Purpose: To describe the clinical features, biological datas and outcome of patients with systemic sarcoidosis and splenomegaly.
Methods: A retrospective analysis of 17 patients presenting splenomegaly and sarcoidosis with histological proof.
Results: Splenomegaly was clinically perceptible in 13 patients, with a spleen size that extended 4 cm or more below the costal margin in 11 patients. It was painful in five cases. The more frequent clinical features are constitutional symptom (fever in 9 cases) and hepatomegaly (N =7). Chest X-ray showed bilateral hilar lymphadenopathy in nine patients and no abnormality in five cases. Serum angiotensin converting enzyme levels were elevated in 81% of cases. Thrombopenia (N =5) and hypersplenism (N =5) were also observed. Corticosteroid were given to 88% with a good clinical and biological response including a decrease in the spleen volume. Corticotherapy and splenectomy (performed in two patients to rule out lymphoma) didn't change outcome of disease. Sarcoidosis is often chronical (82%) and extensive.
Conclusion: Splenomegaly may be present in sarcoidosis. Management is not standardized. Corticosteroid is indicated for symptomatic or massive splenomegaly. Splenomegaly is frequently in chronic and extensive sarcoidosis.