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Key Clinical Message: We reported a patient with systemic lupus erythematosus complicated by livedoid vasculopathy (LV), who responded well to intravenous immunoglobulin and warfarin. Cutaneous lesions of LV resemble those of cutaneous vasculitis. LV should be included in the differential diagnosis of leg ulcerations even in the presence of autoimmune disorders. We reported a patient with systemic lupus erythematosus complicated by livedoid vasculopathy (LV), who responded well to intravenous immunoglobulin and warfarin. Cutaneous lesions of LV resemble those of cutaneous vasculitis. LV should be included in the differential diagnosis of leg ulcerations even in the presence of autoimmune disorders. [ABSTRACT FROM AUTHOR]

A 28-year-old man was admitted to our hospital because of reduced livido and increased fatigability. Four months before admission, he noticed polyuria, which was gradually relieved by admission.Magnetic resonance imaging revealed enhancing lesion centrally in the pituitary stalk. Biopsy fromthe skin revealed noncaseating granuloma composed of epithelioid cells, and a diagnosis of sarcoidosis was made. Although plasma arginine vasopressin (AVP) was undetectable after administration of hypertonic saline, urinary output was within normal range (1.5 to 2.2 L/day). The urine osmolality became above plasma levels during the hypertonic saline test. Hormonal provocative tests revealed partial glucocorticoid deficiency. Soon after the glucocorticoid therapy was begun, moderate polyuria (from 3.5-4.0 liters daily) occurred. At this time, plasma AVP was undetectable, and urine osmolality was consistently below plasma levels during the hypertonic saline test. In conclusion, we showed in human study that masked diabetes insipidus could be mediated by AVP-independent mechanisms. [ABSTRACT FROM AUTHOR]

Abstract: We evaluated the endocrinological outcomes of gamma knife radiosurgery (GKS) for the treatment of growth hormone (GH)-producing pituitary adenomas. Twenty-six patients treated with GKS (median treatment [tumour] volume: 0.8mL; median marginal radiation dose: 20Gy) were followed for a median of 84 months. “Disease remission” was defined as either nadir levels of GH <1μg/L during an oral glucose load, or random GH levels <2μg/L and normal age-adjusted and sex-adjusted levels of insulin-like growth factor without pituitary suppressive medications. The remission rate was 38% (10/26) and the 5-year and 10-year actuarial remission rates were 16.9% and 47.4%, respectively. Two patients (8%) suffered hypopituitarism requiring medication, but no other serious deficits were observed. Although GKS requires a relatively long time to achieve hormonal remission, it is a very useful, long-term treatment for GH-producing adenomas. We propose that compared to continuing life-long medication, GKS is less invasive and more cost effective. [Copyright &y& Elsevier]

To examine the glycemic response during exercise after administration of short-acting insulin lispro, we compared changes in plasma glucose concentrations during exercise performed by patients with diabetes after the administration of either insulin lispro or regular human insulin. Seven patients with diabetes (two with type 1 and five with type 2) participated in this study. Each of the insulin-depleted subjects received the same number of units of either insulin lispro or regular human insulin, delivered subcutaneously to the abdomen. The next day, each subject received a similar injection of the solution not previously administered. After each injection, the subjects were fed a standard meal of approximately 9 kcal/kg body weight. One hour after eating the test meal, the subjects performed 30 min of cycle ergometer exercise at 50% maximal oxygen uptake. Plasma glucose, insulin, glucagon, growth hormone (GH), and catecholamine concentrations were then measured at specific intervals. Insulin concentrations were higher and peaked earlier after administration of insulin lispro than after administration of regular human insulin. The length of time, needed to reach minimum plasma glucose concentration after exercise was begun, was significantly shorter after administration of insulin lispro, and the percentage of plasma glucose decrease induced by exercise relative to the peak concentration was significantly greater. No differences were found in the concentration changes of counterregulatory hormones between the insulin lispro data and the regular human insulin data. Compared with regular human insulin, insulin lispro induces a more rapid and greater decrease in plasma glucose concentration during exercise because of its faster absorption. [Copyright &y& Elsevier]

A 46-year-old woman was admitted to our hospital because of high fever and lumbago. CT revealed increase in density of fat tissue around the aorta, suggesting retroperitoneal panniculitis. The following day, she reported of leg pain, pain in the mouth, painful urination and right eye pain that was exacerbated by eye movement. We observed erythema nodosum, aphthous stomatitis and genital ulceration. Ophthalmologic examination revealed mild right optic disc oedema. Visual acuity was normal in both eyes. MRI with gadolinium infusion revealed enhancement around the right optic nerve, suggesting optic nerve perineuritis (ONP). A diagnosis of Behçet's disease was made. She was treated with prednisolone and colchicine with a favourable response. A warning sign of ONP is eye pain that is exacerbated by eye movement. MRI with gadolinium infusion should be performed immediately in such cases to prevent vision-threatening sequelae., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

A 53-year-old man was admitted to our hospital because of general fatigue and disorientation. He had been diagnosed with Bartter syndrome in his teens and had been taking potassium preparations since then. However, his serum potassium concentration (K + s ) remained persistently low. Ten days before admission, he developed fever. He was diagnosed as having bronchitis and was treated with antibiotics. Although his fever subsided, general fatigue worsened. Laboratory examination showed hyponatraemia (127 mEq/L), while K + s was 2.3 mEq/L. C reactive protein was negative. On admission, laboratory examination revealed deterioration of hyponatraemia (125 mEq/L). Although his serum sodium concentration (Na + s ) was refractory to electrolyte replacement, the level increased towards normal after spironolactone administration, following normalisation of K + s , suggesting that hyponatraemia was caused by K + depletion. Physicians should be aware of the importance of the effects of exchangeable K + (K + e ) on Na + s ., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

An 85-year-old woman was admitted to our hospital because of progressive hypoproteinemia and generalised oedema. Technetium-99m human albumin scintigraphy revealed protein leakage in the gastrointestinal tract. Upper gastrointestinal endoscopy revealed small whitish nodules from the gastric body up to the duodenal bulb. The urease test for Helicobacter pylori infection was positive. We diagnosed her as having protein-losing gastroenteropathy (PLGE) caused by H. pylori infection. The patient's hypoproteinemia and clinical symptoms promptly resolved after H. pylori eradication. Our results suggest that a trial of H. pylori eradication is warranted in patients with PLGE, even if endoscopy reveals neither giant rugal folds, erosion of the mucosa, nor polyposis, which are previously reported characteristic endoscopic findings of PLGE., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

A 56-year-old man was admitted to our hospital due to periodic episodes of acute pericarditis. These episodes occurred monthly along with a high fever and elevation of the C-reactive protein (CRP) level. The patient became afebrile and his CRP level decreased following the administration of a non-steroidal anti-inflammatory drug. A mutation analysis revealed the heterozygote of the familial Mediterranean fever (FMF) gene (E84K, G304R). This finding confirmed our diagnosis, and we treated the patient with colchicine. He responded to treatment and has been visiting our hospital without disease recurrence. FMF should be included in the differential diagnosis of repeated episodes of pericarditis.

A 50-year-old man was admitted to our hospital because of general malaise. Laboratory tests revealed severe hyponatremia (104 mEq/L), which was attributed to central adrenal insufficiency. To treat presumed central diabetes insipidus (CDI), we administered a small dose of hydrocortisone and gradually increased it to maintenance doses to prevent osmotic demyelination syndrome (ODS). Serum sodium levels did not increase more than 10 mEq/L/day and ODS did not occur. Thereafter, the patient was proven to have CDI. Incremental increases in glucocorticoid dose may reduce the risk of ODS for patients with hyponatremia due to central adrenal insufficiency, especially that complicated by CDI.

A case of Churg-Strauss syndrome complicated by chronic symmetrical dacryoadenitis suggestive of Mikulicz's disease is herein presented. A 72-year-old Japanese man, who had been previously diagnosed with asthma, presented with weakness of the left leg and purpura on the lower extremities. A neurological examination showed multiple mononeuropathies and a laboratory examination revealed elevated eosinophil counts, IgE levels and the presence of Myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCAs). Churg-Strauss syndrome was diagnosed, although the patient also exhibited bilateral swelling of the lachrymal glands. Furthermore, elevated serum IgG4 levels, an infiltration of a relatively large number of IgG4-positive plasmacytes in the nasal mucosa and hypocomplementemia were also observed. These findings were consistent with a diagnosis of Mikulicz's disease (MD). Oral prednisolone (30 mg) was administered and the swelling of the lachrymal glands resolved. Churg-Strauss syndrome may be accompanied by Mikulicz's disease (an IgG4-related disease), and common pathogeneses between Churg-Strauss syndrome and IgG4-related disease may exist.

A 23-year-old woman was admitted with complaints of swelling and pain in the left breast, fever, polyarthralgia and erythema nodosum. A fine-needle biopsy of the mass in the left breast revealed non-caseous granulomatous lobulitis. A diagnosis of granulomatous mastitis was thus made. The administration of prednisolone 40 mg/day resulted in the resolution of the patient's symptoms, and the breast mass thereafter decreased in size. The mass relapsed during the subsequent prednisolone taper. Additional therapy with methotrexate resulted in complete remission. Granulomatous mastitis should therefore be included in the differential diagnosis of polyarthralgia.

A 47-year-old man with Cushingoid appearance was admitted to our hospital showing excessive secretion of cortisol at intervals of a few days. Cyclic Cushing's disease was confirmed. Two years earlier, results of hormonal assessment had been within normal limits and macroadenoma had been incidentally detected in the pituitary. Dexamethasone suppression testing revealed various responses and a small lung tumor was detected, therefore his condition was misinterpreted as ectopic ACTH-producing tumor or pituitary cyclical Cushing's disease, leading to lung resection that confirmed cryptococcal pneumonia. The pituitary tumor was finally identified as the cause of cyclic Cushing's syndrome and fully removed, allowing remission.

A 37-year-old man was admitted to our hospital for precordial chest pain. He had taken prednisolone (5 mg/day) for systemic lupus erythematosus (SLE) and had been symptom free for the past 12 years. Echocardiography and contrast-enhanced CT of chest showed an enlarged ascending aortic aneurysm, which is rarely seen in SLE. Severe aortic regurgitation was also present, and surgical replacement of the ascending aorta and aortic valve was successfully accomplished by the Bentall procedure. Medial cystic necrosis in the ascending aorta, which is rarely seen in SLE angiopathy, was confirmed by histology. There were no significant histopathological findings in the aortic valve.

A 26-year-old man was hospitalized with a 1-month history of fever. Cervical lymph node biopsy showed necrosis in the paracortical area with abundant nuclear debris and proliferation of histiocytes. A diagnosis of histiocytic necrotizing lymphadenitis (HNL) (Kikuchi's disease) was made. He received methylprednisolone pulse therapy (MPT) (0.5 g/day for 3 days) without maintenance therapy and experienced dramatic improvement. We also used MPT for another 12 cases of HNL. All patients became afebrile within 1 day without adverse events. Four patients relapsed after the initial MPT, but only 1 patient relapsed during the following year. Our results suggest that MPT is warranted in HNL patients with prolonged fever.

A 53-year-old man was admitted to our hospital with thoracic back pain and weight loss. Computed tomography revealed inflammatory aortic aneurysm (IAA) of the descending aorta. Sealed rupture of the aneurysm occurred while the patient was under corticosteroid therapy. Endovascular aneurysm repair (EAR) was performed without postoperative complications. Periaortic fibrosis was remarkably decreased three months later while the patient was under prednisolone (20 mg) administration. We believe that EAR could become a practical alternative to open surgical repair. The possibility of an aneurysm rupturing during corticosteroid therapy for IAA should be considered.

A 59-year-old man with a history of prostate hyperplasia was admitted to our hospital for further examination of a lung mass and renal dysfunction. Lung biopsy specimens revealed that inflammatory cells had infiltrated into the blood vessel walls. We initially suspected lymphomatoid granulomatosis, but Epstein Barr virus-encoded small RNA was negative. However, 50% of the infiltrating plasma cells were positive for IgG4. Furthermore, the kidneys and prostate contained abundant IgG4-positive plasma cells. He was diagnosed with IgG4-related sclerosing disease even though serum IgG4 levels were not elevated (45.7 mg/dL). Prednisolone reduced the lung masses and ameliorated renal function, but the serum IgG4 level increased (377 mg/dL). Seronegative IgG4-related sclerosing disease should be considered when patients present with such symptoms and treatment responses, and the secretion of IgG4 might be blocked by its active synthesis.

Central diabetes insipidus (CDI) could occurs in patients with myelodysplastic syndrome (MDS) or acute myeloid leukemia (AML), because of infiltration of leukemic cells into the neurohypophysis or some other reason and it is closely associated with abnormalities of chromosome 7. We report a case of MDS with abnormalities of chromosome 7, presenting as CDI followed by deterioration of polyuria and hyponatremia with a decreased extracellular fluid volume. Magnetic resonance imaging (MRI) revealed symmetrically enhanced lesions in the hypothalamus. Fludrocortisone treatment normalized his serum sodium level and cerebral salt wasting syndrome (CSWS) was suspected.

A 76-year-old woman with a 5-mo history of recurrent diarrhea and generalized edema was admitted to our hospital. Colonoscopy revealed edematous mucosa, and histopathological examination was compatible with collagenous colitis. Protein leakage from the colon, particularly in the ascending portion, was identified on 99mTc-human serum albumin scintigraphy. Collagenous colitis associated with protein-losing enteropathy (PLE) without small bowel disease was diagnosed. Prednisolone treatment ameliorated diarrhea and hypoproteinemia. Collagenous colitis should be included in the differential diagnosis of chronic diarrhea with hypoproteinemia for appropriate management.

A 55-years-old man was admitted to our hospital with a 6-month history of general fatigue, purulent nasal discharge, polyuria, and polydipsia. Endocrinological findings revealed central diabetes insipidus (CDI) with mild anterior pituitary dysfunction. Imaging studies revealed thickening of the proximal end of the pituitary stalk just below the third ventricle, a mass in the paranasal sinus, and a mass encompassing the abdominal aorta. Histopathology of the mass in the paranasal sinus revealed abundant IgG4-positive plasma cells, and the IgG4 serum level was markedly elevated. Thus, he was diagnosed with IgG4-related multifocal fibrosclerosis. Therapy with prednisolone resulted in complete resolution of clinical symptoms and reduction in size of the masses in the affected organs. However, CDI remained unchanged. This is the first case in which the cause of CDI was IgG4-related multifocal fibrosclerosis. IgG4-related sclerosing disease should be included in the differential diagnosis of thickening of the pituitary stalk with CDI, and a search for extra-pituitary involvement is essential.

An 18-year-old man with 4-year history of central diabetes insipidus and partial pituitary dysfunction was admitted to our hospital because of headache and nasal discharge. Magnetic resonance imaging (MRI) revealed abnormal mass in the sella turnica invading into the cavernous sinus and sinus maxillaries along with thickened tentorium cerebelli. Histopathology of the mass in the sinus maxillaries revealed spindle-shaped cells arranged in a fascicular pattern with varied myxoid and collagenized regions with various inflammatory cells. Immunostaining revealed the spindle-shaped cells were positive for smooth muscle actin. These features were identical to those of inflammatory myofibroblastic tumor (IMT). He was diagnosed with IMT in the sella turnica and other regions. Corticosteroid therapy improved clinical symptoms and follow-up MRI revealed amelioration of the thickened tentorium cerebelli. However, the other lesions were unchanged and pituitary dysfunction did not improve. Although rare, IMT should be considered in the differential diagnosis of a sellar mass. Early treatment with corticosteroid therapy may reduce the risk of disease progression.

A 78-year-old woman was treated with 40 mg of prednisolone for microscopic polyangitis, and favorable effects were observed. However, her blood pressure increased and she developed severe thrombocytopenia. Thrombotic microangiopathy (TMA) due to malignant hypertension was suspected and she was treated with an angiotensin-converting enzyme inhibitor; her platelet count then rose. She showed a close temporal relationship between initiation of corticosteroid therapy and the onset of TMA. Corticosteroid therapy should be used with caution in patients with underlying vascular endothelial damage.

A 45-year-old man was admitted to our hospital because of bone pain and hypophosphatemia. He had undergone surgery 2 years previously for a "benign unclassified mesenchymal tumor" in the skull, but there were no clinical symptoms related to osteomalacia. His laboratory examination revealed low serum phosphate, high alkaline phosphatase, and normal calcium levels. The diagnosis of tumor-induced osteomalacia due to phosphaturic mesenchymal tumor mixed connective tissue variant (PMTMCT) was made by re-examining the pathologic specimens. Oral supplementation with phosphate and 1-25-dihydroxyvitamin D relieved his clinical symptoms and laboratory values returned to normal. However, subcutaneous administration of octreotide had no clinical effect. Clinicians and pathologists should be aware of the existence of PMTMCT especially nonphosphaturic or asymptomatic variants of this disorder.

A 70-year-old man who had been diagnosed with retroperitoneal fibrosis (RPF) was admitted to our hospital complaining of dyspnea. Imaging studies showed massive pericardial effusion. His condition deteriorated and pericardiostomy was performed. A biopsy of the pericardium revealed marked fibrosis with infiltration of lymphocytes, which was identical to RPF findings. A diagnosis of multifocal fibrosclerosis was made. Despite aggressive treatment, he died with clinical signs of cardiovascular failure. The autopsy specimen revealed proliferation of fibrosis with infiltration of lymphocytes in multiple organs. Even after successful decompression of urinary obstruction for RPF, long-term follow-up is necessary in these patients because of the possibility of other fatal complications such as pericardial fibrosis.

Chymase degrades angiotensin I (AI) to form angiotensin II (AII), probably constituting a bypass of the renin-angiotensin cascade. Chymase activity increases in some vascular diseases. In the kidney, an increase in chymase activity was reported in an animal model of ischemic kidney of renovascular hypertension (RVH); however, no such evidence has been provided in humans. We treated a 64-year-old patient with severe unilateral RVH and atherosclerosis, for whom removal of the ischemic kidney was the only option. Using immunohistochemical staining, we investigated chymase activity in the removed kidney and associated artery and vein. An increase in chymase activity, together with mast cells infiltrating the interstitium, was observed where interstitial fibrosis was seen. In the renal artery, where severe atherosclerosis was seen, and also in the vein, mast cell infiltration in the adventitia was accompanied by chymase. The captopril test showed an increase in serum aldosterone level, with a concomitant increase in plasma renin activity and decrease in blood pressure. Because the decrease in blood pressure implies a decrease in circulatory AII levels, it is plausible that in this patient, chymase had a role in AII formation in the adrenal gland to stimulate aldosterone secretion. Thus, by means of captopril, AI levels increased, and chymase may have produced AII in loci tissues, which, in turn, stimulated aldosterone secretion. This is the first report of an increase in chymase activity in the interstitium of an ischemic kidney and renal artery and vein in a patient with RVH and atherosclerosis.