PMC6031209_01
Morphology of the plasma cells in a bone marrow smear (May-Giemsa staining).
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fig1
MRI/CT image of lower legs. (a) STIR (short tau inversion recovery) magnetic resonance image showing high-signal intensity areas in the lower leg muscles. These lesions had high DWI signals and normal ADC values. The muscle structure was intact. (b) Noncontrast enhanced computed tomography showing high density areas along the vessels.
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fig2
Diffusion-weighted and short tau inversion recovery magnetic resonance imaging revealed diffuse high signal intensity in the crural muscles (Figure 2(a)). CT showed widespread centrilobular opacities of both lungs (Figure 5(a)) and high-density lesions along small blood vessels in the trunk and extremities (Figure 2(b)), but the mediastinum, abdominal organs, and large vessels like the thoracic and abdominal aorta were intact.
Muscle biopsy of the right anterior tibial muscle (hematoxylin-eosin staining). Calcifications are observed in the vascular walls among the muscular bundles. Muscle fibers are slightly basophilic, and myophagia is also observed.
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fig3
A muscle biopsy was performed on the right tibial anterior muscles (Figure 3) and 40 mg/day prednisolone was prescribed by a neurologist because of suspected polymyositis/dermatomyositis. Next, a transbronchial lung biopsy was performed, and microscopy confirmed the presence of calcifications of the alveolar walls (Figure 5(b)) and of a small vessel in the right anterior tibial muscle (Figure 3).
Refractory ischemic skin ulcers of the right exterior thigh due to vascular calcification. This wound later reached the fascia.
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fig4
These ulcers gradually worsened (Figure 4) and the patient experienced severe pain, especially during dialysis or exercise.
(a) Computed tomography of the lungs. Calcifications were observed in both the lungs, especially in the upper fields. (b) Transbronchial lung biopsy specimen (hematoxylin-eosin staining). Basophilic fine calcific substances were observed along the alveolar wall.
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fig5
CT showed widespread centrilobular opacities of both lungs (Figure 5(a)) and high-density lesions along small blood vessels in the trunk and extremities (Figure 2(b)), but the mediastinum, abdominal organs, and large vessels like the thoracic and abdominal aorta were intact. Next, a transbronchial lung biopsy was performed, and microscopy confirmed the presence of calcifications of the alveolar walls (Figure 5(b)) and of a small vessel in the right anterior tibial muscle (Figure 3).
99mTc-hydroxymethylene diphosphonate scintigraphy. Accumulation in both of the upper lung fields was detected.
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fig6
A pulmonary function test demonstrated restrictive impairment with reduced diffusion capacity: the predicted forced vital capacity was 48.6%, the forced expiratory volume of the first breath was 87.9%, and the predicted diffusing capacity of lung for carbon monoxide was 67.1%. 99mTc-hydroxymethylene diphosphonate scintigraphy revealed abnormal diffuse accumulation in both upper lung fields (Figure 6).