ATYPICAL PRESENTATION OF SARCOIDOSIS AT AN ATYPICAL AGE
Chest(2023)
摘要
SESSION TITLE: Diffuse Lung Disease Case Report Posters 13 SESSION TYPE: Case Report Posters PRESENTED ON: 10/09/2023 02:10 pm - 02:55 pm INTRODUCTION: Sarcoidosis is commonly diagnosed in young individuals (between 20 and 60 years) but according to recent studies more than half the cases are diagnosed after 40 years of age. [1] Very late diagnosis of sarcoidosis, after 75 years of age is not common. We present a 79-year-old female with a new diagnosis of hypercalcemia due to new diagnosis of sarcoidosis. CASE PRESENTATION: A 79 year old female with a past medical history of hypertension, GERD, dysphagia due to esophageal stricture s/p multiple dilations, and Diabetes type 2 who presented to the hospital for abnormal labs from primary care office. She was found to have hypercalcemia (14.2 - ionized 7.0) and elevated liver function tests (LFTs). Workup was initiated and IV fluids were ordered. During the first two days of hospitalization, her LFts rapidly improved. calcium improved with IV fluids to 10.6 but corrected calcium remained elevated at 12.1 after correction for albumin of 2.1. PTH was appropriately suppressed. PTHrP was normal. Elevated 1,25 Vit D and Low 25 OH vitamin D were also noticed. ACE levels were also found to be elevated. Extensive workup was negative including beta glucan, urine histoplasma, cryptococcal antigen, Coccidiodes, viral hepatitis panel, ANA, QuantiFERON gold. Serum protein electrophoresis (SPEP) had revealed low albumin, low total protein, low beta 1 globulin with normal gamma globulin levels. Urinary protein electrophoresis was unremarkable. TSH was also normal. CXR was normal with bibasilar atelectasis. CT chest without contrast showed bilateral small pleural effusion with possible superimposed aspiration and passive atelectasis. On review of the chart, patient had not had hypercalcemia before. Lysozyme was elevated.Patient reported a lump in the back. CT Lumbar spine was done for the non-tender lump in the lumbar para-spinal region and showed nonspecific thickened and partially calcified soft tissue within the lumbar subcutaneous fat extending from T12 to L5. She had IR guided lumbar paraspinal soft tissue biopsy remarkable for granulomatous inflammation, fibrosis with calcification. Rheumatology was consulted with suspicion of sarcoidosis with negative otherwise workup.Hypercalcemia, granulomatous soft tissue lesions, elevated ACE, lysozyme, with negative malignancy, fungal/bacterial/mycobacterial infectious workup, negative ANCA, negative antifungal serologies, all supported the diagnosis of sarcoidosis. Steroids were started in the hospital with improvement in calcium levels noticed. The patient was discharged with outpatient rheumatology follow up for methotrexate initiation and outpatient bone scan. DISCUSSION: Sarcoidosis is classified based on organ involvement. About 2-63% of patients with sarcoidosis are found to have hypercalcemia which is related to unmonitored macrophage synthesis of 1,25 dihydroxy-vitamin D-3 leading increased bone resorption and increased calcium absorption in the intestines. [2] Elevated calcium is a rare initial presentation of hypercalcemia. [3] Glucocorticoids given for symptomatic hypercalcemia in sarcoidosis inhibit 1-alpha-hydroxylase hyperactivity in macrophages.[2] CONCLUSIONS: Sarcoidosis can present very late in age and can have an atypical presentation such as hypercalcemia. It must be kept in the differential in patients outside typical diagnostic age. REFERENCE #1: Baughman RP, Field S, Costabel U, Crystal RG, Culver DA, Drent M, Judson MA, Wolff G. Sarcoidosis in America. Analysis Based on Health Care Use. Ann Am Thorac Soc. 2016 Aug;13(8):1244-52. doi: 10.1513/AnnalsATS.201511-760OC. PMID: 27509154. REFERENCE #2: Ackermann D. Die Hyperkalzämie im Verlauf der Sarkoidose--Fallbeispiel, Prävalenz, Pathophysiologie und Therapiemöglichkeiten [Hypercalcemia in sarcoidosis--case report, prevalence, pathophysiology and therapeutic options]. Ther Umsch. 2007 May;64(5):281-6. German. doi: 10.1024/0040-5930.64.5.281. PMID: 17685088. REFERENCE #3: Mulkareddy V, Bhalla V, Upadhye S, Siddam P. The Diagnostic Dilemma of Sarcoidosis: A Case of Acute Hypercalcemia. Cureus. 2020 Sep 11;12(9):e10399. doi: 10.7759/cureus.10399. PMID: 33062519; PMCID: PMC7549992. DISCLOSURES: No relevant relationships by Haris Bilal No relevant relationships by Naira Saleem No relevant relationships by Usama Talib No relevant relationships by Haris Zia
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