A 30-year-old male suffered an intermittent fever with a maximum temperature of 39 C since July 2017. He had a history of type 1 diabetes mellitus for 8 years. He was admitted to a local hospital and received cephalosporin therapy for a week, and then his temperature returned to normal. A CT scan of the chest showed lymphadenopathy in the mediastinum and hilar regions and multiple small nodules in both lungs. His white blood cell count was 2.9 x 109/L with severe lymphopenia of 0.5 x 109/L, and hemoglobin was 107 g/L, while the platelet count was average at 331 x 109/L. He received a positron emission tomography-CT scan to identify whether it was benign or malignant, which revealed multiple enlarged lymph nodes with hypermetabolism in the bilateral hilum, mediastinum, axilla, and clavicle, and lymphoma could not be excluded (Figure 1). Subsequently, the patient underwent a biopsy of mediastinal lymph nodes performed by thoracoscopy, and the pathologic result was granulomatous lesions; thus, type II sarcoidosis was diagnosed. However, he did not receive glucocorticoid therapy with the consideration of his diabetes history and the early stage of sarcoidosis, and then he was told to recheck every 6 months. In the following 2 years, the patient suffered from intermittent fever (T max 37.7 C), which could be alleviated by oral antibiotics, accompanied by dyspnoea, fatigue, occasional cough, less sputum, and significant weight loss (lost 50 pounds). It is unfortunate that the patient was not regularly rechecked as prescribed. Last month, he complained that he had experienced progressive abdominal distention and dyspnoea, so he came to our hospital. The patient was sane without nausea and vomiting, and no positive signs were found on neurological examination. Abnormal physical findings consisted of a few scattered skin ulcers on the legs and splenomegaly extending 5 cm below the left costal margin. Abdominal ultrasonography revealed enhanced hepatic parenchyma echo, gallbladder (8.13 x 3.29 cm) and spleen enlargement (17.29 x 5.48 cm), and peritoneal effusion (depth of ~7.34 cm). An abdominal contrast-enhanced CT scan also revealed multiple retroperitoneal lymph nodes. A CT scan of the chest showed lymphadenopathy in the mediastinum and hilar regions with multiple nodules, patchy shadows, ground-glass opacities, and emerging bilateral pleural effusion as well as right atelectasis. Cardiac ultrasound reported that there were no abnormalities in cardiac structure, blood flow, or left ventricular systolic function, with an ejection fraction of 69%. No abnormal finding was seen in ECG. Ophthalmic tests including vision and fundus oculi were both normal. Superior lymph node ultrasound revealed enlargement throughout the left supraclavicular lymph nodes, bilateral inguinal nodes, popliteal lymph nodes, left neck lymph nodes, and bilateral axillary lymph nodes (Grade 3). Blood cell count was a remarkable abnormality with marked leucopenia and anemia. The liver function test suggested liver damage and hypoproteinemia. In addition, the patient also had severely impaired immune function. Laboratory test results are shown in Table 1.
In summary, the patient had multiple organ involvement, and the disease progressed rapidly during these 2 years. During hospitalization, he took thoracentesis. As shown in Table 2, the pleural effusion was a yellow exudate. Cytopathologic examination of the pleural fluid was negative for malignant cells. In addition, both M. tuberculosis strains (acid-fast) and tuberculosis RT-PCR were negative. Subsequently, a biopsy of the left supraclavicular lymph node revealed the non-caseating granulomas with negative results for acid-fast staining and tuberculosis PCR. This result was in accordance with sarcoidosis (Figure 2). Histological analysis of the pleura obtained by thoracoscopy also revealed granulomas (Figure 3). A bone marrow aspirate and biopsy showed partial hypoplasia and no evidence of a lymphoproliferative disorder was found. In addition, a biopsy was taken from the left leg ulceration also revealed granulomas with a negative mycobacterium genetic test and culture results of fungi and bacteria (Figure 4). Abdominocentesis was not performed due to intestinal bloating and fewer ascites. Based on the above pathological results, the diagnosis of sarcoidosis was strongly suspected. Because of severe conditions and multiorgan involvement, 40 mg methylprednisolone was given intravenously once daily for seven consecutive days, followed by oral methylprednisolone 24 mg once daily.
After 1 month of methylprednisolone therapy for 24 mg once daily, there was no reduction in pleural effusion on high-resolution CT (HRCT), and the spleen was larger than before; thus, we continued to maintain the current dosage (24 mg once daily). After another months' treatment, the right pleural effusion was decreased slightly, then the dose of methylprednisolone was reduced to 16 mg once daily. Six months later, a follow-up HRCT showed that the bilateral pleural effusion was significantly less than before (Figure 5). Hepatic function (Figure 6) and superficial lymph nodes and spleen were reduced. After methylprednisolone administration (the dosage was still 16 mg/day) for 9 months, the patient became asymptomatic with a normal blood cell count (Figure 7), and the CD4+ T cell count increased from 82 to 162. Ascites were negative, and the spleen and gallbladder were both reduced. In addition, the leg skin ulcers and scabs completely disappeared (Figure 8). We then focused on the patient's side effects of glucocorticoids. The patient had no psychiatric symptoms or gastrointestinal discomfort. Blood glucose levels were reasonably controlled by subcutaneous injection of insulin (fasting blood glucose and postprandial blood glucose levels were maintained at 6-8 and 8-10 mmol/l, respectively).
(A-C) Partial images of positron emission tomography-CT scan in 2017. (B) Hilar lymph nodes enlargement.