Optic nerve perineuritis and retroperitoneal panniculitis: rare first presentations of Behçet's disease.

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.

Patient with Bartter syndrome in whom chronic potassium depletion was considered one of the causes of hyponatremia.

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.

Protein losing gastroenteropathy due to Helicobacter pylori infection without giant rugal folds, erosion or polyposis.

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.

Systemic lupus erythematosus with refractory ulcerated livedoid vasculopathy: Successful treatment with intravenous immunoglobulin and warfarin.

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.

Acute pericarditis as the first manifestation of familial Mediterranean fever: a possible relationship with idiopathic recurrent pericarditis.

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.

Pulmonary and gastric metastatic calcification due to milk-alkali syndrome: a case report.

The incidence of metastatic calcification is influenced by high serum calcium and phosphate concentrations and local physicochemical conditions, such as pH. A high pH accelerates tissue calcification. Patients with milk-alkali syndrome typically present with renal failure, hypercalcemia, and metabolic alkalosis, which are caused by the ingestion of calcium and absorbable alkali. Among patients with impairment of renal function, milk-alkali syndrome is a major cause of hypercalcemia. Long-term use of furosemide will lead to hypokalemia, metabolic alkalosis, and eventually renal failure (i.e., pseudo-Bartter syndrome). Even if the level of calcium ingestion is relatively low, the renal failure caused by long-term furosemide use can readily lead to milk-alkali syndrome. We describe a case of a 45-year-old woman who was admitted with cough and dyspnea and presented with pulmonary and gastric metastatic calcification. She had been taking alfacalcidol and oral alkaline medications such as sodium bicarbonate and calcium carbonate as well as oral furosemide for a long time. The patient was found to have hypercalcemia, chronic renal failure, and metabolic alkalosis, so milk-alkali syndrome was diagnosed. Saline was administered and oral medications were discontinued. Serum creatinine levels subsequently decreased, but pulmonary metastatic calcification was not diminished. In this case, the milk-alkali syndrome that caused the severe metastatic calcification was exacerbated by multiple factors, including oral alkaline medications such as sodium bicarbonate and calcium carbonate. In addition, metabolic alkalosis and renal failure were affected by long-term furosemide use (i.e., pseudo-Bartter syndrome).

A case of chronic active Epstein-Barr virus infection mimicking adult-onset Still's disease.

An 83-year-old man was diagnosed with adult-onset Still's disease (AOSD) based on clinical and laboratory findings. However, glucocorticoid had little effect. Epstein-Barr virus (EBV)-DNA was detected in peripheral blood, and autopsy findings confirmed a diagnosis of chronic active EBV infection (CAEBV). CAEBV mimics AOSD, and the presence of articular involvement and leukocytosis does not exclude the possibility of CAEBV. CAEBV should be included in the differential diagnosis of AOSD, and measurement of EBV-DNA is essential.

Granulomatous mastitis complicated by arthralgia and erythema nodosum successfully treated with prednisolone and methotrexate.

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.

Churg-Strauss syndrome concomitant with chronic symmetrical dacryoadenitis suggesting Mikulicz's disease.

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.

Pituitary cyclic Cushing's syndrome concomitant with solitary cryptococcal pneumonia confused with ectopic ACTH-producing tumor.

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.

Incremental increases in glucocorticoid doses may reduce the risk of osmotic demyelination syndrome in a patient with hyponatremia due to central adrenal insufficiency.

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.

Usefulness of a semi-quantitative procalcitonin test and the A-DROP Japanese prognostic scale for predicting mortality among adults hospitalized with community-acquired pneumonia.

BACKGROUND AND OBJECTIVE: The solid-phase immunoassay, semi-quantitative procalcitonin (PCT) test (B R A H M S PCT-Q) can be used to rapidly categorize PCT levels into four grades. However, the usefulness of this kit for determining the prognosis of adult patients with community-acquired pneumonia (CAP) is unclear. METHODS: A prospective study was conducted in two Japanese hospitals to evaluate the usefulness of this PCT test in determining the prognosis of adult patients with CAP. The accuracy of the age, dehydration, respiratory failure, orientation disturbance, pressure (A-DROP) scale proposed by the Japanese Respiratory Society for prediction of mortality due to CAP was also investigated. Hospitalized CAP patients (n = 226) were enrolled in the study. Comprehensive examinations were performed to determine PCT and CRP concentrations, disease severity based on the A-DROP, pneumonia severity index (PSI) and confusion, urea, respiratory rate, blood pressure, age ≥65 (CURB-65) scales and the causative pathogens. The usefulness of the biomarkers and prognostic scales for predicting each outcome were then examined. RESULTS: Twenty of the 170 eligible patients died. PCT levels were strongly positively correlated with PSI (ρ = 0.56, P < 0.0001), A-DROP (ρ = 0.61, P < 0.0001) and CURB-65 scores (ρ = 0.58, P < 0.0001). The areas under the receiver operating characteristic curves (95% CI) for prediction of survival, for CRP, PCT, A-DROP, CURB-65, and PSI were 0.54 (0.42-0.67), 0.80 (0.70-0.90), 0.88 (0.82-0.94), 0.88 (0.82-0.94), and 0.89 (0.85-0.94), respectively. The 30-day mortality among patients who were PCT-positive (≥0.5 ng/mL) was significantly higher than that among PCT-negative patients (log-rank test, P < 0.001). CONCLUSIONS: The semi-quantitative PCT test and the A-DROP scale were found to be useful for predicting mortality in adult patients with CAP.

Arginine vasopressin-independent mechanism of impaired water excretion in a patient with sarcoidosis complicated by central diabetes insipidus and glucocorticoid deficiency.

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 from the 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.

Onset of adult-onset Still's disease following influenza vaccination.

We describe that case of a 61-year-old woman who developed high spiking fever, sore throat, polyarthralgia, and salmon pink evanescent rash following influenza vaccination. A diagnosis of adult-onset Still's disease (AOSD) was made based on clinical and laboratory findings. Methylprednisolone pulse therapy followed by oral prednisolone resulted in a favorable outcome. This is the second published case in which a causal relationship between vaccination and onset of AOSD is suggested. Bystander activation would appear to play an important role in inducing the immune reaction.

A Case of Sarcoidosis with Unusual Radiographic Findings that Developed 5 Years after Silicone Augmentation Mammoplasty Complicated by Miliary Tuberculosis during Corticosteroid Treatment.

A 54-year-old woman with a past history of silicone augmentation mammoplasty was admitted with fever and dyspnea with diffuse interstitial shadows on computed tomography (CT). Although radiological findings were atypical, we diagnosed sarcoidosis by laboratory, microbiological, and bronchoalveolar lavage fluid analysis. Corticosteroids ameliorated the condition, but she had recurrent of fever and CT revealed miliary nodules while interstitial shadows disappeared. Liver biopsy showed that noncaseating granuloma and Ziehl-Neelsen stain was positive. We diagnosed miliary tuberculosis which developed during corticosteroid therapy. Antituberculotic therapy resulted in favorable outcome. Possibility exists that onset of sarcoidosis was induced by mammoplasty, namely, human adjuvant disease.

Treatment of histiocytic necrotizing lymphadenitis (Kikuchi's disease) with prolonged fever by a single course of methylprednisolone pulse therapy without maintenance therapy: experience with 13 cases.

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.

Aortic aneurysm with severe aortic regurgitation in a patient with systemic lupus erythematosus.

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 case of lymphomatoid granulomatosis-like lung lesions with abundant infiltrating IgG4-positive plasma cells whose serum IgG4 levels became high following the start of corticosteroid therapy.

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.