scholarly journals MON-340 Isolated Extrapulmonary Sarcoidosis Presenting as Symptomatic Hypercalcemia

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Janya Swami
Keyword(s):  
Vitamin D ◽  
Liver Biopsy ◽  
Mental Status ◽  
Calcium Absorption ◽  
Granulomatous Inflammation ◽  
Normal Serum ◽  
Unknown Etiology ◽  
Granulomatous Disease ◽  
Organ Systems ◽  
Sarcoidosis Present

Abstract Introduction: Sarcoidosis is a granulomatous disease of unknown etiology often involving multiple organ systems. Sarcoidosis most frequently affects the lungs, but in upto 30% of cases, can present with extrapulmonary manifestations. Less than 10% of patients with sarcoidosis present with disease at extrapulmonary sites. 10%-20% patients with sarcoidosis present with hypercalcemia. Hypercalcemia in sarcoidosis is secondary to increased intestinal calcium absorption due to increased levels of 1,25-dihydroxy Vitamin D. Clinical Case: 71-year-old Caucasian male presenting to his primary care physician with 3-week duration of fatigue, anorexia, mild confusion and unintentional weight loss was found to have moderate hypercalcemia and acute kidney injury. He was admitted the hospital for evaluation and management. Labs at admission revealed albumin-corrected calcium of 13.5 mg/dL (normal 8.5-11.0 mg/dL), creatinine of 1.78 mg/dL (normal 0.7- 1.3mg/dL) and alkaline phosphatase of 173 U/L (normal 45-117 U/L). Workup noted low PTH (3,normal 14-72 pg/mL), normal 25-hydroxy Vitamin D (50.7,normal 30-100 ng/dL), normal PTHrP and normal serum electrophoresis and immunofixation indicating non-PTH dependent hypercalcemia. 1,25-dihydroxy Vitamin D (125, normal 18-64 pg/mL) and ACE levels (159 U/L, normal 16-85 U/L) were elevated. Patient denied being on any Vitamin D supplements. A CT chest, abdomen and pelvis was done to look for occult malignancy and hepatosplenomegaly was noted with only mild compressive atelectasis of lungs. Abdominal ultrasound confirmed hepatomegaly with lobulated outer contour consistent with cirrhosis. Acute hepatitis, infectious and autoimmune work up was negative. Patient was discharged with mild improvement in calcium and mental status with hydration. Due to high suspicion for granulomatous disease, a liver biopsy was done. Liver biopsy confirmed granulomatous hepatitis with stage 2 of 4 fibrosis with numerous foci of non-caseating granulomatous inflammation. With negative acid-fast staining, no fungal organisms, absence of foreign material, normal eosinophil counts and low clinical suspicion for Crohn’s disease, a diagnosis of abdominal sarcoidosis was made. The patient was started on 10 mg prednisone daily and within one week, his albumin- corrected calcium levels improved to 10.4 mg/dL with significant improvement in appetite and mental status. Conclusion: While isolated extrapulmonary sarcoidosis is rare, it is an important cause of hypercalcemia due to elevated 1,25-dihydroxyvitamin D levels. Management of hypercalcemia secondary to sarcoidosis often consists of initiating glucocorticoids which act mainly by inhibition of 1,25-dihydroxy vitamin D synthesis in addition to inhibiting calcium absorption and osteoclast activity.

scholarly journals A Case of Sarcoidosis Disseminated to Skeletal Tissues

2013 ◽  
Vol 1 (1) ◽  
pp. 43-45
Author(s):  
Edon Rabinowitz ◽  
Chinwe Ogedegbe ◽  
Joseph Feldman
Keyword(s):  
Cell Tumor ◽  
Unknown Etiology ◽  
Granulomatous Disease ◽  
Presenting Symptoms ◽  
Organ Systems ◽  
Mediastinal Germ Cell Tumor ◽  
History Of ◽  
Hilar Adenopathy ◽  
Eye Lesions ◽  
Musculoskeletal Involvement

Sarcoidosis is a systemic granulomatous disease of unknown etiology that typically affects young adults. Diagnostic criteria for sarcoidosis include involvement of two or more of the following organ systems: 1) pulmonary infiltrates; 2) bilateral hilar adenopathy; and 3) skin and/or eye lesions. Musculoskeletal system is less commonly involved. For that reason potential presenting symptoms can vary and make the diagnosis very challenging; particularly if a patient has symptoms that mimic other conditions. Musculoskeletal involvement for example can mimic malignancy. The following case describes a patient with known history of primary metastatic mediastinal Germ Cell Tumor (GCT) with teratomatous elements who is diagnosed with sarcoidosis involving skeletal tissues.

scholarly journals Autoimmune Hepatitis and Sarcoidosis

2017 ◽  
Vol 03 (02) ◽  
pp. 124-126
Author(s):  
Shoaib Junejo ◽  
Sandeep Tuli
Keyword(s):  
Autoimmune Hepatitis ◽  
Granulomatous Inflammation ◽  
The Body ◽  
Unknown Etiology ◽  
African American Female ◽  
Smooth Muscle Antibody ◽  
Organ Systems ◽  
Systemic Disorder ◽  
Right Upper Quadrant Ultrasound ◽  
Noncaseating Granulomas

Abstract Background: Sarcoidosis is a systemic disorder of unknown etiology. It affects the organ systems in the body, and is characterized by the presence of noncaseating granulomas in the involved organs. The most common manifestation is bilateral hilar adenopathy along with skin, joint, and eye lesions. Here, we discuss an unusual manifestation of sarcoidosis along with autoimmune hepatitis (AIH). Case report: We report the case of a 33-year-old African American female presented with two-day history of progressive jaundice and deranged Liver function test (LFTs) the autoimmune work up was positive for anti-nuclear antibody (ANA) 1:640 and anti-smooth muscle antibody (SMA) 1:160, The serum Angiotensin converting enzyme (ACE) level was elevated 299 U/L; a right upper quadrant ultrasound revealed a mildly enlarged liver, subsequent CT chest and CT abdomen enlarged paratracheal and subcarinal adenopathy periportal edema in the liver and numerous small hypodense nodules in the spleen. Subsequent liver biopsy showed chronic, non-granulomatous, portal and parenchymal inflammation that was compatible with Autoimmune Hepatitis. Bronchoscopy with transbroncial biopsy revealed chronic, non-caseating granulomatous inflammation that was consistence with sarcoidosis. Thus the patient was diagnosed with sarcoidosis and AIH. The patient was started on Prednisone 60 mg daily, with subsequent reduction to 15 mg. The serum transaminase levels returned to normal level within three months. Conclusion: This case underscores the significance of association of Sarcoidosis that presented with Autoimmune Hepatitis. Physicians should be aware of this possibility so they may efficiently pick up the diagnosis after relevant investigations and start appropriate management. Timely intervention is key to treat the acute presentation and to prevent the further complications.

scholarly journals An Uncommon Cause of a Small-Bowel Obstruction

2017 ◽  
Vol 2017 ◽  
pp. 1-4
Author(s):  
Ali Zakaria ◽  
Bayan Al Share ◽  
Issam Turk ◽  
Samira Ahsan ◽  
Waseem Farra
Keyword(s):  
Small Bowel ◽  
Small Bowel Obstruction ◽  
Bowel Obstruction ◽  
Previous History ◽  
Granulomatous Inflammation ◽  
Giant Cells ◽  
Unknown Etiology ◽  
Granulomatous Disease ◽  
History Of ◽  
Noncaseating Granulomas

Sarcoidosis is a systemic granulomatous disease of unknown etiology, characterized by the formation of noncaseating granulomas. Gastrointestinal (GI) system involvement that is clinically recognizable occurs in less than 0.9% of patients with sarcoidosis, with data revealing small intestine involvement in 0.03% of the cases. A high index of suspension is required in patients presenting with small-bowel obstruction and previous history of sarcoidosis. Establishing a definitive diagnosis of GI sarcoidosis depends on biopsy evidence of noncaseating granulomas, exclusion of other causes of granulomatous disease, and evidence of sarcoidosis in at least one other organ system. Treatment of GI sarcoidosis depends on symptomatology and disease activity. Herein, we are presenting a case of 67-year-old female patient who had acute small-bowel obstruction at the level of jejunum with postoperative histopathologic evidence of noncaseating granulomatous inflammation with multinucleated giant cells, consistent with sarcoidosis.

scholarly journals Isolated Hepatic Sarcoidosis Presenting With Severe Hypercalcemia

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A204-A204
Author(s):  
Mc Anto Antony ◽  
Vipin Verma ◽  
Diego Bello ◽  
Ritika R Surabhi ◽  
Ravi Kant
Keyword(s):  
Vitamin D ◽  
Ct Scan ◽  
Mental Status ◽  
Serum Calcium ◽  
Oral Prednisone ◽  
Altered Mental Status ◽  
Unknown Etiology ◽  
Inflammatory Disorder ◽  
Thoracic Imaging ◽  
Hepatic Sarcoidosis

Abstract Introduction: Sarcoidosis is an inflammatory disorder of unknown etiology that can affect various organs. Lungs, intra thoracic lymph nodes and skin are the most commonly affected organs. The prevalence of hepatic sarcoidosis ranges between 5 to 30%. However, isolated hepatic sarcoidosis is rare. Hypercalcemia in sarcoidosis varies considerably due to the varying disease course and is reported to occur between 2 to 63% cases. We report a unique care of isolated liver sarcoidosis that presented with severe parathyroid hormone (PTH) independent hypercalcemia. Case: A 58 years old woman of Asian ethnicity with a past medical history of type 1 diabetes, hypothyroidism and chronic kidney disease presented to emergency department with headaches and altered mental status. The headaches were present since 4 weeks and was associated with polyuria and polydipsia. Non contrast CT scan of brain was negative for acute intracranial process. Physical examination revealed unremarkable vital signs and physical findings except for the altered mental status on neurological exam (orientation to self only). Initial laboratory testing revealed high corrected serum calcium 12.2 (8.0- 10.1 mg/dl), acute renal failure with high serum creatinine 2.90 (0.57–1.0 mg/dl), abnormal liver panel with elevated AST 84 (5-32U/L), ALT 85 (5–33 U/L), Alkaline Phosphatase 151 (35-104U/L). Repeat corrected serum calcium was still high at 12.4 mg/dl which prompted further evaluation to search for the etiology of hypercalcemia and testing revealed 1, 25 dihydroxy vitamin D (1, 25 vit D) mediated hypercalcemia. Labs showed low PTH 14.6 (15–65 pg/ml), normal serum protein electrophoresis, low PTH-related peptide <2.0 pmol/L, low 25 hydroxy vitamin D 20 (30–100 ng/ml) and high 1, 25 vit D 96.5 (19.9–79.3 pg/ml). Imaging evaluation revealed multiple hypodense nodules in both lobes of the liver seen on ultrasound, and CT scan of chest, abdomen and pelvis was unremarkable except for the similar liver findings. Biopsy of the liver lesion revealed non-caseating granulomas with no evidence for lymphoma and negative for acid fast bacteria and fungal organisms. Patient was treated with intravenous fluids, zoledronic acid and initiated on a course on oral prednisone that was tapered over a period of 6 months. There was a tremendous improvement in overall clinical condition. Serum calcium and 1, 25 vit D levels normalized. CT scan performed 3 months later showed complete resolution of all liver lesions. Conclusion: While most cases of hepatic sarcoidosis are asymptomatic and are incidentally found due to abnormal liver function tests or imaging done for other causes, we present a case of isolated hepatic sarcoidosis diagnosed due to symptomatic severe PTH independent hypercalcemia. Even though rare, extra-pulmonary sarcoidosis should be in differentials for 1, 25 vit D mediated hypercalcemia even if thoracic imaging are unremarkable.

OCCULT MALABSORPTION CAUSING OSTEITIS FIBROSA CYSTICA

1962 ◽  
Vol 40 (4) ◽  
pp. 481-492
Author(s):  
J. G. Devlin ◽  
D. K. O'Donovan
Keyword(s):  
Vitamin D ◽  
Iron Absorption ◽  
Calcium Absorption ◽  
Calcium Balance ◽  
Gluten Free ◽  
Osteitis Fibrosa Cystica ◽  
Mucosal Atrophy ◽  
Cystic Changes ◽  
Calcium Malabsorption ◽  
Radiological Examinations

ABSTRACT A case is reported of intermittent hypercalcaemia, hypophosphataemia and severe skeletal rarefaction with cystic changes. An occult calcium malabsorption was found. Fat, triolein and iron absorption and radiological examinations were normal. There was gross intestinal mucosal atrophy. She was shown to be in a state of negative calcium balance and this was reversed by vitamin D. A gluten-free diet also improved calcium absorption. It is postulated that the patient had severe secondary hyperparathyroidism as the skeleton reverted to normal after six months of medical treatment.

Calcium absorption and vitamin D in the chick

1951 ◽  
Vol 34 (1) ◽  
pp. 105-111 ◽  
Author(s):  
B.B. Migicovsky ◽  
A.M. Nielson
Keyword(s):  
Vitamin D ◽  
Calcium Absorption

The Temporal Distribution of the 1α,25-Dihydroxycholecalciferol Receptor in the Rat Jejunal Villus

1984 ◽  
Vol 67 (3) ◽  
pp. 285-290 ◽  
Author(s):  
S. D. H. Chan ◽  
D. Atkins
Keyword(s):  
Vitamin D ◽  
Vitamin D Deficiency ◽  
Calcium Absorption ◽  
Sedimentation Coefficient ◽  
Temporal Distribution ◽  
High Affinity ◽  
High Affinity Receptor ◽  
Affinity Receptor ◽  
Cell Fractions ◽  
Crypt Cells

1. The distribution of the 1α,25-dihydroxycholecalciferol receptor was studied in enterocytes isolated from the upper, mid and lower villus and crypt cells of the jejunum of normal and rachitic rats. 2. In all cell fractions a high-affinity receptor (KD ⋍ 0.07 nmol/l) with a sedimentation coefficient of 3.5S was demonstrated. 3. In normal rats there was a 60% reduction in receptor numbers in crypt cells compared with the mid and upper villous cells. 4. Vitamin D deficiency led to a reduction in receptor numbers in all cell fractions (45% upper villus, 78% crypt cells). 5. The data are compatible with the concept of calcium absorption occurring in the differentiated villous cells and also account for the reduction in absorption in rachitic animals.

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