[Headache in Behçet's disease: case-control study and literature review]. / La cefalea nella malattia di Behçet: contributo casistico e revisione della letteratura.

OBJECTIVE: To evaluate the prevalence of headache and its different patterns in patients with Behçet's disease (BD) with and without neurological involvement and to investigate clinical correlations. METHODS: Patients fulfilling the International Study Group criteria for Behçet disease (ISGc) were studied. Patients were invited to fill a "headache questionnaire", which consisted of two sections: the first one included demographic and anamnestic data, family history for both headache and BD, disease duration and clinical manifestations of BD; the second section included items about headache, investigated accordingly to International Headache Society diagnostic criteria (IHS, 2004). Clinical history and current comorbidities-medications were collected. Each patient underwent a neurological examination to assess neurological involvement (Neuro-BD) and, if necessary, instrumental investigations. One hundred-fifty healthy subjects matched for age and gender were used as control group for comparison. RESULTS: Of the 55 patients diagnosed as BD (ISG criteria) 41 patients adhered and were enrolled into the study. Headache occurred in 29 of BD patients (70,7%) and in 13 of Neuro-BD patients (92,8%). Migraine without aura did prove the most frequent type of headache in BD patients (with and without neurological involvement) and there were no differences in the frequency of the different pattern of headache between BD patients and controls. CONCLUSIONS: Headache is a frequent manifestation in BD and primary headache like migraine emerged as the most frequent type of headache. A careful search for headache should be included in the diagnostic work-up of BD since this manifestation may be related to the underlying disease.

Visceral leishmaniasis and anti-TNF-alpha therapy: case report and review of the literature.

OBJECTIVES: Visceral leishmaniasis (VL) is an extremely rare example of opportunistic infection in patients treated with TNF-alpha antagonists and only a few cases have been described. In this paper risk factors, clinical features, diagnostic work-up and outcome of patients developing VL under biologic therapy are described. METHODS: Case report and review of the published cases of VL in patients under biologic treatment. RESULTS: We retrieved six patients, including ours, all of whom presented anarchic fever and pancytopenia. In 5 cases, splenomegaly was detected. The same number of patients came from endemic areas for VL. In the majority of the cases a bone marrow examination was not diagnostic, requiring the performance of a second one and/or the execution of other diagnostic tests. One fatal outcome was observed. CONCLUSION: Even if VL represents a sporadic complication of biologic treatments, its presence should always be suspected in patients developing a triad of signs and symptoms constituted by fluctuant fever, pancytopenia and splenomegaly, especially if coming from endemic areas. In these cases an extensive diagnostic work-up must be warranted. Atypical and confusing features may resemble autoimmune diseases at presentation and during the course of the illness.

Propionibacterium acnes and SAPHO syndrome: a case report and literature review.

OBJECTIVE: To describe the presence of Propionibacterium acnes (P. acnes) in a series of patients with SAPHO syndrome in which a bone biopsy has been carried out and to discuss the results comparing them to the data described in the literature. METHODS: In 6 out of 56 patients with SAPHO syndrome, a bone biopsy from osteitic lesion was carried out. This invasive investigation was performed only in those cases in which it was necessary to clarify the diagnosis. RESULTS: Of the 6 biopsies processed, P. acnes was isolated in only one case. No other infectious agents were identified. CONCLUSION: P. acnes is not often found in bone lesions of SAPHO syndrome. A bone biopsy may represent a procedure useful for corroborating the diagnosis or for excluding other diseases only in specific cases.

Visceral leishmaniasis complicating a connective tissue disease: three case reports from Italy.

We report the occurrence of visceral leishmaniasis in three patients coming from the northern part of Italy treated with immunosuppressive drugs for different rheumatic conditions. This paper highlights the importance of adequate work-up in those patients presenting with serious clinical manifestations that can complicate, exacerbate or mimic a systemic connective tissue disorder. A prompt diagnosis is very important because visceral leishmaniasis has a high mortality and, if untreated, can be fatal.

Hearing loss evaluation of Sjögren's syndrome using distortion product otoacoustic emissions.

Sjögren's syndrome (SS) is a cell-mediated immune disorder primarily affecting the exocrine glands and hearing loss may be the first otological manifestation of this autoimmune disease. In order to assess the degree of sensorineural hearing loss in SS, 22 female patients were examined by means of standard audiometric tests (pure-tone audiometry, acoustic reflexes and impedance testing) and using distortion product otoacoustic emissions (DPOAEs). The results indicated that only 36.3% of the patients had mild sensorineural hearing loss. Hearing level and distortion product threshold estimates were found to be significantly correlated. No relationship was found between the duration of the disease and the DPOAE and hearing threshold variables. The data suggest that SS may not directly cause sensorineural hearing loss.

[Anterior chest wall examination reviewed]. / La semeiotica del massiccio toracico anteriore rivisitata.

Anterior chest wall involvement is not infrequently observed within inflammatory arthropaties, particularly if one considers seronegative spondyloarthritides and SAPHO syndrome. Physical examination is unreliable and conventional X-rays analysis is an unsatisfactory tool during diagnostic work-up of this region. Scintigraphic techniques yield informations both on the activity and on the anatomical extent of the disease while computerized tomography visualize the elementary lesions, such as erosions, which characterize the process. Moreover, when available, magnetic resonance imaging couple the ability to finely visualize such lesions with the possibility to show early alterations and to characterize the "activity" of the disease, presenting itself as a powerful tool both for diagnosis and follow-up. This review briefly shows the applications of imaging techniques for the evaluation of the anterior chest wall focusing on what has been done in the SAPHO syndrome which can be considered prototypical for this regional involvement since it is the osteo-articular target mainly affected by the disease.

Multicentric reticulohistiocytosis: a rare cause of erosive arthropathy of the distal interphalangeal finger joints.

BACKGROUND: Multicentric reticulohistiocytosis (MRH) is a rare systemic disease, presenting with typical skin abnormalities and erosive polyarthritis, which is often associated with malignancy. CASE REPORT: A case of MRH arthropathy, in which the typical nodular skin manifestation of the disease was absent, is described in a patient with a past history of breast cancer and no evidence of recurrent or new malignancy. RESULTS: Careful clinical and roentgenological evaluation disclosed important clues to differentiate this condition from other more common distal interphalangeal arthritides--namely, osteoarthritis and its "erosive" variant, rheumatoid arthritis, psoriatic arthritis, tophaceous gout, dialysis related hand arthropathy, and from the rarer fibroblastic rheumatism, all of which can be mimicked by MRH. Histopathology showed the characteristic histiocytic and multinucleated giant cell infiltrate with ground glass cytoplasm, and immunohistochemical analysis showed markers evocative of a monocyte/macrophage origin of MRH.

[Autologous stem cell transplantation in a patient with diffuse systemic sclerosis]. / Trapianto autologo di cellule staminali in paziente affetta da sclerosi sistemica a varietà diffusa.

Systemic Sclerosis (SSc) is a systemic disease of unknown etiology presenting with disseminated skin thickening and fibrotic impairment of various organs including lung and kidney. According to the rate and degree of skin involvement, SSc can be classified in a limited and a diffuse form, the latter showing a severe and progressive lung involvement, which is responsible for its high related morbidity and mortality along with resistance to standard therapeutic protocols. High dose chemotherapy, followed by autologous stem cell transplantation, is a standard therapeutic regimen for haematological diseases: re-infusion of mobilised peripheral blood progenitor cells overcomes the myeloablative effect of super-maximal eradicative doses of chemotherapeutic agents. Recently, this therapeutic approach has been applied in some cases of resistant SSc and, albeit the low number of cases, it has been proven effective in early diagnosed and rapidly progressive forms of the disease showing a clinical improvement and an instrumentally detectable decrease of fibrosis extent. We report the case of a young woman affected by diffuse SSc with a rapid progression of clinical signs and instrumentally detectable lesions who underwent a conditioning regimen with fludarabine, cyclophosphamide and anti-thymoglobulines followed by re-infusion of autologous peripheral blood stem cells. Two years after transplantation a clinical and instrumental evidence of treatment was observed, with good control of disease evolution. The only sign of disease resumption was a slow worsening of skin involvement.

A study of growth hormone release in man after oral administration of amino acids.

A study was carried out in 15 male volunteers to evaluate qualitatively the secretion of growth factors following stimulation by oral amino acids. The results showed that oral administration of a combination of two amino acids (1200 mg 1-lysine plus 1200 mg 1-arginine) provoked a release of pituitary somatotropin and insulin. This phenomenon was reproducible and the growth hormone secreted in response to this stimulation had biological activity (as demonstrated by a radioreceptor assay and somatomedin induction). The effect appeared to be specific to the combination of the two amino acids; neither of the amino acids demonstrated appreciable stimulating activity when administered alone, even at the same doses.