Systemic complications of esophageal lichen planus.

Lichen planus is an uncommon inflammatory mucocutaneous disorder affecting the skin and its appendages, as well as oral and genital mucosa. Involvement of the esophageal mucosa is rare and causes significant morbidity, with dysphagia and risk of long-term complications, such as esophageal strictures and stenosis. Esophageal lichen planus is an underreported condition in the spectrum of lichenoid tissue reactions, presenting the risk of systemic manifestations. We describe a patient with severe, long-standing esophageal lichen planus, which had led to marked weight-loss, malnutrition syndrome and chronic respiratory distress due to recurrent aspiration pneumonia. Diagnosis was confirmed by the presence of concomitant muco-cutaneous lesions and characteristic endoscopic and histological findings. Systemic therapy with cyclosporine A and micronutrient supplementation led to rapid clinical improvement. Early diagnosis of esophageal lichen planus as well as effective systemic immunosuppressive treatment is crucial in order to prevent short- and long-term complications.

Giant cell arteritis and polymyalgia rheumatica after influenza vaccination: report of 10 cases and review of the literature.

Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are inflammatory rheumatic diseases common in people over the age of 50 years. Herein, we report 10 cases of previously healthy subjects who developed GCA/PMR within 3 months of influenza vaccination (Inf-V). A Medline search uncovered additional 11 isolated cases of GCA/PMR occurring after Inf-V. We discuss the role of individual susceptibility, the potential function of immune adjuvants as triggers of autoimmunity post-vaccination, and the correlation of our observation with the 'ASIA' syndrome, i.e. autoimmune/inflammatory syndrome induced by adjuvants and including post-vaccination phenomena.

[Comparison of a new medical device with domperidone in functional dyspepsia: a randomized, cross-over, controlled study]. / Confronto tra un nuovo dispositivo medico e domperidone in pazienti con dispepsia funzionale: studio randomizzato, cross-over, controllato.

AIM: Management of functional dyspepsia is still controversial. Different controlled trials reported a superiority of prokinetics, H2-receptor antagonists and proton-pump inhibitors over placebo; nevertheless, symptomatic improvement after therapy is often incomplete and some of these drugs possess serious side effects. The aim of the study was to evaluate the efficacy of a new medical device in respect to domperidone in patients with functional dyspepsia. METHODS: In a cross-over, randomized trial, 36 patients with functional dyspepsia ingested two daily doses of a medical device (Digerfast) or domperidone (Peridon) for 21 days. Clinical evaluation was performed at baseline (T0) and after 21 days (T1) for each treatment. A Visual Analogue Scale (VAS) and the generic scale 36-item Short Form (SF-36) were used to assess symptom intensity and changes in health-related quality of life, respectively. RESULTS: At T0 no statistical difference was found for each symptom between medical device and domperidone. At T1 both treatments significantly improved in respect to baseline values all the evaluated gastrointestinal symptoms (P<0.5 for all comparisons) except for vomiting. No difference in gastrointestinal symptoms between the two treatments was found at T1. Regarding SF-36 evaluation, at T0 no statistical differences were found for each SF-36 parameter between the two regimens. At T1 both treatments significantly improved most of the evaluated SF-36 parameters in respect to baseline values. No difference in SF-36 parameters between the two treatments was found at T1. CONCLUSION: Both the medical device and domperidone significantly improved gastrointestinal symptoms and quality of life in subjects with functional dyspepsia, not showing significant difference in efficacy.

Association between familial mediterranean fever and retroperitoneal fibrosis: retroperitoneal fibrosis regression after colchicine therapy.

Retroperitoneal fibrosis (RPF) is a disease characterized by inflammatory fibrotic processes affecting the retroperitoneal structures. Familial Mediterranean Fever (FMF) is an autosomal recessive disorder, characterized by fever and attacks of sterile serositis. Colchicine is the only suitable drug for prevention of acute episodes. We describe a case of association between RPF and FMF in a 48-year-old male, in whom therapy with colchicine, besides preventing acute episodes, allowed RPF regression. To date the association between FMF and RPF and the use of colchicine therapy alone for RPF has not been described.

Atypical sarcoidosis: case reports and review of the literature.

Sarcoidosis is a granulomatous disease of unknown origin, with pulmonary findings in more than 90% of patients. Extrapulmonary involvement is common and all organs can be involved (especially lymph nodes, eyes, joints, central nervous system) but it is rare to find an isolated extrapulmonary disease (less than 10% of patients). Granulomatous inflammation of the spleen and the liver is common in patients with systemic sarcoidosis, while hepatosplenic enlargement is unusual and splenic involvement rare. We report two cases of systemic sarcoidosis, that onset with splenic and hepatosplenic disease, and one case with splenic sarcoidosis without pulmonary involvement. In the first case a 53-year-old woman with mild abdominal pain underwent sonography and CT, which revealed one hypoechoic/hypodense splenic lesion. Laboratory tests were normal. In order to exclude a lymphoma, splenectomy was performed: histology revealed a sarcoid granuloma. After surgery the patient was asymptomatic and now, after two years, disease is silent. The second case is a 66-year-old woman with a recent weight loss (8 kg in two months) and alterated liver function tests (AST 61 U/l, ALT 72 U/l, Alkaline phosphatase 748 U/l, g-GT 381 U/l). Since she had a familiar history of colon cancer, abdominal US scan, abdominal CT scan and MRI were performed and showed inter-aorto-caval lymphadenopathies and discreet multiple bilobar hepatic and splenic substitutive lesions, with no signs of primary tumor. Upper and lower GI endoscopy, full gynecological workup, complete set of tumor markers, bone marrow biopsy were performed. All resulted negative for neoplasia. Small pulmonary infiltrations were observed on chest-CT scan but cytology on BAL was normal. Infections were also excluded. An exploratory laparotomy showed whitish peritoneal, hepatic and splenic nodules. The histological exam revealed chronic granulomatous lesions typical for sarcoidosis. During a two-year follow-up after the splenectomy the patient feels well without any treatment. The third patient is a 32-year-old woman with mild epigastric pain after meals. Neck-thoracic CT, bone scintigraphy and upper GI endoscopy were negative. Abdominal US and MR showed splenomegaly with multiple splenic lesions. Splenectomy was performed and histological exam showed chronic granulomatous lesions typical for sarcoidosis. Further laboratory tests were normal, except for ACE (66 UI/l). After the surgery ACE became normal and now, three years later, the patient is still asymptomatic. We conclude that hepatosplenic involvement is less rare than it is thought. It is often oligosymptomatic or accompanied with unspecific manifestations and laboratory abnormalities. The diagnosis could be difficult; in fact typical laboratory findings of sarcoidosis such as ACE, lysozyme, calcium, were not diagnostic. Ultrasonography and CT were important but the diagnosis was established only with the histological examination of suspected lesions. This latter required to differentiate liver and/or spleen sarcoidosis from tuberculosis and other infections, primary biliary cirrhosis, metastasis or malignant lymphoma.

Gastrointestinal amyloidosis: a case of chronic diarrhoea.

Amyloidosis is a rare disease caused by extracellular deposits of insoluble fibrillar proteins in various organs and tissues. There are different forms of amyloidosis distinguished by the type of protein fibrils, by the sites of deposition and by associated conditions. Gastrointestinal involvement is common both in primary and secondary amyloidosis, while isolated gastrointestinal amyloidosis is rare. We describe a case of AL amyloidosis with a gastrointestinal involvement and restrictive cardiomiopathy. A 64 year old woman came to our attention with a history of chronic diarrhoea and weight loss, associated with dysphagia, dry mouth, xerophtalmia, chronic gastritis and depression. Clinical diagnosis has been difficult because of aspecificity of symptoms that mimed other more common diseases, like gastro-paresis, epigastric discomfort, gastric or duodenal ulcers, perforation, malabsorption, intestinal pseudo-obstruction. There is an important risk of misunderstanding and diagnostic delay. Indeed in this patient a diagnosis of irritable colon syndrome was erroneously established two years before admission in our hospital. Therefore gastrointestinal amyloidosis should be considered among differential diagnoses of chronic diarrhoea and weight loss when other more common diseases have been excluded.

Clinical features of familial Mediterranean fever: an Italian overview.

Familial Mediterranean Fever (FMF) is the most frequent periodic febrile syndrome among the autoinflammatory syndromes (AS), nowadays considered as innate immunity disorders, characterized by absence of autoantibodies and autoreactive T lymphocytes. FMF is a hereditary autosomal recessive disorder, characterized by recurrent, self-limiting episodes of short duration (mean 24e72 h) of fever and serositis. In FMF, periodic attacks show inter- and intra-individual variability in terms of frequency and severity. Usually, they are triggered by apparently innocuous stimuli and may be preceded by a prodromal period. The Mediterranean FeVer gene (MEFV) responsible gene maps on chromosome 16 (16p13) encoding the Pyrine/Marenostrin protein. The precise pathologic mechanism is still to be definitively elucidated; however a new macromolecular complex, called inflammasome, seems to play a major role in the control of inflammation and it might be involved in the pathogenesis of FMF. The most severe long-term complication is type AA amyloidosis, causing chronic renal failure. Two types of risk factors, genetic and non-genetic, have been identified for this complication. Currently, the only effective treatment of FMF is the colchicine. New drugs in a few colchicine resistant patients are under evaluation

Cyclosporine A: good response for patients affected by autoimmune disorders and HCV infection?

INTRODUCTION: In autoimmune disorders (ADs), if Hepatitis C Virus (HCV) is present, immunosuppressive treatment could increase virus replication. Cyclosporine A (CsA), in standard therapeutic doses, has been proven able to inhibit HCV cyclophilin in vitro. Therefore CsA could improve the therapy of HCV patients with ADs. AIM: In these patients, we started an open pilot study to evaluate the safety of 3 mg/kg CsA and the ability to reduce steroid therapy. PATIENTS AND METHODS: Five females and 1 male were recruited; mean age 66 +/- 8 years, mean disease duration 13 +/- 5 years. Three patients are affected by Psoriasic Arthritis, 1 by Rheumatoid Arthritis, 1 by Sjogren Syndrome, and 1 by Myasthenia Gravis. None of them had chronic active hepatitis. HCV genotypes were type 2 (in 3 cases) and type 1 (in 3 cases). Patients were treated with 3 mg/kg of CsA for a period of time ranging from 6 to 12 months. The starting mean dose of prednisone was 12.5 mg/day. Liver function tests were checked monthly and serum HCV-RNA load was checked by RT-PCR before and 2 months into the therapy. RESULTS: The prednisone dose was reduced from 12.5 mg/day to 7.5 mg/day. The aminotransferases levels were unchanged after 6 months. In patients with low HCV-RNA levels before treatment, no modifications of viral load were observed, whereas patients with increased levels at onset showed mild reduction 2 months into the treatment. CONCLUSIONS: Immunosuppressive treatment of ADs patients with HCV infection can be safely provided with the integration of CsA.

Wegener's granulomatosis: an update on diagnosis and therapy.

Wegener's granulomatosis (WG) is a unique clinicopathological disease characterized by necrotizing granulomatous vasculitis of the respiratory tract, pauci-immune necrotizing glomerulonephritis and small-vessel vasculitis. Owing to its wide range of clinical manifestations, WG has a broad spectrum of severity that includes the potential for alveolar hemorrhage or rapidly progressive glomerulonephritis, which are immediately life threatening. WG is associated with the presence of circulating antineutrophil cytoplasm antibodies (c-ANCAs). The most widely accepted pathogenetic model suggests that c-ANCA-activated cytokine-primed neutrophils induce microvascular damage and a rapid escalation of inflammation with recruitment of mononuclear cells. The diagnosis of WG is made on the basis of typical clinical and radiologic findings, by biopsy of involved organ, the presence of c-ANCA and exclusion of all other small-vessel vasculitis. Currently, a regimen consisting of daily cyclophosphamide and corticosteroids is considered standard therapy. A number of trials have evaluated the efficacy of less-toxic immunosuppressants and antibacterials for treating patients with WG, resulting in the identification of effective alternative regimens to induce or maintain remission in certain subpopulations of patients. Recent investigation has focused on other immunomodulatory agents (e.g., TNF-alpha inhibitors and anti-CD20 antibodies), intravenous immunoglobulins and antithymocyte globulins for treating patients with resistant WG.