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A Case of Splenic Sarcoidosis: Initial Diagnosis and One-Year Symptom Follow-up FREE TO VIEW

Abhay Vakil, MD; Hineshkumar Upadhyay, MD; Khalid Sherani, MD; Kelly Cervellione, PhD; Alan Fein, MD
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Jamaica Hospital Medical Center, Jamaica, NY

Chest. 2014;145(3_MeetingAbstracts):210A. doi:10.1378/chest.1826507
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SESSION TITLE: ILD Case Report Posters II

SESSION TYPE: Case Report Poster

PRESENTED ON: Sunday, March 23, 2014 at 01:15 PM - 02:15 PM

INTRODUCTION: Isolated extra-pulmonary manifestations of sarcoidosis occur in only 10% of cases. Exclusive splenic involvement is extremely rare; a review revealed only 5 prior cases reported in the literature. We report the case of a middle-aged female incidentally found to have splenomegaly on physical exam after presenting with non-specific constitutional symptoms. After extensive work-up, splenic resection confirmed sarcoidosis. One year follow-up revealed no development of pulmonary manifestations.

CASE PRESENTATION: A 40-year-old female presented with low-grade fever and fatigue for 6 weeks. She denied any cough, shortness of breath, weight loss or loss of appetite. Physical exam revealed splenomegaly extending 4 fingers below the costal margin, with no hepatomegaly. Laboratory studies including calcium level were normal. Peripheral smear and bone marrow biopsy were normal. HIV test was negative. Imaging studies confirmed massive splenomegaly with no lymphadenopathy. PET scan showed multiple hypermetabolic splenic lesions (SUV 13.0). Laparoscopic splenectomy was performed. Pathology revealed non-caseating granulomas with negative fungal and AFB stains, consistent with sarcoidosis. She was monitored for development of pulmonary and other extra-pulmonary manifestations. After 1 year she developed joint pain, swelling and erythema nodosum-like skin lesions. Extensive rheumatologic work-up was negative. Treatment with oral steroid therapy led to resolution of her symptoms.

DISCUSSION: Diagnosing isolated splenic sarcoidosis is difficult due to non-specificity, or complete lack, of overt symptoms. When no other systemic manifestations are present, it is essential to rule out other causes of splenomegaly including malignancy, infections, infiltrative diseases and hypersplenic states. Patients with isolated splenic sarcoidosis should be monitored for later development of pulmonary or extra-pulmonary manifestations. When asymptomatic, steroid therapy is not required. Indications for splenectomy in patients with an established diagnosis of sarcoidosis include severe hypersplenism, prophylaxis for splenic rupture, splenomegaly unresponsive to corticosterioids and neoplastic exclusion.

CONCLUSIONS: This case illustrates the importance of including sarcoidosis in the differential diagnosis of patients presenting with isolated splenomegaly and no pulmonary symptoms.

Reference #1: Giovinale M, Fonnesu C, Soriano A, et al. European Review for Medical and Pharmacological Sciences 2009; 13(Suppl 1): 37-44.

Reference #2: Zia H, Zemon H, Brody F. Journal of Laparoendoscopic & Advanced Surgical Techniques. April 2005, 15(2): 160-162.

Reference #3: Warshauer DM, Lee JKT. AJR 2004; 182: 15-28

DISCLOSURE: The following authors have nothing to disclose: Abhay Vakil, Hineshkumar Upadhyay, Khalid Sherani, Kelly Cervellione, Alan Fein

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