Multisystemic sarcoidosis—important lessons learnt from one of the great imitators

Abstract

We report a case of a woman who was admitted with a suspicion of metastatic malignancy of unknown primary origin. A few months prior to her admission, she presented to a rheumatologist with acute anterior uveitis, psoriasiform rashes and polyarthritis. A diagnosis of psoriatic arthropathy was made and she was treated accordingly. Soon after she presented with persistent back and right upper quadrant abdominal pain for which she had a CT scan done with evidence of hilar lymphadenopathy, liver hypodensities and lytic-sclerotic bone lesions. She was referred to our hospital for further investigations and management. After re-exploring her clinical presentation and further investigations (including a liver biopsy), a diagnosis of multisystemic sarcoidosis with ocular, reticuloendothelial, hepatic and skeletal involvement was made. The patient was started on systemic glucocorticoids and second line immunosuppressants and demonstrated significant clinical improvement with resolution of her liver granulomata on imaging and improvement in her back pain. The case illustrates the importance of a thorough clinical assessment, review of investigations and an open mind in the evaluation of a patient.

Imbalance of Lymphocyte Subsets and CD45RA-Expressing Cells in Intrathoracic Lymph Nodes, Alveolar Compartment and Bloodstream of Pulmonary Sarcoidosis Patients

“…Moreover, our study is the first to describe altered Th-CD45RA − subsets in three anatomical compartments of sarcoidosis patients with higher Th2 and Th17 cell percentages in PB than in BAL samples, and higher percentages of Th1 and Treg in LLN and BAL samples than in PB, respectively. Besides supporting the multisystem nature of the disease [ 21 , 22 ], these findings raise concerns about low cell immunity in peripheral blood of sarcoidosis patients [ 23 ]. The regulatory counterpart of Th cells accumulating in the alveolar compartment may be due to peripheral recruitment; nevertheless, the proportion of regulatory CD127-expressing cells (Th naïve) was lower in the alveolar compartment than in PB, probably because pathogenic T cells are resistant to suppression signalling by Treg cells [ 24 ].…”

Sarcoidosis with hepatic involvement in a 60-year-old patient

“…CECT gives a better overview of the extent and distribution of abdominal lymphadenopathy. While more sensitive than ultrasound, only 10-15% of patients with abdominal sarcoidosis show hypoattenuating liver and/or spleen nodules on CECT [28]. CECT may also be useful in detecting sub-capsular fibrosis, which reflects loss of hepatocytes and replacement with fibrosis as seen in liver cirrhosis [29].…”

Liver Sarcoidosis

“…В этих случаях требуется исключить в первую очередь метастатическое поражение печени, рак печени, лимфогранулематоз, а также генерализованный гематогенный туберкулез. Длительно протекающий саркоидоз печени может привести к развитию портальной гипертензии и цирроза печени [1].…”

“…В настоящее время нет стандартных протоколов лечения пациентов с саркоидозом печени [1]. Как правило, лечение включает глюкокортикостероиды и препараты урсодезоксихолевой кислоты (УДХК).…”

“…Случай из практики Введение Саркоидоз -это хроническая мультисистемная болезнь неизвестной этиологии, в основе которой лежит неспецифическое гранулематозное воспаление, нередко поражающее печень [1].…”