Idiopathic nonspecific interstitial pneumonia: an interstitial lung disease associated with autoimmune disorders?

Recent evidence suggests that idiopathic nonspecific interstitial pneumonia (iNSIP) is a distinct clinical entity amongst other idiopathic interstitial pneumonias, and some data seem to suggest a possible pathogenetic role of autoimmunity. The aim of the present study was to assess if iNSIP might represent an early lung manifestation of an autoimmune disease. After initial review of cases found in the medical records database by searching for the term "NSIP" (n = 63), 37 iNSIP cases were identified, and were re-evaluated using a dynamic integrated multidisciplinary approach. 27 cases with iNSIP were selected for the study. Mean ± sd age at first respiratory symptom was 54.2 ± 8 yrs, 70% were females, and 59% were never-smokers. At follow-up (mean ± sd 59.7 ± 29 months, range 12-138 months), autoimmune diseases occurred in 14 (52%) patients, with seven (26%) cases of autoimmune thyroiditis, six (22%) of undifferentiated connective tissue disease and three (11%) of connective tissue disease. Patients developing autoimmune diseases were older and more frequently never-smoking females. In >50% of patients diagnosed with iNSIP, evidence of autoimmune diseases develops within 2 yrs, suggesting a probable link between the clinical entity of iNSIP and autoimmune disorders.

Mycophenolate mofetil is effective in reducing disease flares in systemic lupus erythematosus patients: a retrospective study.

Mycophenolate mofetil (MMF) is effective in the treatment of patients with active systemic lupus erythematosus (SLE). We sought to evaluate its efficacy in reducing the number of disease flares in adults with SLE. For this retrospective study, all patients seen at our institution over a six year period, 1999-2005, with the diagnosis of SLE treated with MMD were identified. Data regarding lupus flare was obtained for patients at least one and up to two years prior to starting MMF. The number of flares prior to MMF therapy was compared to the number of lupus flares in the one to two year period after starting MMF. Clinical assessment was performed with the SELENA-SLEDAI instrument. Differences between groups were compared using the signed rank test. The rate of flares (flares per person-year), before and after the introduction of MMF were compared assuming the occurrence of flares followed a Poisson distribution. In the evaluable 67 patients, the mean time period of followup prior to starting MMF was 14.1 months (range 0.3-24), mean time period of follow-up after starting MMF was 14.8 months (range 1.5-24); and mean MMF dose was 1328 mg/day (range 250-3000). The mean flare rate was reduced from 8.9 to 5.3 per 10 personyears and the mean prednisone dose was reduced on average 7.3 mg/day after starting MMF therapy. MMF treatment significantly reduced the total number of lupus flares and prednisone requirements. Even with the reduction in mean daily prednisone dose, both the SLEDAI and physican global assessment also improved during MMF therapy.

Frequency and duration of clinical remission in patients with peripheral psoriatic arthritis requiring second-line drugs.

OBJECTIVE: To evaluate the frequency and duration of clinical remission in patients with PsA. METHODS: All consecutive new outpatients with peripheral PsA requiring second-line drugs and RA observed between January 2000 and December 2005 were included in a prospective, case-control study. Primary end point was to assess the frequency of remission in peripheral PsA compared with RA. Secondary end points were to compare the duration of clinical remission during treatment and after therapy interruption, ACR 20, 50, 70 response rates and to detect any remission predictor at diagnosis. Treatment regimen was standardized in both groups. From January 2003 to December 2005, therapy was suspended in PsA patients and controls if achieving remission. RESULTS: One or more episodes of remission occurred in 57/236 (24.1%) PsA patients and in 20/268 (7.5%) controls (P < 0.001). The mean duration of remission was of 13 +/- 9.4 months in PsA patients and 4 +/- 3.7 in controls (P > 0.001). Remission episodes were more frequent in PsA patients treated with anti-TNF compared with those receiving traditional DMARDs (P > 0.001), with no differences regarding the duration. After therapy interruption, the remission duration was 12 +/- 2.4 months in PsA and 3 +/- 1.5 in RA (P < 0.001). No remission predictor at diagnosis resulted by multivariate analysis. CONCLUSION: Remission is possible in up to 24% of patients with peripheral PsA. It is significantly more frequent, but not longer, in patients receiving anti-TNF drugs compared with those treated with traditional DMARDs. Patients remain in remission for a long period after therapy interruption, thus suggesting an intermittent therapeutic strategy.

Long-term efficacy of infliximab in refractory posterior uveitis of Behcet's disease: a 24-month follow-up study.

OBJECTIVES: To evaluate the long-term efficacy and safety of infliximab in patients with Behçet's disease (BD) and refractory bilateral posterior uveitis, and to assess the proportion of relapse-free subjects through months 12 and 24. METHODS: Open-label, multicentre, 24-month, prospective, follow up study on 12 consecutive patients with BD and refractory posterior uveitis who had failed at least one immunosuppressive drug. At baseline patients received prednisolone 1 mg/Kg/day with rapid tapering and nine infliximab infusions (5 mg/kg) over a 12-month period. Non-responders after the third infusion withdrew from the study. Patients were evaluated for ocular inflammation degree, visual acuity (VA), number of ocular attacks and incidence of adverse events (AEs). RESULTS: At 12-month visit, 9/12 (75%) patients achieved a complete remission with no relapse during the treatment period. All had a dramatic improvement of ocular inflammation after the first infusion, six were in complete remission after three infusions, and three after four. All these patients suspended corticosteroids at week 22. At 24-month visit, seven out of nine (78%) were still in remission. Mean VA improved from 0.2 +/- 0.6 to 0.5 +/- 0.2 (P < 0.001), and ocular attacks dropped from 40 in the year before therapy to 5 after infliximab cessation (P < 0.001). One patient had a partial remission with two relapses during treatment, and 2/12 (17%) patients showed no improvement. Infliximab was well tolerated with no serious AEs. CONCLUSIONS: Infliximab is rapidly effective and safe in a high proportion BD patients with refractory posterior uveitis, and may be helpful to prevent recurrences.

Inflammatory changes of hip synovial structures in polymyalgia rheumatica.

OBJECTIVE: To investigate the hip inflammatory lesions and to evaluate the accuracy of clinical examination compared to magnetic resonance imaging (MRI) in patients with polymyalgia rheumatica (PMR) with pelvic girdle symptoms. Secondary end-point was to evaluate the sensitivity and specificity of ultrasonography (US) compared to MRI in the assessment of hip lesions. METHODS: Case-control study of 20 consecutive PMR patients and 40 controls with different rheumatic conditions. Both groups were clinically assessed for the presence of hip synovitis, trochanteric, iliopsoas and ischiogluteal bursitis. Hip MRI was performed in all case-patients and in 10 controls. Both groups were examined by US. An additional group of 10 healthy controls was examined by hip US. RESULTS: Both MRI and US detected trochanteric bursitis in 100% of PMR patients, bilateral in 18/20 (90%), and in 12/40 (30%) controls (p < 0.001). Hip synovitis was detected in 17/20 (85%) by MRI and in 9/20 (45%) by US (p < 0.02) in case-patients and in 18/40 (45%) controls. In PMR, MRI and US showed iliopsoas bursitis in 10/20 (50%) and 6/20 (30%) and ischiogluteal bursitis in 5/20 (25%) and 4/20 (20%) with no differences compared to controls. Clinical examination showed a good accuracy for hip synovitis, trochanteric and ischiogluteal bursitis, while it overestimated the presence of iliopsoas bursitis. US was less sensitive than MRI for the detection of hip synovitis and iliopsoas bursitis (53% and 60%). CONCLUSION: Trochanteric bursitis represents the most frequent hip lesion in PMR. A careful physical examination allows to detect all inflammatory lesions excluding iliopsoas bursitis. US is less sensitive than MRI in the assessment of hip synovitis and iliopsoas bursitis.

Pulmonary hypertension in primary Sjögren's syndrome: report of a case and review of the literature.

A 61-year-old female with a history of vaginal dryness, Raynaud's phenomenon, xerostomia and xerophthalmia presented with exertional dyspnoea and weakness. Laboratory and instrumental examinations enabled us to make the diagnosis of primary Sjögren's syndrome, while cardiologic and imaging investigations evidenced isolated pulmonary hypertension and ruled out pulmonary fibrosis. Oral anticoagulant and furosemide therapy induced a partial improvement of exertional dyspnoea and weakness.

Encephalopathy associated with Hashimoto's thyroiditis: an additional case.

A 35-year-old female with a history of Hashimoto's thyroiditis (HT) presented with episodic dysphasia and a burst of axial jerks with abdominal muscle contraction and asymmetric blepharospasm as manifestations of encephalopathy associated with HT. Brain magnetic resonance imaging (MRI) showed scattered supratentorial white matter hyperintense T2 foci. Brain single photon emission computerized tomography (SPECT) demonstrated a cortical hypoperfusion in the upper frontal and parietal areas. Corticosteroid therapy induced a rapid improvement of the clinical picture.

"Skirt" technique for coronary artery bifurcation stenting.

Stent implantation in the treatment of coronary artery bifurcation lesions frequently impairs blood flow and gives the coronary tree a new, metallic configuration. The new technique we describe uses a single short stent in a "skirt" shape which produces no "jailing" effects and can be used in the treatment of true coronary Y-shaped bifurcation lesions.

Clinical Experience with the NIR Stent: Retrospective Observation on Acute Results and Follow-up in 709 Patients.

The NIR stent is an expandable cellular coronary prosthesis with high flexibility and excellent trackability. The very low crossing profile, associated to the high flexibility, makes this stent suitable for complex and distal lesions. The aim of this study was to evaluate the feasibility, safety, and long-term (clinical follow-up) efficacy of elective and/or urgent deployment of the NIR stent in a broad patient group with coronary artery disease. Between June 1996 and May 1997, 986 NIR stents were implanted in 781 vessels (de novo or restenotic lesions in native vessels, saphenous vein grafts and internal mammary conduits) of 709 consecutive patients (498 men, 211 women) undergoing coronary angioplasty in two Villa Maria Group Catheterization Laboratories. Procedural angiographic success was achieved in 98.4% of all the lesions treated, ranging from 100% success rate in type A lesion to 97.6% in type C lesions (overall procedural success, 98.4%). Major adverse cardiac events (MACE) were considered from PTCA/stenting time to the patient discharge. The in-hospital MACE were limited and occurred in 15 patients: urgent surgical revascularization 0.8%, death 0.7%, sub-acute stent thrombosis 0.5% (overall in-hospital MACE rate 2.1%). Clinical follow-up data were obtained at 8.6 +/- 2.8 months following the stenting procedure. The event-free survival rate was 87.9%. The late MACE rate evaluation showed death, target lesion revascularization 9.9% (over-all late MACE rate 12.1%). The NIR stent performances in this broad patient population were excellent, showing very high procedural success rate both in normal and complex coronary anatomy. On the basis of the previous data, we can define NIR stent a safe multifunctional device suitable both for easy and complex situations. The clinical late results are very promising in term of event-free survivals and late MACE.