|IMAGES IN RHEUMATOLOGY
|Year : 2016 | Volume
| Issue : 4 | Page : 230-231
Sarcoidosis presenting as proximal myopathy in a patient with unexplained hypercalcemia
Department of Medicine, The Sarvajanik Medical Trust Hospital, Surat, Gujarat, India
|Date of Web Publication||8-Nov-2016|
Dr. Ankur Dalal
70, Sankalp Society, Near Jamana Nagar, Ghod Dod Road, Surat - 395 001, Gujarat
Source of Support: None, Conflict of Interest: None
Keywords: Hypercalcemia, Magnetic resonance imaging, HRCT, myopathy, sarcoidosis
|How to cite this article:|
Dalal A. Sarcoidosis presenting as proximal myopathy in a patient with unexplained hypercalcemia. Indian J Rheumatol 2016;11:230-1
A 48-year-old female presented with a history of weakness, sense of imbalance, and difficulty in standing up from sitting position for the last 3–4 months. She also gave a history of falls thrice in the same period. Recently, she has had multiple troubles including bilateral knee joint pain with swelling 3 months ago and renal dysfunction 1 month ago. She was under treatment for diabetes, hypertension, and hypothyroidism for the last 5 years. General examination was unremarkable. Systemic examination showed weakness with muscle power of grade 4+/5 around both hip girdles. Musculoskeletal examination was also unremarkable.
The patient had been extensively investigated during the last 3–4 months and was found to have unexplained hypercalcemia. On investigations, her serum calcium was 14.9 mg/dl (8.6–10 mg/dl) and 24 h urine calcium was 312.42 mg/day (100–300 mg/day on average 800 mg/day calcium in normal diet). Her serum phosphorus was 3.7 mg/dl (2.5–4.9 mg/dl) and serum parathyroid hormone 20 pg/ml (9.5–75 pg/ml). Her serum urea, serum creatinine, and serum potassium were 77 mg/dl (10-50 mg/dl), 2.7 mg/dl (0.6-1.4 mg/dl), and 4.1 mEq /L (3.5-5.5 mEq/L) respectively. Serum protein electrophoresis did not show paraproteinemia. A nerve conduction study showed bilateral symmetrical sensory polyneuropathy. Magnetic resonance imaging (MRI) of the brain showed age-related atrophy, and MRI of the spine showed only mild posterior bulging of L3–L4 and L4–L5 discs.
On further investigations, her hemoglobin and platelet count were within normal limits but total count was 13500/cumm (4000-11000/cumm) with 9% (1-6%) eosinophils. Her erythrocyte sedimentation rate (ESR) was elevated at 60 mm/h (3–12 mm/h). Muscle enzyme creatine phosphokinase was normal. Her antinuclear antibody by indirect immunofluorosence was +2 speckled but extractable nuclear antigen profile by immunoblotting was negative. Her serum angiotensin-converting enzyme (ACE) level was grossly elevated at 230 IU/L (20–70 IU/L). A chest X-ray showed minor fissure thickening on the right side. After careful analysis of history and investigation results, the patient was suspected to have myopathy related to sarcoidosis due to the presence of persistent bilateral lower-limb proximal muscle weakness, recent history of bilateral knee joint synovitis, eosinophilia, raised ESR, unexplained hypercalcemia, hypercalciuria, elevated serum ACE, and fissure thickening on chest X-ray. As of these, not a single feature was diagnostic of this condition; a MRI of both thighs was done. It showed diffuse bilateral muscle hyperintensity suggestive of myositis [Figure 1]. High-resolution computed tomography (HRCT) scan of the lungs showed ill-defined randomly distributed peribronchial nodules [Figure 2], suggestive of possible sarcoidosis. Muscle biopsy from the left vastus lateralis muscle showed noncaseating epithelioid granuloma, which was compatible with the clinical diagnosis of sarcoidosis.
|Figure 1: Diffuse bilateral muscle hyperintensity suggestive of myositis|
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The patient was treated with hydroxychloroquine and prednisolone (according to weight) along with treatment to comorbidities. She showed clinical improvement within 1 month, and her calcium level and renal dysfunction were also normalized.
Sarcoidosis is an inflammatory disease characterized by the presence of noncaseating granulomas. Respiratory complaints are the most common presenting symptoms followed by cutaneous and ocular. Hypercalcemia and/or hypercalciuria can occur in about 10% of sarcoidosis patients. The mechanism of abnormal calcium metabolism is increased production of 1,25-dihydroxyvitamin D by the granuloma itself which causes increased intestinal absorption of calcium, leading to hypercalcemia. Direct granulomatous bone and muscle involvement can be seen in only about 10% while direct kidney involvement in <5% of sarcoidosis patients. However, hypercalcemia is the most likely cause of sarcoidosis-associated renal disease in 1–2% of sarcoidosis patients.
Activity of serum ACE was found to be a useful tool in predicting the diagnosis of sarcoidosis. It is recommended that a serum ACE can be useful differential diagnostic marker at levels >100 IU/L or <52 IU/L in the diagnosis of sarcoidosis.
The case is presented due to two reasons:First, because direct muscle involvement is rare in sarcoidosis and presentation of this disease as myopathy is even rarer; second, sarcoidosis should be kept in mind as a differential diagnosis while dealing with unexplained hypercalcemia.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]