Pulmonary Calciphylaxis Associated with Acute Respiratory and Renal Failure Due to Cryptogenic Hypercalcemia: An Autopsy Case Report
A 40-year-old male patient was transferred to our hospital due to acute renal failure and hypercalcemia. Except for the eight weeks prior to admission, the patient had a history of good health. First, mild fever and generalized myalgia developed, and he was then admitted to a local general hospital with a drowsy mentality approximately two weeks later. With the impression of acute respiratory distress syndrome, support from a mechanical ventilator was administered immediately. The blood chemistry showed increased levels of urea nitrogen, creatinine, calcium, and ionized calcium in the serum. Increased levels of carbohydrate antigen 125 (CA-125) and parathyroid hormone (PTH)-related peptide were also observed. Other laboratory findings are shown in . Emergent hemodialysis was performed and pamidronate was administered for treatment of the hypercalcemia. Physical examination showed multiple scattered, violaceous, ulcerative, cutaneous lesions on both limbs, and no palpable lymph nodes were observed. High resolution chest computed tomography indicated diffuse ground-glass attenuation and calcified consolidation in both lungs, with diffuse thickening of bronchovascular bundles and pleural effusion. Computed tomography of the abdomen, brain, and spinal cord showed no evidence of tumors. Results of a bone marrow study showed normocellular marrow with trilineage hematopoiesis. Growth of a methicillin-resistant Staphylococcus aureus was observed on the sputum culture. Despite treatment with hydration, intravenous administration of furosemide and salcatonin, the serum calcium level showed a progressive increase, up to 17.4 mg/dL, and the patient went into cardiac arrest on the 40th hospital day. Cardiopulmonary resuscitation failed. A limited autopsy, except on the brain and spinal cord, was performed.\nOn opening of the thorax, a bilateral brownish pleural effusion of 300 mL was observed, and clear effusion of 100 mL was present in the pericardial sac. The thyroid gland and all of the four parathyroid glands were found to be normal and not enlarged. Enlarged mediastinal lymph nodes and periaortic lymph nodes were observed, with conglomeration up to 10 mm. The cut surfaces of the bilateral lungs were found to be heavy, firm, and brittle (). On opening of the peritoneal cavity, accumulated brownish ascites was observed. The mesenteric vessels were firm, and luminal calcification was observed on the cut surface. Coarse, thin bone marrow was taken from the iliac wing. The cut surface of the bilateral kidneys was brittle and showed no demonstrable hemorrhage or necrosis. Careful examination of the pelvic and abdominal cavity, including the gastrointestinal tract, found no gross abnormalities.\nHistologically, near complete destruction of the pulmonary architecture caused by calcium deposits (), which were stained using a von-Kossa stain, was observed. Calcification was observed in the alveolar septa, bronchus, bronchioles, and blood vessels. Diffuse calcific deposits, mainly in the glomeruli and interstitial spaces, were observed in the parenchyma of the kidney (). The skin necropsied from the violaceous extremity was characterized by total epidermal necrosis and calcification (calciphylaxis) was observed in small and medium-sized cutaneous vessels in the deep dermis and subcutaneous tissue (). The enlarged lymph nodes showed angiomatous changes, presumably caused by congestion and impaired local circulation due to vascular calcification, rather than direct calcium deposits.