Kiyofumi Hagiwara

Extremely high salivary β2‐microglobulin and Na+ levels in a Sjögren syndrome patient

Dear Editor, Extremely high levels of salivary b2-microglobulin (b2MG) and Na + were observed in a Sjögren syndrome (SS) patient. This would be the first case report that shows the most extreme sialochemistries and their changes after steroid therapy. A 49-year-old woman had recurrent dental caries, angular cheilosis and angular conjunctivitis since her grade-school years. Eight years ago, keratoconjunctivitis sicca was diagnosed. Four years previously, she developed meningo-encephalitis, which was treated with steroidal and antiviral drugs. After the steroid dose was tapered, her keratoconjunctivitis exacerbated and bilateral corneal opacification appeared. Neither punctum plug insertion nor keratoplasty in one eye gave improvement and thus she visited our hospital. Little white fila on both corneas and dry tongue with atrophic taste buds were observed. The major laboratory findings were confined to salivary gland and kidney involvement. Her estimated glomerular filtration rate (eGFR) had decreased from 101 to 42.5 mL/min during these 4 years. After admission, eGFR deteriorated further to 28.4 mL/min and serum creatinine (SCr) levels altered from 1.12 mg/dL to 1.62 mg/dL. Urinalysis showed alkaline urine of pH 7.5 and increased levels of b2MG and N-acetyl glutamate (NAG) of 11 321 lg/L and 14 IU/L, respectively, without glucosiuria, proteinuria, hematuria or any sediment. Antinuclear, anti-SS antigen A/Ro, anti-SS antigen B/La antibodies and rheumatoid factor were not detected. Peripheral blood white blood cell and lymphocyte counts were 4500/lL and 1440/lL, respectively. Neither hypocomplementemia nor cryoglobulinemia were present. Serum C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) were slightly increased. Sialometry for whole saliva showed 0.5 mL/ 30 min flow, and sialochemistry revealed 32.8 mg/L of b2MG, 115 mmol/L of Na , 32.5 mmol/L of K and 127 mmol/L of Cl. Both Schirmer’s test and Rose-Bengal staining showed positive at both eyes. Salivary scintigraphy showed low accumulation in both parotid and submandibular glands with no excretion response to acid stimulus. Sialography showed sialiectasis in a parotid gland. The biopsied minor salivary gland in the lower lip demonstrated dense infiltrates of mononuclear cells (Fig. 1). Sustained alkaline urine with high b2MG and NAG levels suggested active interstitial nephritis. Her disease met the criteria of the American-European Consensus Group SS Classification Criteria. The European League Against Rheumatism (EULAR) Sjögren syndrome disease activity index (ESSDAI) was 5 points. Prednisolone at 0.8 mg/kg per day (32 mg/day) was administered for 4 weeks and then tapered. The patient’s dry eyes and dry mouth got milder, and angular cheilosis and conjunctivitis disappeared. Both sialometry and sialochemistry soon showed obvious improvement (Fig. 2). Salivary flow rate increased to 2 mL/10 min, and salivary b2MG, Na , K and Cl levels decreased to 9.0 mg/L, 37.0, 24.9, and 44.0 mmol/ L, respectively, after 6 weeks (Fig. 2). The deterioration of renal function also reversed as SCr levels decreasing to 1.16 mg/dL and eGFR increasing to 40.9 mL/min. Immunoglobulin G (IgG), IgA and IgM levels decreased from 1693 to 1052, 285 to 119, and 100 to 42 mg/dL, respectively and both CRP and ESR returned to normal. ESSDAI decreased to 1 point. She was still well after tapering prednisolone dosage to 5 mg/day. The most novel finding observed in this patient is that salivary b2MG level was as high as 6 times of that in the serum, and Na level was as high as near the serum level. The salivary b2MG and Na + levels in normal individuals have been scantily reported and vary. In one report, parotid saliva collected for 10 min showed b2MG level of 0.77 mg/L. 3 As for salivary Na, whole saliva collected for 10 min was 4.9 0.48 mmol/L. Our SS patient in this report showed the highest levels of both b2MG and Na + to appear in the literature. International Journal of Rheumatic Diseases 2012; 15: e31–e33

Clinical Images: Arytenoid Chondritis

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SAPHO Syndrome: Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis

A 68-year-old woman with a history of breast cancer, who had undergone left quadrantectomy 24 years prior, had suffered from palmoplantar pustulosis (PPP) and pain in multiple joints and bones for 3 years. Technetium bone scanning showed increased uptake in the sternum, sternocostoclavicular joint, cervical vertebrae, lumbar vertebrae, and right knee (Picture 1, left). A radiograph of the lumbar spine showed osteosclerotic vertebral lesions and paravertebral hyperostosis (Picture 2, left). Coronal reformatted images of computed tomography (CT) of the lumbar spine revealed inflammatory spondylodiscitis with irregularity and erosions in the endplates, osteosclerotic vertebral lesions, and paravertebral hyperostosis (Picture 2, right). A diagnosis of synovitis,

Synovial Chondromatosis of the Suprapatellar Pouch of the Knee in a Patient with Systemic Lupus Erythematosus

Synovial chondromatosis is an uncommon benign neoplastic disease that mainly affects men in the third to fifth decades of life. Secondary synovial chondromatosis is considered to be associated with slowly progressive degenerative joint disease, including trauma and neurological disease1. Systemic lupus erythematosus (SLE) is not related to the etiological factor. A 75-year-old woman with a history of SLE and schizophrenia presented with a complaint of right knee …

Clinical experience in 115 patients with arthritis and/or enthesitis who met the classification criteria for psoriatic arthritis (CASPAR) within the last two years-Possible association with malignant disorders.

Abstract Among about 400 patients with active arthritis and/or enthesitis who were referred to our department within the last two years, 140 of them were strongly suspected as having psoriatic arthritis by a comprehensive diagnostic procedure and after consulting specialists from dermatology, orthopedics, and radiodiagnostics at our institution and other institutions. Among them, 115 patients strictly met the classification criteria for psoriatic arthritis (CASPAR). Among the 115 patients, 19 patients (9 males and 10 females) had current psoriasis and 96 patients (22 males and 74 females) did not have current psoriasis. Nineteen (16.5%) of the 115 patients had developed malignant tumor before the onset of arthritis, and 4 (3.5%) developed malignant tumor after the onset of arthritis. Twenty-two of the 23 patients who developed malignancy were female and 10 patients developed breast cancer. Differential diagnoses in these 23 patients may include paraneoplastic syndrome. We consider that it is important to take into account the possibility of paraneoplastic syndrome in patients with arthritis and/or enthesitis who apparently meet the CASPAR criteria, and detailed screening and monitoring of malignant disease may be beneficial to the patients.

580 Eleven Cases of Angioedema with Eosinophilia Treated in a Single Hospital in Japan.

Background Angioedema with eosinophilia (AE) is mostly reported in Japanese patients, and only as case reports. In this study, we aimed to determine the prevalence, clinical and laboratory characteristics, and courses of AE; the therapies for AE and the outcomes; and to evaluate whether steroid therapy for AE is necessary or not. Methods The patients whose blood samples showed an eosinophil count of ≥2000/&mgr;L, among the samples tested for blood cell counts and differential counts between Jan. 2006 and Oct. 2010, in Japanese Red Cross Medical Center, were first included. Among these patients with AE were extracted. The AE diagnosis was based on angioedema developing concurrently with eosinophilia and improving with the recovery from eosinophilia. Results All of the 11 patients were Japanese young females. One patient with clear arthralgia showed radioisotope accumulation in the joints by bone scintigraphy, and was diagnosed as having arthritis. The peak peripheral blood eosinophil count was 7,839 ± 6,008 (2,130–23,170)/&mgr;L after visiting our hospital. An increase in white blood cell count was only due to an increase in eosinophil count. Serum C-reactive protein and Immunoglobulin E levels remained almost normal. Peripheral blood eosinophil count decreased steadily for 8 weeks after the first visit, regardless of steroid use. Edema in all of the patients and arthralgia in 6 patients improved within 12 weeks. None of the patients had a recurrence of AE. Conclusions AE developed in Japanese young females and likely showed a single course. In AE, the count of eosinophil of 104/&mgr;L was observed. Only eosinophil count increased without changes in changes in the counts of other leukocyte series. Serum C-reactive protein and Immunoglobulin E levels remained almost normal. The eosinophil count in AE patients will return to the normal level within 8 weeks even without steroid therapy.