Yoshifuji Matsumoto

Hypoperfusion of brain single photon emission computerized tomography in patients with antiphospholipid antibodies

To investigate cerebral lesions in patients with antiphospholipid antibodies only complaining of mild headaches, but without any neurological abnormalities or abnormal computerized tomography or magnetic resonance imaging findings, brain single photon emission computerized tomography (SPECT) using N-isopropyl-p-[123I] iodoamphetamine was employed as a sensitive method. Focal low perfusion areas and/or non-uniform radioisotope uptake could be shown on brain SPECT in all patients. Quantification of cerebral blood flow with a microsphere method revealed decreased cerebral blood flow. Hypoperfusion areas might be caused by microarterial thrombosis, microvenous thrombosis or vascular spasms. Early detection of cerebral abnormalities allows steps to be taken to protect against irreversible progress of cerebral blood flow. Therefore, brain SPECT should be performed in patients with antiphospholipid antibodies.

Reduced bone mineral density in Japanese premenopausal women with systemic lupus erythematosus treated with glucocorticoids

Abstract We evaluated bone mineral density (BMD) in Japanese female patients with systemic lupus erythematosus (SLE) and assessed the influence of the use of glucocorticoids. Lumbar BMD was measured by dual x-ray absorptiometry (DXA) in 60 premenopausal females who previously had been receiving glucocorticoid therapy. Therapeutic- and disease-related variables for SLE were analyzed and bone resorption or formation markers were measured. Osteoporosis was defined as a T-score below 2.5 SD by DXA; 12 patients (20%) showed osteoporosis, and 30 (50%) had osteopenia. Compared with the nonosteoporotic group (n = 48), the osteoporotic group (n = 12) had a significantly longer duration of glucocorticoid treatment (P = 0.01), a cumulative prednisolone dose (P = 0.002), and an SLE damage index (SLICC/ACR). There was no difference in the incidence of osteoporosis either with or without the previous use of methyl-prednisolone pulse or immunosuppressive drugs. There was a significant positive correlation between urinary type I collagen cross-linked N-telopeptides (NTx) and serum bone-specific alkaline phosphatase (BAP) (r = 0.404, P = 0.002), but these bone metabolic markers showed no difference between the osteoporotic and nonosteoporotic groups. A good significant negative correlation was shown between BMD and the cumulative glucocorticoid dose (r = −0.351, P = 0.007). Stepwise logistic regression analysis showed that the cumulative glucocorticoid intake was independently associated with osteoporosis. Glucocorticoid-induced osteoporosis was frequently observed in Japanese SLE patients, as in Caucasian populations. The cumulative glucocorticoid dose was associated with an increased risk for osteoporosis. Bone metabolic markers such as NTx and BAP were not influenced by glucocorticoid treatment and could not predict current osteoporosis in SLE patients.

Pulmonary AL amyloidosis in a patient with primary Sjogren syndrome

Abstract The condition of a 29-year-old woman with primary Sjögren syndrome (SS) was complicated by amyloid light chains- (AL-) type amyloidosis in the paranasal sinus. She had not complained of respiratory symptoms, but her chest computed tomography (CT) scan revealed bilateral multiple nodular shadows. Lung biopsy specimens using video-associated thoracoscopy showed amyloidoma in a subpleural nodular lesion and amyloid deposits in the interstitial parenchymal walls and pulmonary vessels. Pulmonary AL amyloidosis, presumably related to a chronic inflammatory lymphoproliferative process in SS, has rarely been reported.

Successful treatment of reactive hemophagocytic syndrome by plasmapheresis and high-dose γ-globulin in a patient with systemic lupus erythematosus.

Abstract A 31-year-old woman who had been administered corticosteroid and immunosuppressive agents for systemic lupus erythematosus (SLE) without flare-up was diagnosed as having reactive hemophagocytic syndrome (HPS) with severe disseminated intravascular coagulation. The causative underlying disease was uncertain, but it was not the SLE itself. Her fulminant HPS with increased serum ferritin and inflammatory cytokines (sIL-2R, TNF-α, IL-6, and IFN-γ) was successfully treated with plasmapheresis and high-dose γ-globulin therapy.

Lichenoid Eruption due to Mercaptopropionylglycine

Two patients with lichenoid eruption due to mercaptopropionylglycine (MPG) are reported. Case 1 was a 63‐year‐old woman, who developed a pruritic eruption after oral ingestion of MPG 600 mg/day for a month and a half. The clinicopathological findings were a lichenoid reaction. She also showed diffuse alopecia, which has not been reported as a side effect of this drug. Case 2 was a 71‐year‐old man who developed discoid lupus erythematosus‐like lesions after oral MPG 200 mg/day for two months, but the histological findings were a lichenoid reaction. In both patients, the lesions disappeared rapidly after discontinuing the drug. The eruption was reproduced by readministrating MPG in case 1. The skin reaction in both patients seemed to be the cell‐mediated immune reaction to MPG.

Seroprevalence ofHelicobacter pylori and association with atrophic gastritis in patients with Sjögren’s syndrome

To investigate the association betweenHelicobacter pylori (HP) infection and atrophic gastritis in Sjögren’s syndrome (SS), we conducted an age-matched case-control study examining serum HP-IgG antibodies and pepsinogen (PG) I and II levels using ELISA. The sera of 82 primary SS (1-SS), and 57 secondary SS (2-SS) were studied, as well as 198 controls having a diagnosis of connective tissue disease (CTD), except for SS which were obtained according to age. The titers of HP-IgG in 1-SS were significantly much higher than those in either 2-SS or control. The HP-IgG level revealed an exclusively positive correlation with the serum PG II level and a negative correlation with the PG I/II ratio. Serum PG II levels and PG I/II ratios were associated with the positivity of HP-IgG antibodies. The age-specific seroprevalence rates of HP infection in SS patients compared with controls showed a high positivity in patients less than 49 years old, but no difference among the higher age groups because of increasing positive rates with advancing age in the control. The matched odds ratio with HP infection in all SS (1-SS and 2-SS) and in 1-SS were 2.33 (95% CI: 1.43–3.81) and 2.75 (95% CI: 1.50–5.05), respectively. However, the positive PG I/II ratio did not show a statistically significant odds ratio for SS. We conclude that SS patients have a highly positive association with HP infection and that atrophic gastritis with SS may occur as a result of HP infection.

Lymphocytic interstitial pneumonia and non-caseating epithelioid granuloma in mediastinal lymph nodes in a patient with primary Sjögren’s syndrome

A 63-year-old woman diagnosed with Sjögren’s syndrome (SS) was indicated as having interstitial pneumonia with enlarged mediastinal lymph nodes. Lung tissue specimens showed lymphocytic interstitial pneumonia (LIP) and non-caseating epithelioid granulomas were recognized in only the mediastinal lymph nodes. Monoclonality was not detected using polymerase chain reaction of IgH rearrangement. The pulmonary lesions including lymphadenopathy were remarkably reduced by admistration of prednisolone. In this case, the sarcoid-like granulomas may have been associated with LIP, rather than co-existence of SS with sarcoidosis.