Pleural Effusion with Splenic Rupture as Manifestations of Recurrence of Sarcoidosis following Prolonged Remission

Abstract

A 55-year-old man presented with a 3-week history of dry cough and left pleuritic chest pain with a new exudative pleural effusion. Sixteen years earlier, he was diagnosed with sarcoidosis presenting with hilar lymphadenopathy, erythema nodosum, mildly disturbed liver function tests and noncaseating granulomata on liver biopsy, with no evidence of pulmonary parenchymal disease. He was treated with prednisone and in recent years maintained at a low daily dose, until it was eventually discontinued two years prior to his present illness. There was no evidence of infection or malignancy, and the fluid resolved following treatment with naproxen. Three weeks later the patient presented with sudden onset of dyspnea and left chest pain. After starting intravenous heparin for suspected pulmonary emboli, the patient developed hemodynamic instability which was accompanied by abdominal tenderness and decreasing hematocrit. Splenic rupture was diagnosed, and the patient underwent splenectomy. Pathology specimens revealed a hemorrhagic infarct with subcapsular hematoma, and numerous noncaseating granulomata within the splenic tissue. This patient had recurrent sarcoidosis with two rare manifestations of the disease, 2 years after withdrawing low dose prednisone, given for a prolonged time. The possibility of reemergence of the disease in organs other than the organs involved in the initial presentation should always be considered in sarcoidosis.

Splenic Rupture, Beyond and Behind: A Histological, Morphometric and Follow-Up Study of 254 Cases

“…Surprisingly, sarcoidal reactions have so far only been reported 6 times in ruptured spleens [36] . In autopsy studies, splenic involvement has been noted in 40-80% of the sarcoidosis patients, often accompanied by minimal splenomegaly with a homogeneous appearance [37] .…”

A Rare Involvement of Sarcoidosis: Massive Splenomegaly

“…Diğer bir çalışmada da 235 sarkoidoz hastası incelenmiş, 32'sinde (%14) splenomegali, 11'inde (%5) kosta sınırını 4 cm ve üzerinde geçen masif splenomegali tespit edilmiştir [7]. Literatürde sarkoidoza bağlı hem masif splenomegali [5,8] hem de spontan veya travmatik splenik rüptür şeklinde rapor edilmiş nadir olgu sunumları mevcuttur [4,12]. Bizim ülkemizden ilk kez bu yazı ile sarkoidoza bağlı masif dalak tutulumu rapor edilmektedir.…”

Clinical analysis of sarcoidosis presenting with heterochronic cardiac involvement

“…reported that cardiac involvement may occur at any point during the course of sarcoidois, in the absence of pulmonary or systemic involvement, and may present postdiagnosis because sarcoidosis can involve any part of the heart 12 . The possibility of disease re‐emergence in organs other than those involved at initial presentation should be considered in sarcoidosis 13 . Cardiac involvement is a leading cause of sudden death, and so warrants attention.…”

“…12 The possibility of disease re-emergence in organs other than those involved at initial presentation should be considered in sarcoidosis. 13 Cardiac involvement is a leading cause of sudden death, and so warrants attention.…”