Keigo Setoguchi

Serum IgG4 concentrations and IgG4-related sclerosing disease

BACKGROUND Based on histological and immunohistochemical examinations of various organs of patients with autoimmune pancreatitis (AIP), a new clinicopathological entity, IgG4-related systemic disease, was proposed. This study aimed to clarify clinical utility of serum IgG4 levels in differentiating AIP from other pancreatobiliary diseases, clinical utility of serum IgG4 levels in differentiating Mikuliczs disease from other salivary gland disorders, as well as in identifying other IgG4-related diseases. METHODS Serum IgG4 levels were measured in 468 patients. RESULTS The median serum IgG4 level was significantly greater in AIP (301.5mg/dl) than in other pancreatobiliary diseases (p<0.01). Using the cutoff value of 119 mg/dl that was determined on the basis of this studys ROC curve data, the sensitivity and specificity to distinguish AIP from pancreatic cancer were 82.1% and 94.8%, respectively. The median serum IgG4 level was significantly greater in Mikuliczs disease (357.0mg/dl) than in other salivary gland diseases (p<0.01). Of 75 patients with elevated serum IgG4 levels, 15 had diseases other than pancreatobiliary and salivary gland diseases. CONCLUSIONS Serum IgG4 levels were useful for diagnosing AIP and Mikuliczs disease. Some diseases with serum IgG4 level elevations may be lesions of IgG4-related systemic disease without manifestations of AIP and Mikuliczs disease.

Clinical Characteristics of Patients with Autoimmune Pancreatitis with or without Mikulicz's Disease and Mikulicz's Disease Alone

Background/Aims The objective of this study was to compare the clinical characteristics of patients with autoimmune pancreatitis (AIP) with or without Mikuliczs disease (MD) and with MD alone. Methods We investigated the clinical findings in 15 AIP patients with MD (group A+M), 49 AIP only patients (group A), and 14 MD only patients (group M). Results The male-female ratio was significantly higher in group A+M (73%, p<0.05) and group A (78%, p<0.01) than group M (21%). Serum immunoglobulin G (IgG) levels were significantly higher in group A+M than in group A (p<0.01) and group M (p<0.05). Serum IgG4 levels were significantly higher in group A+M than in group A (p<0.01). Other organ involvement was observed in 73% (11/15) of patients in group A+M. The number of patients with diabetes mellitus was significantly higher in group A+M (66%, p<0.01) and group A (51%, p<0.05) than in group M (7%). All of the patients responded well to steroid therapy, but the relapse rate in group A+M (33%) was significantly higher than that in group A (3%, p<0.01). Salivary gland function was impaired in all groups compared with the control group, but the degree of dysfunction was less in group A compared with group A+M and group M. Conclusions The relapse rate of AIP in MD patients was significantly higher than that of AIP in patients without MD.

Disseminated Mycobacterium abscessus Infection Following Septic Arthritis: A Case Report and Review of the Literature

AbstractMycobacterium abscessus is a rapidly growing mycobacterium found mainly in patients with respiratory or cutaneous infections, but it rarely causes disseminated infections. Little is known about the clinical characteristics, treatment, and prognosis of disseminated M abscessus infection.A 75-year-old Japanese woman who had been treated for 17 years with a corticosteroid for antisynthetase syndrome with antithreonyl-tRNA synthetase antibody developed swelling of her right elbow. X-ray of her right elbow joint showed osteolysis, and magnetic resonance imaging revealed fluid in her right elbow joint. M abscessus grew in joint fluid and blood cultures. She was diagnosed with a disseminated M abscessus infection following septic arthritis. Antimicrobial treatment by clarithromycin, amikacin, and imipenem/cilastatin combined with surgical debridement was administered. Although blood and joint fluid cultures became negative 1 week later, the patient died at 6 weeks from starting antimicrobial treatment.We reviewed 34 cases of disseminated M abscessus infections from the literature. Most of the patients had immunosuppressive backgrounds such as transplantation, use of immunosuppressive agents, hematological malignancy, and end stage renal disease. The duration from onset of symptoms to diagnosis was over 3 months in half of the cases. All fatal cases had positive blood cultures or use of immunosuppressive agents.Clinicians should bear in mind that mycobacterial infections including M abscessus are one of the differential diagnoses in patients with subacute arthritis and soft tissue infections.

Severe inflammation associated with synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome was markedly ameliorated by single use of minocycline.

We describe the case of a 63-year-old female who presented with severe inflammatory spondylitis, refractory to various antibiotics. Mycobacterial and fungal osteomyelitis were unlikely. Although asymptomatic, she also had osteomyelitis in the sternocostoclavicular region, and was suspected of having synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome, against which minocycline showed marked efficacy. The presence of severe inflammatory SAPHO, albeit rare, together with the marked efficacy of tetracycline, should be noted.

A case of severe bilateral lupus chorioretinopathy without major organ involvement

Dear Editor, Here we report a very rare case of severe bilateral lupus chorioretinopathy without other vital organ damage. A 65-year-old woman with systemic lupus erythematosus (SLE) continued to attend our hospital regularly since the diagnosis had been made at the age of 56 based on the presence of butterfly rash, photosensitivity, arthritis and positive antinuclear antibodies (ANA). Although she suffered from photosensitive eruptions every summer, she was generally in a stable condition, taking low-dose glucocorticoids. She began to feel general malaise and lost weight in autumn 2007. In July 2008, she suddenly experienced impaired eyesight in both eyes and almost 2 weeks later, she was diagnosed with bilateral serous retinal detachment and was hospitalized for close examination. Physical examination revealed that she had a normal blood pressure of 120/70 mmHg, and normal heart and lung sounds. She was neurologically intact and her intelligence level was maintained. She had a malar butterfly rash and photosensitive rash over her chest and arms, and polyarthritis. The major laboratory data revealed ANA at 1 : 320 with a speckled pattern and low complement level, represented by total hemolytic complement (CH50) at 16 U/mL (normal range: 30–47). In contrast, blood count, urinalysis and spinal fluid findings were normal and antiphospholipid antibodies were negative. The Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) was 16 (arthritis, low complement, skin rash, visual disturbance). Ophthalmologic investigation was further performed. No abnormal findings were observed in the conjunctivae, anterior and posterior sclera, anterior segment, lens and vitreous body. Visual acuity (VA) was 6/60 for the right and 12/60 for the left, accompanied by altered color vision. Visual field test was normal. Fundoscopy revealed a normal vasculature with no findings of exudates, cottonwool patch or hemorrhage. Mild papilledema was observed in the right, but the optic disc was normal in the left eye. Serous retinal detachment was not rhegmatogenous, and was observed extensively on the temporal side of the optic disc in the right eye, extending from below the disc to the proximal part of the superior temporal arcade, and the temporal margin involved the macular lesion. In the left eye, it was located temporal to the optic disc, and the macula was slightly involved (Fig. 1a). Fluorescein angiography (FAG) revealed multiple spots of leakage in the area and along the rim of the detached retina (Fig. 1b). Indocyanine green angiography (IA) revealed leakage and subretinal pooling of the dye in the late phase (Fig. 1c). Optical coherence tomography (OCT) revealed fluid collection above the retinal pigment epithelium (RPE) (Fig. 1d). These findings were consistent with lupus retinochoriopathy. However, as similar findings are also seen in Vogt-Koyanagi-Harada disease (VKH), we had to make a differential diagnosis. Our patient did not have inner ear or meningeal symptoms, nor did she have vitritis or iridocyclitis, which often accompanies posterior uveitis in VKH. Furthermore, she did not show the disease-characteristic human leukocyte antigen DR4 or DR53; thus we thought that a diagnosis of VKH was inappropriate for her. As she had been treated with glucocorticoids for a long period, central serous chorioretinopathy (CSC) was another differential diagnosis. However, the number of leakage spots in our patient was too numerous for CSC, in which only one or several spots are generally observed. Finally, taking into consideration the increased lupus activity, we reached a diagnosis of lupus chorioretinopathy. Methylprednisolone (MPSL) pulse was started, followed by high-dose oral prednisolone (PSL) at 45 mg/ day. The VA as well as color vision recovered completely (6/60–6/6 in the right and 12/60–6/6 in the left) and the SLEDAI also improved (16–0). Together with the improvement of fundus and FAG findings, International Journal of Rheumatic Diseases 2012; 15: e153–e155

Fallopian Tube Cancer with Palmar Fibromatosis or Fasciitis without Polyarthritis.

A 64-year-old Japanese woman had rapidly progressing bilateral palmar contracture associated with severe pain on both palms over the previous 8 weeks, without a history of arthritis in any joints. We suspected palmar fibromatosis or fasciitis without polyarthritis. Because palmar fibromatosis is known to be associated with cancer, we performed cancer screening and the patient was subsequently diagnosed with fallopian tube cancer. This is the first case report of palmar fibromatosis or fasciitis without polyarthritis associated with fallopian tube cancer. The characteristic rapid progression of palmar contracture is a key finding that suggests the potential existence of a malignancy.

Usual interstitial pneumonia following resolution of cavitated pulmonary masses in a patient with ANCA-associated vasculitis

A 72‐year‐old woman with slight pulmonary interstitial reticular markings was initially diagnosed with microscopic polyangiitis (MPA). Two years later, cavitated pulmonary masses appeared, and a biopsy specimen revealed granulomas. Granulomatosis with polyangiitis (GPA) was diagnosed. The masses resolved with treatment. Ten years later, the usual interstitial pneumonia (UIP) pattern appeared on chest computed tomography (CT). The diagnosis of lung toxicity from methotrexate (MTX) or cyclophosphamide (CYC) was precluded by the clinical course. Despite treatment with prednisolone (PSL), the UIP progressed. The change of pulmonary pathology from masses to UIP is rare in patients with GPA.

Autoimmune pancreatitis that developed over a 3-month observation period for IgG4-related dacryoadenitis and sialadenitis

Autoimmune pancreatitis (AIP) that developed in a short period in a patient with IgG4-related dacryoadenitis and sialadenitis is reported. A 67-year-old man was diagnosed as having IgG4-related dacryoadenitis and sialadenitis histologically, serologically, and radiologically. At that time, the pancreas was normal on computed tomographic scanning, magnetic resonance imaging, and 18F-fluorodeoxyglucose positron emission tomography. However, AIP occurred in the pancreatic body and tail during the 3-month period of observation of the initial diseases. All IgG4-related lesions improved promptly after steroid therapy. This report emphasizes that, since IgG4-related disease can develop in other organs within a short period in a patient with IgG4-related disease, periodic imaging follow-up is necessary.