MAMC Journal of Medical Sciences

: 2015  |  Volume : 1  |  Issue : 3  |  Page : 164--166

Isolated abdominal sarcoidosis presenting with hypercalcemic crisis: A rare case

Nidhi Anand, Sunita Aggarwal, Mradul Kumar Daga, Alpana Meena 
 Department of Medicine, Maulana Azad Medical College, New Delhi, India

Correspondence Address:
Nidhi Anand
Maulana Azad Medical College, Bahadur Shah Zafar Marg, New Delhi


Sarcoidosis is a granulomatous disease of unknown origin, with pulmonary findings in more than 90% of patients. Extrapulmonary involvement is common and all organs can be involved (especially lymph nodes, eyes, joints, and central nervous system) but it is rare to find an isolated extrapulmonary disease (<10% of patients). Granulomatous inflammation of the spleen and the liver is common in patients with systemic sarcoidosis while hepatosplenomegaly is unusual and splenic involvement rare. We are describing a rare case of abdominal sarcoidosis presenting with the hypercalcemic crisis but no pulmonary involvement.

How to cite this article:
Anand N, Aggarwal S, Daga MK, Meena A. Isolated abdominal sarcoidosis presenting with hypercalcemic crisis: A rare case.MAMC J Med Sci 2015;1:164-166

How to cite this URL:
Anand N, Aggarwal S, Daga MK, Meena A. Isolated abdominal sarcoidosis presenting with hypercalcemic crisis: A rare case. MAMC J Med Sci [serial online] 2015 [cited 2020 Feb 27 ];1:164-166
Available from:

Full Text


Sarcoidosis commonly presents with pulmonary involvement. It can also involve other organ systems, but isolated extrapulmonary sarcoidosis is less commonly seen. Furthermore, asymptomatic hypercalcemia occurs in about 11% cases of sarcoidosis, but clinically significant hypercalcemia is a rare presentation. In our case, the patient had hypercalcemia with isolated hepatic sarcoidosis. Although there is one case report in the literature with hepatosplenic involvement, but no association with hypercalcemia was seen, which makes this presentation in our case an unusual one.

 Case Report

A 35-year-old female presented with a history of low-grade intermittent fever associated with diffuse abdominal pain, vomiting, decreased appetite, and significant weight loss for 6 months and altered behavior since 2–3 days. Glasgow Coma Scale was 13/15. There was no history of altered bowel habits, melena, hematemesis, jaundice, cough, sputum or hemoptysis, use of tobacco, alcohol, or any other recreational drug. There was no history of renal calculi in the past. Family history was not significant for the presence of any malignancy or tuberculosis. There was no history of seizures or focal neurological deficit. On physical examination, she was cachexic in appearance, hemodynamically stable with no cyanosis, pallor, icterus, clubbing, or lymphadenopathy. The systemic exam revealed hepatomegaly measuring approximately 2–3 cm below right costal margin with a palpable spleen. Central nervous system examination revealed no abnormality except for altered behavior. Fundoscopic examination was normal and there was no rash or any other lesion on dermatological examination. Laboratory investigations showed a total leucocyte count - 10290/mm 3, platelet count - 3.7 lac/cc, alkaline phosphate - 194 (33–96 U/L), serum calcium - 16.4 (8.7–10.2 mg/dl), serum phosphate - 2.7 (2.5–4.3 mg/dl), total protein - 7.5 (6.7–8.6 g/dl), serum albumin - 2.69 (4–5 mg/dl) (reversed A: G ratio), serum lactate dehydrogenase - 899 (115–221 U/L), serum uric acid - 5.4 (2.5–5.6 mg/dl), serum parathyroid hormone - 7.78 (8–51 pg/ml), and serum Vitamin D3 levels - 77 (36–180 pmol/L). Kidney and liver function tests were within normal range. [Table 1] shows patients hemoglobin, ESR, Serum Calcium and CT findings before and after treatment.{Table 1}

On the basis of history and investigation, differential diagnosis was multiple myeloma, malignancy (unknown primary), disseminated tuberculosis, and sarcoidosis. Bone marrow biopsy and aspiration were suggestive of a focal increase in plasma cells (<8%). Serum electrophoresis was normal with no "M" spike. Ultrasonography of abdomen showed hepatosplenomegaly. A computed tomography of the chest-abdomen-pelvis revealed hepatosplenomegaly with multiple, focal hypodense lesion [Figure 1] with normal lung parenchyma and mediastinum. Ultrasound-guided biopsy of the liver was performed. Histopathology evaluation of the biopsy specimen revealed well formed, large, noncaseating granulomas seen composed of epithelioid cells and langerhans type giant cells [Figure 2] with stain for acid-fast bacilli negative.{Figure 1}{Figure 2}

She was managed for hypercalcemia initially with intravenous fluids. Later she was started on systemic steroids (prednisolone 1 mg/kg/day) which was continued for 6 weeks and thereafter tapered. The patient showed marked improvement in the laboratory and clinical parameters [Figure 3]. On follow-up, the patient is asymptomatic, but she developed steroid-induced diabetes after 4–5 months of therapy.{Figure 3}


The most common site of involvement in sarcoidosis is lungs. Other organ systems that can be involved are lymph nodes, especially the nodes of the thorax, skin, eyes, liver, heart, nervous, and musculoskeletal system. Prevalence estimates of sarcoidosis range from <1 to 40 cases per 100,000 population.[1] It can occur with unusual presentations such as splenic or hepatosplenic involvement. It is also possible that there is an exclusive involvement of these organs without pulmonary disease. Imaging findings are often nonspecific, and, in cases of isolated abdominal organ involvement, a diagnosis of sarcoidosis can be achieved only by revealing noncaseating granulomas in tissue specimens and excluding other causes of granulomatous inflammation.[2] In the literature, similar findings were shown in a rare case of isolated granulomatous disease of the spleen diagnosed and treated laparoscopically, and two cases of unusual presentation of systemic sarcoidosis that had onset with early nodular hepatosplenic and splenic lesions, respectively.[3] Asymptomatic hypercalcemia occurs in about 11% cases of sarcoidosis, but clinically significant hypercalcemia is a rare presentation in this disease.[4] There are two case reports in literature where hypercalcemia was the initial presentation with subsequent mediastinal lymph node biopsy showing evidence of sarcoidosis.[4] However, no study of hypercalcemia in association with hepatic sarcoidosis has been found in the literature until now as described in our case.

Atypical sarcoidosis without pulmonary symptoms often poses a diagnostic challenge to the clinicians. In our patient, hypercalcemia was found with a suppressed parathyroid hormone levels suggesting that the cause of hypercalcemia was not parathyroid hormone dependent. Hypercalcemia can give us a clue to the diagnosis, and atypical sarcoidosis should be kept as a possibility even when not associated with pulmonary symptoms or a normal chest X-ray.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.


1American Thoracic Society. Statement on sarcoidosis. Am J Respir Crit Care Med 1999;160:736-55.
2Tana C, Dietrich CF, Schiavone C. Hepatosplenic sarcoidosis: Contrast-enhanced ultrasound findings and implications for clinical practice. Biomed Res Int 2014;2014:926203. doi:10.1155/2014/926203.
3Giovinale M, Fonnesu C, Soriano A, Cerquaglia C, Curigliano V, Verrecchia E, et al. Atypical sarcoidosis: Case reports and review of the literature. Eur Rev Med Pharmacol Sci 2009;13 Suppl 1:37-44.
4Karnchanasorn R, Sarikonda M, Aldasouqi S, Gossain VV. Severe hypercalcemia and acute renal failure: An unusual presentation of sarcoidosis. Case Rep Med 2010;2010:423659. doi: 10.1155/2010/423659. Epub 2010 Dec 16.