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.

MMP-3 can distinguish isolated PMR from PMR with GCA: A retrospective study regarding PMR and GCA in Japan.

OBJECTIVE: We sought to identify clinical features at diagnosis that can distinguish isolated polymyalgia rheumatica (PMR) without giant cell arteritis (GCA) from PMR with GCA, and clinical features at diagnosis of isolated PMR that can predict subsequent relapse and corticosteroid discontinuation. METHODS: A retrospective study of 115 patients with isolated PMR and 29 patients with GCA was performed. A comparison between isolated PMR patients, GCA patients (with or without PMR), and PMR with GCA patients was performed. Predictors of relapse and corticosteroid discontinuation were identified using a logistic regression in the patients with isolated PMR. RESULTS: Matrix metalloproteinase-3 (MMP-3) level was significantly different among the patient groups. MMP-3: 230.5 ± 201.5 ng/mL in isolated PMR, 80.5 ± 47.5 ng/mL in GCA (p < 0.01), and 96.8 ± 54.8 ng/mL in PMR with GCA (p = 0.03). In the patients with isolated PMR, female gender (odds ratio [OR], 2.73; 95% confidence interval [CI], 1.16-6.41; p < 0.05) and creatinine (Cr) < 50 µmol/L (OR, 2.48; 95% CI, 1.02-5.99; p < 0.05) were significant prognostic factors that predicted relapse. CONCLUSION: A low level of MMP-3 is an excellent positive predictor for PMR with GCA. Among patients with isolated PMR, female gender and Cr < 50 µmol/L were significant prognostic factors that predicted relapse.

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.

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

Mycobacterium 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.

Tuberculous lymphadenitis and the appearance of Behçet's disease-like symptoms.

A 48-year-old man visited our hospital complaining of a tender mass in the left side of the neck. He was diagnosed with tuberculous lymphadenitis based on the results of a biopsy. Shortly after the diagnosis, oral aphthae, erythema nodosum-like lesions on the lower legs and genital ulcers developed. A diagnosis of cutaneous tuberculosis was ruled out according to a negative mycobacterial culture of tissues obtained from stained smears and lesional biopsy specimens. The patient's symptoms remitted following the introduction of antituberculous therapy. We assume that tuberculous lymphadenitis was strongly associated with the appearance of Behçet's disease-like symptoms in this case.

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.

Skin ulcer is a predictive and prognostic factor of acute or subacute interstitial lung disease in dermatomyositis.

The aim of this study was to determine whether skin ulcer can be used as a predictive and prognostic factor of acute/subacute interstitial lung disease (ILD) in Japanese patients with dermatomyositis (DM). We reviewed the medical records of 39 consecutive DM patients who were admitted to Tokyo Metropolitan Komagome Hospital from January 2000 to December 2009. The mean follow-up period was 63.9 ± 51.6 months. Fifteen patients had acute/subacute ILD and 11 patients had chronic ILD. Seven out of 15 acute/subacute ILD led to respiratory failure and 3 of them died due to ILD. Skin ulcers were observed in 5 out of 15 patients with acute/subacute ILD (33.3 %) and in 2 out of 24 patients without acute/subacute ILD (8.3 %). The presence of skin ulcers was revealed to be a significant predictive factor for acute/subacute ILD among various parameters by multivariate analysis. In the 15 patients with acute/subacute ILD, the presence of skin ulcers was a significant poor prognostic factor (p = 0.0231) and the cumulative survival rate of patients with skin ulcers was 53.3 % for 12 months. Skin ulcer is a significant predictive and prognostic factor of acute/subacute ILD in patients with DM.

Clinical and pathological features of Churg Strauss syndrome among a Japanese population: a case series of 18 patients.

BACKGROUND: Many autoimmune diseases differ in individual of different races, but there has been very scarce information on the clinicopathological features of Churg Strauss syndrome (CSS) among Asians patients. OBJECTIVE: To clarify the clinicopathological details of Japanese CSS patients. METHODS: The medical records of CSS patients hospitalized in 1980-2007 were carefully reviewed. RESULTS: Seventeen patients fulfilled the Japanese Ministry of Health, Labour and Welfare (MHLW) criteria and all 18 fulfilled the American College of Rheumatology criteria. Sixteen patients (89%) had the history of asthma. Frequently involved organs were peripheral nerves (PNS) (94%), skin (50%), gastrointestinal tract (33%), kidney (22%), and heart (17%). The mean (range) eosinophil count, C-reactive protein, and number of damaged organs was 18,108 (3,820-36,760)/microL, 51 (0-126) mg/L, and 2.7 (1-6), respectively. Four patients died, of whom three had heart involvement while only one without it died (100 versus 9%, respectively, p = 0.0088). Regarding the pathology, vasculitis was observed in six of seven skin but in only 2 of 10 PNS biopsies. Eosinophilia was found in all of the tissues except for PNS and muscle (40%). Granuloma was observed in only three of the total of 29 biopsies. CONCLUSION: The usefulness of MHLW criteria was verified. The clinical features of Japanese CSS patients were mostly similar to those previously reported, except for lower asthma- and ANCA-positivity rates. Regarding the pathology, vasculitis and eosinophilia were much more frequently observed in skin.

Pulmonary thrombotic microangiopathic hemolytic anemia treated successfully with anticoagulant monotherapy.

Thrombotic microangiopathic hemolytic anemia (TMHA) caused by antiphospholipid syndrome (APS) is generally associated with renal or neurological complications and plasma exchange is the first line of treatment. We present the case of a 72-year-old woman with APS who had chronic TMHA and pulmonary hypertension without other major complications. TMHA and pulmonary hypertension were refractory to plasma exchange but were treated successfully with anticoagulant monotherapy. Contrast-enhanced computed tomography and perfusion scintigraphy did not detect signs of pulmonary embolism. TMHA localized in pulmonary microvasculature which causes pulmonary hypertension is a very rare complication of APS.

Polymyositis associated with focal mesangial proliferative glomerulonephritis with depositions of immune complexes.

A 58-year-old man concurrently developed polymyositis (PM), interstitial lung disease, and nephrotic-range proteinuria. Renal biopsy revealed focal mesangial proliferative glomerulonephritis (mesPGN) with depositions of immunoglobulin and complements. A combination therapy of corticosteroid, intravenous immunoglobulin, and cyclosporine was found very effective for the patient. Glomerulonephritis associated with PM/dermatomyositis (DM) is rare. In our review of related literature, mesPGN was exclusively observed in polymyositis while membranous nephropathy in DM. The mechanism underlying the association between myositis and glomerulonephritis remains to be elucidated.