300 mg bid, amlodipine 10 mg qd, candesartan 32 mg qd, clonidine 0.3 mg bid, aspirin 325 mg qd, atorvastatin 10 mg qd, colchicine 0.6 mg qd, multivitamin, and metoclopramide 5 mg prn

Allergies: Amoxicillin, penicillin

Social History: 90 pack-year smoking history, quit 3 years ago.

Family History: Mother with hypertension, father died of a myocardial infarction.

Physical Exam:

Vital signs: Temp = 39.2 BP = 150/90 (120/78 after diuresis) HR =124 RR = 24 SaO2 = 99% 2L nasal cannula

General: Adult male, no acute distress

HEENT: No jugular venous distension, sclerae anicteric

CVS: Tachycardie, normal S1/S2, no murmur, rub, or gallop

Lungs: Fine crackles halfway up bilateral lung fields

Abdomen: Normoactive bowel sounds, soft, nontender, no organomegaly

Extremities: Trace bilateral lower extremity edema, left upper extremity arteriovenous fistula without redness, warmth, or edema

Neurologic: Nonfocal

Labs:

CBC:

WBC count: 6,800 per mL

Hemoglobin: 11.3 g/dL

Hematocrit: 36%

Platelet count: 111,000 per mL

Differential: 78% neutrophils, 8% lymphocytes

Chem 7:

Sodium: 133 mmol/L

Potassium: 6.4 mmol/L

Chloride: 96 mmol/L

Bicarbonate: 21 mmol/L

BUN: 42 mg/dL

Creatinine: 6.8 mg/dL

Glucose: 105 mg/dL

EKG: Sinus tachycardia, rate 120, left anterior fascicular block

Chest X-ray: Low lung volumes with an enlarged cardiac silhouette. Engorged hilar vasculature, haziness in the perihilar region and over the left hemidiaphragm.

Hospital Course: In the emergency department, the patient was treated for hyperkalemia. His dyspnea was attributed to incompletely treated pneumonia and volume overload, and he was admitted to the medical service. Treatment included furosemide, albuterol, azithromycin, and ceftriaxone.

Despite aggressive fluid removal, the patient continued to require supplemental oxygen. He complained of dry cough and mild dyspnea on exertion. Fine crackles persisted on pulmonary exam. High resolution computed tomography (CT) of the chest, obtained to evaluate for interstitial lung disease, was significant for honeycombing and bronchiectasis in the lower lobes, as well as extensive hilar and mediastinal lymphadenopathy.

The patient underwent thorascopic biopsy of the right lung. Mediastinal biopsy was not performed because the patient became hypoxic and hypotensive. He was extubated and admitted to the postanesthesia care unit. Phenylephrine was continued for persistent hypotension. Several hours later, he developed a wide complex irregular cardiac rhythm with a heart rate in the 90s. Within minutes, he became bradycardic and lost consciousness.

Cardiopulmonary resuscitation was begun. The patient was intubated and a transvenous pacer was placed. Scant fluid was obtained from pericardiocentesis. Per protocol, he received alternating boluses of atropine and epinephrine. Defibrillation performed for ventricular fibrillation was unsuccessful. Calcium, insulin, dextrose, and bicarbonate were administered for suspected hyperkalemia. The patient became asystolic, and resuscitation efforts were suspended after 45 minutes. Chemistries drawn during resuscitation were significant for potassium of 9.4 mmol/L.

An autopsy revealed extensive pulmonary, cardiac, and gastric sarcoidosis. Given the degree of cardiac involvement, it was hypothesized that the patient suffered a lethal arrhythmia. Hyperkalemia would have contributed, although chemistries obtained during resuscitation may not accurately reflect potassium levels immediately prior to the inciting event.

DISCUSSION:

1. What are the clinical manifestations cardiac sarcoidosis? How is it treated?

Sarcoidosis, a multisystem disorder involving formation of noncaseating granulomas, frequently affects the heart. Although cardiac involvement is documented in up to 30% of patients at autopsy, it is clinically evident in only 5%. Sudden cardiac death may be the first manifestation. Cardiac involvement can occur at any point and may precede pulmonary findings. Presentation depends on the cardiac site involved. Manifestations include rhythm and conduction disturbances, repolarization abnormalities, papillary muscle dysfunction, infiltrative cardiomyopathy with congestive heart failure, and pericarditis. Conduction abnormalities, ranging from first-degree atrioventricular block to complete heart block, are the most common findings in large case series. Rhythm disturbances most commonly take the form of sustained or non-sustained ventricular tachycardia (VT). VT is the second most common presentation of cardiac sarcoidosis and is believed to cause the majority of sudden deaths.

A high index of suspicion is essential in the diagnosis of cardiac sarcoidosis. Testing may include endomyocardial biopsy, echocardiography, radionucleotide imaging, and magnetic resonance imaging (MRI). Treatment focuses on reducing the inflammatory response. Corticosteriods can slow the progression of fibrosis, although reversal of existing fibrosis is unlikely. Resolution of conduction and repolarization abnormalities with steroid treatment has been reported. Pacemakers and automated defibrillators may be indicated. Anti-arrhythmic drugs generally are not used, with recent reviews demonstrating a strong association with increased arrhythmia recurrence and incidence of sudden death.

2. What is the typical radiographic appearance of sarcoidosis?

The lungs are the most common site involved in sarcoidosis, affecting 90% of patients. The radiographic appearance can vary considerably, but three patterns are described. Pattern I is typified by bilateral hilar adenopathy; pattern II has bilateral hilar adenopathy with concomitant interstitial infiltrates (usually in the upper lung zones); pattern III is characterized by receding hilar adenopathy and increasing interstitial infiltrates. Advanced fibrosis without adenopathy is considered a subtype of pattern III.

High resolution CT of the chest is used increasingly to evaluate the patient presenting with interstitial lung disease. In sarcoidosis, the CT demonstrates hilar adenopathy and varying degrees of ground glass opacities and fibrosis with distortion of the underlying lung architecture. The distinguishing feature is that these changes are more prominent in the upper lung zones. This patients presentation was unusual, as the underlying changes were mostly in the lower lung zones.

3. Is there an association between sarcoidosis and focal segmental glomerulosclerosis (FSGS)?

Increased calcium absorption with nephrocalcinosis is the most common cause of chronic renal failure in sarcoidosis. Interstitial nephritis with granuloma formation is seen in approximately 20% of cases, but renal insufficiency is uncommon. The course is rapidly progressive when such patients present with renal failure.

Glomerular disease is rare in sarcoidosis. The underlying pathology is diverse, including membranous nephropathy, FSGS, IgA nephropathy, and proliferative or crescenteric glomerulonephritis. FSGS can present late in the course; the pathogenesis may be related to T-cell dysfunction. Renal sarcoidosis is usually treated with corticosteriods.

REFERENCES

Altiparmak M. A rare cause of focal segmental glomerulosclerosis: sarcoidosis. Nephron 2002;90:2111-2.

Bargout R, Russell FK. Sarcoid heart disease. Int J Cardiol 2004;97:173-82.

Crystal RG. Sarcoidosis. In Kasper DL, et al, eds. Harrison's Principles of Internal Medicine New York; McGraw-Hill, 2005:2002.

Gobel U. The protean face of renal sarcoidosis. J Am Soc Neph 2001;12:616-23.

Hannedouche T, et al. Renal granulomatous sarcoidosis. Nephrol Dialysis Transplantation 1990;51:18-24.

Sharma O. Cardiac sarcoidosis. UpToDate January 13, 2005.

ANA C. TUYA, MD, AND MARY H. HOHENHAUS, MD

CORRESPONDENCE:

Ana Tuya, MD

Phone: (401) 444-4000

e-mail: atuya@lifespan.org

ACKNOWLEDGEMENTS:

David Yoburn, MD

Copyright Rhode Island Medical Society Nov 2005
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