Isolated Splenic Sarcoidosis and Vitamin D deficiency – A synergistic combination
Dr. Sanjeev Sharma , Dr. Prasanth Vas, Dr. Craig Parkinson
Sarcoidosis is a granulomatous disease of unknown etiology. Over 90% of patients with sarcoidosis present with pulmonary findings at the time of diagnosis. Extrapulmonary involvement is common, including the liver, eyes, central nervous system, lymph nodes, and joints. However, isolated granulomatous disease confined to the spleen is rare. This case report documents a rare case of isolated granulomatous disease of the spleen whose management was complicated with concomitant vitamin D deficiency.
A 63-year-old female was admitted with a 4 week history of weight loss and hyperosmotic symptoms. Her past medical history comprised of chronic atrial fibrillation and sero-negative arthritis. Her initial assessment found her to be hypercalcaemic with corrected Calcium at 3.54 mmol/L (N=2.2-2.6mmol/L), Phosphate 1.24 mmol/L (N=1.2-1.8mmol/L), PTH 3.3 pmol/L (N=0.95-5.7 pmol/L), and in acute renal failure (urea: 12.1 and creatinine: 293). She was managed initially with intravenous fluids and bisphosphonate therapy. The differential diagnosis at this time included parathyroid pathologies, neoplasm, sarcoidosis and other granulomatous infections. Subsequent investigations demonstrated a negative myeoloma screen and an elevated ACE level at 207 u/L (N=8-55 u/L). Although the CXR was normal, USG abdomen showed a homogenously enlarged spleen (14.3 cm) with focal calcifications. This was further confirmed with a CT thorax and abdomen which demonstrated moderate splenomegaly with multiple small lymph nodes in the chest and abdomen but without any definitive pulmonary parenchymal involvement. USG neck showed a suggestive parathyroid adenoma in the right inferior thyroid aspect, but a parathyroid substraction scan showed discordance without any focal tracer uptake. The diagnosis of an Isolated Splenic sarcoidosis was confirmed with a splenic biopsy which showed non-necrotising epithelioid granulomas consistent with sarcoidosis. She was treated with oral Prednisolone and demonstrated a favourable response with reductions in both ACE and calcium levels.
Her management was complicated with low 25-OH Vitamin D (8.4 μg/L) levels which could be explained as a result of increased substrate conversion to 1-25(OH)2 Vitamin D by macrophage activation. Hence, despite quite low levels of vitamin D, a judicious strategy not to treat this deficiency was undertaken. This intriguing case demonstrates two interesting aspects: firstly, isolated extrapulmonary manifestations of sarcoidosis occur in only 10% of patients and of which, isolated splenic sarcoidosis is rare. Secondly, it also highlights the complexities of Vitamin D management in granulomatous conditions like sarcoidosis. Our patient continues to have follow-up for systemic manifestations and associated complications of sarcoidosis.