The Clinical Relevance of Antifibrillarin (anti-U3-RNP) Autoantibodies in Systemic Sclerosis.

Systemic sclerosis (SSc) is a heterogeneous autoimmune disease associated with several antinuclear autoantibodies useful to diagnosis and prognosis. The aim of the present multicentric study was to determine the clinical relevance of antifibrillarin autoantibodies (AFA) in patients with SSc. The clinical features of 37 patients with SSc positive for AFA (AFA+) and 139 SSc patients without AFA (AFA-) were collected retrospectively from medical records to enable a comparison between AFA- and AFA+ patients. Antifibrillarin autoantibodies were screened by an indirect immunofluorescence technique using HEp2 cells and identified by an in-house Western blot technique and/or an EliA test. Comparing AFA+ and AFA- patients, AFA+ patients were significantly younger at disease onset (36.9 versus 42.9; P = 0.02), more frequently male (P = 0.02) and of Afro-Caribbean descent (65% versus 7.7%; P < 0.001). At diagnosis, the Rodnan skin score evaluating the cutaneous manifestations was higher (13.3 versus 8.7; P = 0.01) and myositis was also more common in the AFA+ group (31.4% versus 12.2%; P < 0.01). Patients with AFA+ were not associated with diffuse cutaneous SSc or with lung involvement and no difference in survival was observed. Antifibrillarin autoantibodies are associated with patients of Afro-Caribbean origin and can identify patients with SSc who are younger at disease onset and display a higher prevalence of myositis.

Serum CC chemokine ligand-18 predicts lung disease worsening in systemic sclerosis.

Elevated serum CC chemokine ligand (CCL)18 reflects lung fibrosis activity in systemic sclerosis (SSc) and could be an early marker of lung function worsening. Therefore, we sought to evaluate whether serum CCL18 levels at baseline could predict worsening of lung disease in SSc. In this prospective study, 83 SSc patients were analysed longitudinally over a 4-yr observation period for the risk of occurrence of combined deleterious events, defined as a 10% decrease from baseline of total lung capacity or forced vital capacity % predicted, or death, according to serum CCL18 at inclusion. Receiver operating characteristic (ROC) curve analysis was performed for prediction of events during the first year after inclusion. The best cut-off level of serum CCL18 for prediction of a combined event within the follow-up period was 187 ng · mL(-1), with 53% sensitivity and 96% specificity (area under the ROC curve 0.86; p < 0.001). After a mean ± SD follow-up of 33.7 ± 10.8 months, a higher rate of disease progression occurred in the group with serum CCL18 levels >187 ng · mL(-1). The adjusted hazard ratio was 5.36 (95% CI 2.44-11.75; p < 0.001). In summary, serum CCL18 is an accurate predictive biomarker for the identification of patients with a higher risk of subsequent scleroderma lung disease worsening.

Diagnostic value of exhaled nitric oxide to detect interstitial lung disease in systemic sclerosis.

BACKGROUND AND AIM: Increased alveolar concentration of nitric oxide (CA(NO)) is related to the severity of interstitial lung disease (ILD) in systemic sclerosis (SSc). However, cut-off levels of CA(NO) to rule out, or to rule in, the presence of ILD in individual patients are unknown. We aimed to assess the validity of CA(NO) for the diagnosis of ILD in SSc and to determine the thresholds of CA(NO) that can be used in clinical practice to predict the likelihood of ILD in SSc. METHODS: Lung HRCT scan, PFTs and partitioned exhaled NO measurements were performed in 65 consecutive SSc patients. ILD was diagnosed on pulmonary HRCT according to the presence of ground glass or reticular opacities. Diagnostic performance of CANo for ILD diagnosis was assessed using ROC curves. RESULTS: 38 out of 65 SSc patients had ILD. CA(NO), at a cut-off level of 4.3 ppb, had a sensitivity and specificity for the diagnosis of ILD of 87% (95% CI: 77 to 99) and 59% (95% CI: 41 to 78), respectively. The same cut-off level of CA(NO) could detect impairment of gas exchange with a sensitivity and specificity of 78% (95% CI: 67 to 90) and 73% (95% CI: 46 to 99), respectively. Moreover, ILD could be ruled in (positive predictive value > 95%) when CA(NO) > or = 10.8 ppb, and ruled out C(ANO) values < or = 3.8 ppb (negative predictive value > 95%). CONCLUSION: CA(NO) could be a valid non-invasive biological marker of ILD in SSc, and be of use in clinical practice.

[Short-term effect of dietary-sodium intake on arterial blood pressure of patients treated with systemic corticosteroids: a prospective, randomised, crossover study]. / Effet à court terme des apports sodés sur la pression artérielle des patients recevant une corticothérapie systémique: étude prospective, randomisée, croisée.

INTRODUCTION: It is unknown if the level of dietary-sodium intake influences blood pressure in patients receiving systemic corticosteroids. METHODS: Randomized, single centre, crossover trial involving patients starting systemic corticosteroid therapy and having initial blood pressure less or equals to 159/99 mm Hg. The first period of sodium regimen was randomized (<3 g/j versus >6 g/j) and each period of sodium regimen lasted 3 weeks. No washout period was performed. Blood pressure was recorded for each patient at inclusion and after 3 weeks and 6 weeks. Moreover, all patients were asked to record on a standardized questionnaire everything they ate during 1 week of each period regimen. Questionnaires were analysed by a dietician for mean daily energy and sodium intakes during each period. Mixed models were used to estimate the relationship between sodium intake and blood pressure variations. RESULTS: Between June 2006 and June 2008, 49 patients were randomized, 24 in group 1 (first period regimen=salt<3g/day; women: 63%; mean age: 56+/-21 years; baseline prednisone dosage: 54+/-19 mg/day) and 25 in group 2 (first period regimen=salt>6g/day; women: 56%; mean age: 60+/-19 years; baseline prednisone dosage: 56+/-16 mg/day). Mean daily salt intakes were 2.5+/-1.8 and 9.3+/-1.9 g/day during the first period and 7.8+/-3.2 and 3.8+/-2.9 g/day during the second period, respectively for group 1 and group 2. Blood pressure variations were not significantly associated with daily salt intakes or with randomisation group. No order effect was evidenced. By comparison with baseline, systolic blood pressure increased by greater than 20 mm Hg at week 6 in five patients (2 in group 1 and 3 in group 2). CONCLUSION: At short-term, sodium intake does not seem to influence blood pressure variations in patients starting systemic corticosteroids therapy.

[Schwannoma of the duodenojejunal angle]. / Schwannome kystique de l'angle duodénojéjunal.

Neurogenic tumors of the small intestine are extremely rare. Although schwannoma is often clinically indolent for many years, complications such as gut compression or bleeding might occur. In these cases, surgical management is required. We reported a case of asymptomatic schwannoma of the duodenojejunal angle. Surgical treatment was performed to provide definitive immunohistochemistry diagnosis and to prevent complications.

[Primary chronic autoimmune neutropenia successfully treated with sirolimus]. / Neutropénie auto-immune primaire avec autoanticorps antineutrophiles traitée avec succès par sirolimus.

We report a 74-year-old man with severe chronic primary neutropenia (neutrophil count: 390 per millimeter cube) uncovered following surgery for perianal abscess collection. Clinical, laboratory and roentgenographic findings revealed no abnormality. Antineutrophil antibodies were positive in two consecutive serum samples. Under cyclosporine, neutrophil count reached 1970 per millimeter cube. However, this therapy was discontinued due to new onset of severe renal failure. After six weeks, neutrophil count was 950 per millimeter cube and sirolimus was started, resulting in renal function improvement and resolution of neutropenia.

[Long-term systemic corticosteroid-therapy associated measures: description of the French internal medicine physicians' practices]. / Mesures adjuvantes à une corticothérapie systémique prolongée : description des pratiques des médecins internistes français.

INTRODUCTION: Except for the prevention of osteoporosis, no consensual recommendations are available regarding the therapeutic measures associated with the prescription of long-term corticosteroid therapy. The aim of this study was to assess the internal medicine physicians' practices regarding the prescription of long-term corticosteroid therapy. METHODS: In September 2007, we sent, by e-mail, a questionnaire to 813 internal medicine physicians, members of the French National Society of Internal Medicine. With this questionnaire, we assessed the frequency of prescription of measures sometimes associated with systemic corticosteroids and for whom no consensual recommendations were available (dietary advices, physical training, potassium supplementation, gastric protection, influenza vaccination and prescription of hydrocortisone). RESULTS: Three hundred and thirty-six out of 813 internal medicine physicians completed the questionnaire (response rate: 41%). The practitioners were predominantly male (71%) and mainly engaged in tertiary centres (53%). Regarding the dietary measures associated with the prescription of corticosteroids, low-sodium diet was recommended by most of the physicians, 69% of them prescribing such dietary regimen in more than 80% of their corticosteroid-treated patients. The concomitant prescription of caloric restriction, low-carbohydrate diet and/or high-protein diet was not consensual. The prescription of muscular physiotherapy was unusual, 74% of physicians prescribing such reeducation in less than 20% of their patients. The frequency of recommendation for daily physical training varied between physicians as well as for potassium supplementation, gastric protection, influenza vaccination or hydrocortisone prescription. CONCLUSION: There is no consensus between French internal medicine physicians regarding most of the measures, which must be prescribed in association with a long-term corticosteroid therapy.

[Systemic corticosteroid therapy: patients' adherence to dietary advice and relationship between food intake and corticosteroid-induced lipodystrophy]. / Corticothérapie systémique et alimentation: suivi des recommandations diététiques et relation entre apports alimentaires et apparition d'une lipodystrophie.

PURPOSE: No data is available about: 1) the adherence of corticosteroid-treated patients to dietary advice provided by physicians; 2) the relationship between food intake and the corticosteroid-induced lipodystrophy (CIL). METHODS: We conducted a cohort study in 2 French tertiary centers between June 2003 and May 2005 and enrolled all consecutive patients starting long-term systemic corticosteroid therapy. They received individual dietary advice from a qualified dietetician and were asked to record on a standardized questionnaire everything they ate during one week of the first and third months of treatment, including details of each meal. Each questionnaire was analysed by two qualified dieteticians for daily calorie, carbohydrate, fat, protein and sodium intake. Moreover, 3 investigators assessed the development of CIL from standardized patients' photographs. The relationship between food intake and CIL was investigated by a multiple logistic regression model. RESULTS: Eighty-eight patients were included and 80 were monitored until at least month 3 (women: 76%, mean age: 59.1+/-18.7 years). Most patients (65%) had giant-cell arteritis or connective tissue disease. The mean initial dosage of prednisone was 54+/-17 mg/day and the mean M3 dosage was 31+/-15 mg/day. Most patients were adherent to dietary advice during the first 3 months of therapy except for protidic ration which was below expected value. Sodium restriction was more strictly followed by women than by men. Multivariate analysis showed independent relationship between CIL and higher calorie intake (>30 kcal/kg/day). No relationship was evidenced between carbohydrate, protein, fat or sodium intake and the risk of CIL. CONCLUSION: During the first 3 months of therapy, corticosteroid-treated patients are adherent to dietary advice. A calorie-controlled alimentation could be beneficial to limit the risk of CIL.

[Natural history of corticosteroid-induced lipodystrophy: a prospective follow-up of 37 patients]. / Histoire naturelle de la lipodystrophie cervicofaciale cortico-induite: suivi prospectif d'une cohorte de 37 patients.

PURPOSE: No data is available about the natural history of the corticosteroid-induced lipodystrophy. The purpose of this study is to describe the natural history of corticosteroid-induced lipodystrophy in a selected and homogenous population. METHODS: We conducted a cohort study between June 2003 and September 2005 and enrolled all consecutive patients starting long-term systemic corticosteroid therapy for giant cell arteritis (because of a standardized therapeutic schedule). After enrollment, patients were seen every month until the end of the corticosteroid therapy. After the drug withdrawal, they consulted every 3 months during 6 months. At each consultation, they were photographed in a standardized way. At the end of the study, the development of lipodystrophy was assessed by analyzing these photographs. We evaluated the incidence of corticosteroid-induced lipodystrophy during the course of giant cell arteritis therapy and the time between initiation of therapy and its apparition. Lastly, we evaluated the time between the prednisone withdrawal and the disappearance of lipodystrophy (or the mean prednisone dosage if the disappearance was observed under treatment). RESULTS: Thirty-seven patients were included (women: 73%; mean age: 75+/-7 years; mean initial daily prednisone dosage: 44+/-13 mg). The mean duration of follow-up was 23.6+/-7.4 months. Incidence of corticosteroid-induced lipodystrophy was 48% after 3 months and 60% after 12 months of therapy. The median time between treatment initiation and appearance of lipodystrophy was short (3 months [1-4]). During the decrease of the therapy or the 6 months following its end, we observed a regression of lipodystrophy in 71% of the patients. The median time between corticosteroids initiation and disappearance of lipodystrophy was 19 months [16-22] (concomitant median daily dosage of prednisone: 4 mg [0-7]). CONCLUSION: Lipodystrophy is frequently observed in corticosteroid-treated old patients. It appears precociously after the initiation of therapy and is usually reversible.

Human leukocyte antigens and susceptibility to tuberculosis: a meta-analysis of case-control studies.

OBJECTIVE: To evaluate the association between human leukocyte antigens (HLA) of the major histocompatibility complex (MHC) and thoracic tuberculosis (TB) in immunocompetent adults. DESIGN: We searched Medline, Pascal, Pascal Biomed and Francis databases (all years) with the terms 'tuberculosis' and 'HLA'. Case-control studies were included that reported frequencies for the full range of antigens analysed by serological methods in healthy controls and adult patients not treated with glucocorticoids or immunosuppressive drugs, human immunodeficiency virus status negative or not reported, no debilitating chronic disease, and with a diagnosis of thoracic TB based on microbiological or histological criteria. Two authors independently abstracted the data and resolved disagreements by consensus. RESULTS: We summarised 60 HLA antigens reported in at least four of 22 studies totalling 1988 patients and 2897 controls. A lower risk of thoracic TB was found in carriers of B13 (OR 0.64, 95% CI 0.50-0.81, P < 0.0001), DR3 (OR 0.72, 95% CI 0.59-0.89, P = 0.002), and DR7 antigens (OR 0.65, 95% CI 0.53-0.80, P < 0.0001). Carriers of DR8 were at higher risk for thoracic TB (OR 1.72, 95% CI 1.21-2.46, P = 0.003). For these antigens, we found no significant heterogeneity between samples or evidence of publication bias. The risk of thoracic TB tended to be higher in carriers of DR2 (OR 1.67, 95% CI 1.16-2.41, P = 0.006), but the results were not consistent between studies (P value for heterogeneity < 0.0001). CONCLUSION: Susceptibility to TB is modulated by class I and II HLA antigens. However, these results based on the serological determination of antigens require confirmation by DNA-based methods to precisely identify those alleles involved.

[Cytomegalovirus and arterial disease. Current aspects]. / Cytomégalovirus et pathologies artérielles. Aspects actuels.

The cytomegalovirus (CMV) is the only microbial agent implicated in three particular types of arterial disease: coronary disease of the transplanted heart, post-angioplasty restenosis and atherosclerosis. The object of this article is to analyse the recent data on the role of CMV in these pathologies with an exhaustive review of the literature. The available data is mainly epidemiological but the interpretation is difficult because of the multiplicity and imperfections of the diagnostic techniques of the infection. However, the results are quite concordant in favour of a real association. Different physiopathological mechanisms are proposed. In coronary disease of the transplanted heart, the lesions could be initiated by an inflammatory process. In post-angioplasty restenosis, the virus seems able to trigger cellular proliferation by inhibiting the mechanisms of apoptosis. Finally, in atherosclerosis, CMV infection seems to promote atherothrombotic processes and accelerate the progression of atherosclerotic plaques by activating inflammatory cells. Direct methods of detection of viral DNA show the presence of the virus within these lesions. There is, therefore, epidemiological, anatomo-pathological and physiopathological evidence in favour of a relationship between CMV infection and these three forms of arterial disease.

[Quantitative assessment of carotid stenosis: comparison between Doppler ultrasound, spiral computed tomography angiography, magnetic resonance angiography and digital angiography]. / Quantification du degré de sténose carotidienne. Comparaison entre l'échographie Doppler, le scanner hélicoïdal, l'imagerie par résonance magnétique et l'angiographie numérise.

BACKGROUND: The North American Symptomatic Carotid Endarterectomy Trial has confirmed the benefit of carotid endarterectomy in comparison to medical treatment in stroke prevention in symptomatic patients having a carotid stenosis of 70% or more. The Asymptomatic Carotid Atherosclerosis Study has concluded that the benefit of surgical treatment remains significant in asymptomatic patients with 60% (or more) stenosis of the ipsilateral internal carotid artery, when mortality rate remains inferior to 3%. In these two trials, angiography has been used to quantify the stenosis. Though this test is carrying some neurological and renal risks, replacing the angiography stenosis grading for a non or less invasive test, seems to be permissible. METHODS: In our retroprospective study, the assessments of the carotid stenosis by several non-invasive tests findings were compared to the angiography results. Nineteen carotid arteries of fifteen patients, both symptomatic and asymptomatic, having a carotid stenosis at least 60% or more and being detected by the Doppler ultrasound were explored either by magnetic resonance angiography (MRA), spiral computed tomography angiography (SCTA) and angiography. RESULTS: The ultrasonography and angiography findings were well correlated (r=0,88; p<0.002) according to the Spearman test. The assessments of the MRA were better correlated to the angiography than to the SCTA (respectively r=0.91, p<0.0001 and r=0,68, p<0.001). Using both ultrasonography and MRA as a confirmatory test, the rate of injustified carotid endarterectomy was 25%. And this rate rose up to 33% when the ultrasonography was used with the SCTA. It is noteworthy that negative predictive value of ARM was 100%. To reduce the mortality rate, several surgical teams managed the carotid stenosis without angiography. CONCLUSION: MRA could replace angiography, on condition that the rate of unjustified carotid endarterectomy lowers and becomes acceptable. Far reaching complementary studies are necessary to confirm the fiability of those non-invasive tests. In order to raise the benefit to carotid endarterectomy, the research studies should turn to the predictive score determination of a surgical international risk and towards the "High benefit" patients groups after endarterectomy.

[Treatment of chronic postinfectious fatigue: randomized double-blind study of two doses of sulbutiamine (400-600 mg/day) versus placebo]. / Traitement de l'asthénie en période postinfectieuse: étude randomisée en double aveugle de deux doses de sulbutiamine (400-600 mg/j) versus placebo.

PURPOSE: Chronic fatigue remains a medical mystery and a therapeutic failure. The subgroup of chronic fatigue postinfectious fatigue (CPIF) is an interesting one since it is quite frequent in general practice. METHODS: We studied sulbutiamine (Su), isobutyryl-thiamine disulfide in this context. We included 326 general-practice patients suffering from CPIF: they received randomly either Su, 400 mg daily (n = 106), or Su, 600 mg daily (n = 111), or placebo (n = 109) for 28 days in a double-blind, parallel-group study. 315 patients completed the study. RESULTS: The evaluation of fatigue, by multiple means including mainly MFI, a validated multidimensional fatigue scale, showed overall no significant difference between the groups. On the 7th day, however, women receiving Su, 600 mg had less fatigue (P < 0.01), but the figures were quite diverse and no persistent effect was noted at the 28th day. CONCLUSION: Thus, we showed for the first time that a high level general-practice study of fatigue is feasible using specific tools. Whether the effect observed after 1 week in women represents a true finding needs additional research. Further studies are in progress in order to characterize better the potential usefulness of Su in chronic fatigue.

[Laryngeal amyloidosis: a rare cause of hoarseness]. / Amylose laryngée: une cause rare de dysphonie.

INTRODUCTION: The larynx is a rare site of deposition for amyloidosis. Diagnosis may be delayed and evoked in patients with prolonged hoarseness. We have reported two cases of laryngeal amyloidosis. EXEGESIS: One man and one woman suffered from hoarseness during one and three years, respectively. Laryngoscopic examination showed diffuse infiltration of the larynx. Amyloidosis was confirmed by the characteristic Congo-red staining of laryngeal biopsies. The search for other localizations of amyloidosis was negative. No monoclonal plasma cell proliferation was detected. Both patients received endoscopic CO2 laser excision. In one case, chemotherapy was initially associated due to dystrophic plasma cells in bone marrow aspiration and then withdrawn because of clinical failure. With a 6-month follow-up, hoarseness remained stable. CONCLUSION: Laryngeal amyloidosis is an essentially localized disease revealed by hoarseness. Treatment is endoscopic by laser excision. In nearly half of the cases in the literature, it had to be repeated due to localized recurrent lesions. Long-term prognosis of localized laryngeal amyloidosis is better than systemic AL amyloidosis.

[Severe Moorens ulcer: efficacy of monthly cyclophosphamide intravenous pulse treatment]. / Ulcères de Mooren sévères: efficacité du traitement par cyclophosphamide en bolus intraveineux mensuels.

PURPOSE: Mooren's ulcer (MU) is a chronic peripheral corneal ulceration featuring conjunctival immunoglobulin deposits. It is considered as the result of a limbic immune process with hyperactivation of T and B lymphocytes. The etiology remains unknown. The response to topical steroid therapy and surgical procedures usually poor and the visual outcome can be devastating. METHODS: Clinical follow-up of 3 patients who had rebel MU to conventional therapy, and were treated with 1g monthly intravenous cyclophosphamide. RESULTS: First patient was a 24-years-old man who had MU in his left eye. The response to surgical procedure and intravenous steroid treatment was poor and corneal perforation occurred. The affected cornea healed after 9 months of Cy treatment. The second patient was a 50-years-old man who had MU in his left eye, which did not improved with lamellar keratoplasty and topical steroid therapy. Corneal healing was obtained after 20 months of Cy treatment. The third patient was a 70-years-old man who presented with a furrowed MU in his right eye which healed with conjunctival resection and 4 months of Cy perfusion. No adverse effects of Cy was noted as opposed to Cy given orally. CONCLUSION: We report the effectiveness of 1g monthly intravenous cyclophosphamide (Cy) treatment in rebel MU. We suggest that immunosuppressive therapy using IV monthly Cy may be proposed in severe rebel MU.

[The methods for screening pulmonary hypertension related to systemic sclerosis in France. Descriptive survey of the French Research Group on Sclerosis]. / Pratiques françaises pour le dépistage de l'hypertension artérielle pulmonaire associée à la sclérodermie systémique. Enquête descriptive du Groupe de Recherche Français sur la Sclérodermie.

CONTEXT AND OBJECTIVES: Pulmonary hypertension (PHT) represents one of the severest complications and is life-threatening for patients suffering from systemic sclerosis (SSc). In France, the modalities for screening and treating PHT related to SSc are not well codified and no consensus has been reached. We conducted a survey among physicians inscribed on the list of the French Research Group on Sclerosis (GRFS - Groupe de Recherche Francais sur la Sclerodermie) to gather information on the status of the management of PHT related to SSc. METHODS: In 2002, we sent a questionnaire to 160 physicians, members of the GRFS, to assess the epidemiology and clinical profile of SSc patients as well as the modalities of screening and management of PHT in these patients. RESULTS: Eighty-eight physicians in 71 centres replied to the questionnaire. Each centre followed-up a mean of 33 SSc patients, with a global distribution of 53% limited and 47% diffused SSc. These physicians saw a mean of 5 new cases of SSc per year. The patients had been referred by town practitioners (53%) or from the hospital (47%). The mean number of SSc patients with PHT was of 5.1 per physician (1.5 new SSc + PHT patients per year). Almost all the centres (65/67) who replied systematically screened for PHT in SSc patients using Doppler echocardiography a mean of every 1.3 years. For the management of the patients exhibiting PHT, the majority (41/63) of centres collaborated with a specialized unit. Around one third of the centres treated these patients with calcium channel inhibitors (82%) and/or prostacyclin (90%). All the patients were followed-up by Doppler echocardiography. The majority of the physicians (72%) were interested in a research protocol on the subject and each could have included 4 patients, i.e., a total of 160. CONCLUSION: Pulmonary hypertension, a severe complication of SSc is screened for by the physicians of the GRFS using echocardiography with a frequency similar to Who guidelines (1.3 versus once/year).

[Use of pulmonary function tests and biomarkers studies to diagnose and follow-up interstitial lung disease in systemic sclerosis]. / Intérêts des explorations fonctionnelles respiratoires et des études des biomarqueurs dans le diagnostic et le suivi évolutif de la pneumopathie interstitielle diffuse dans la sclérodermie systémique.

Interstitial lung disease (ILD) is becoming one of the main causes of death of patients with systemic sclerosis (SSc). The prevalence of ILD associated with SSc (SSc-ILD) varies from 33% to 100% according to diagnostic methods. Clinical features such as dyspnea on exertion, dry cough, and chest pains are not specific and usually late-appearing, implying more specific tests in the diagnostic, prognosis, and follow-up of ILD in patients with SSc. High resolution thoracic CT scanner (HRCT) is more sensitive than chest X-ray in the detection of SSc-ILD. Pulmonary function tests (PFT) are non-invasive and periodically used to assess the impacts of SSc on respiratory function. Diagnostic values of bronchoalveolar lavage and histological examination on lung biopsy are controversial. However, these techniques are essential for studying cellular and molecular mechanisms underlying the pathophysiology of SSc-ILD. Several biomarkers such as surfactant-A (SP-A), -D (SP-D), mucin-like high molecular weight glycoprotein (KL-6), and chemokine CCL-18 have been implicated in SSc-PID. Serum levels of these proteins are correlated with the severity of SSc-ILD, as assessed by HRCT and/or PFT. Finally, alveolar concentration of exhaled nitric oxide can be used to screen SSc patients with high risk of deterioration of respiratory function, in whom immunosuppressant treatment could be useful in preventing the evolution to irreversible lung fibrosis.