Pulmonary arterial hypertension (PAH), idiopathic pulmonary fibrosis (IPF), and scleroderma (SSc) are three interrelated medical conditions that can result in significant morbidity and mortality. Pulmonary hypertension, a condition marked by high blood pressure in the lungs, can lead to heart failure and other complications. Idiopathic pulmonary fibrosis, a progressive lung disease characterised by scarring of lung tissue, can cause breathing difficulties and impaired oxygenation. Scleroderma, an autoimmune disease, can induce thickening and hardening of the skin and internal organs, including the lungs, leading to pulmonary fibrosis and hypertension. Currently, there is no cure for any of these conditions. However, early detection and proper management can improve the quality of life and prognosis of a patient. This review focusses on PH and IPF in patients with SSc, providing information on the causes, symptoms, and treatment of these conditions, together with illustrative images. It also provides an overview of interrelated medical conditions: PH, IPF, and SSc. It emphasises the importance of early detection and proper management to improve patient quality of life and prognosis.

OBJECTIVE: To assess the influence of systemic sclerosis (SSc) on the survival rate of dental implants in SSc patients receiving implant-supported treatments.
METHODS: The Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) Statement and the Cochrane Collaboration\'s guiding principles were followed during the study\'s execution. The data from three databases, PubMed, Google Scholar, and Scopus, available until January 2023, were used to compile the material for our research. Only English-language publications were submitted for this research and evaluated based on their titles, abstracts, and full texts. For performing a quality assessment, quality scores were calculated.
RESULTS: The total number of patients and implants studied were 37 and 153, respectively, all having had scleroderma. The patients\' ages ranged from 28 to 77 years old, with a mean (SD) age of 58.16 (12.88). All the patients in the case reports and most in the case series study were female. The range of follow-up duration was from 1 to 10 years. In case report studies, the survival rate was 100%; in case series, it was 89.2%.
CONCLUSIONS: The SSc status had no discernible impact on the implant survival rate. Implant-based treatments in SSc patients should not worsen the overall morbidity and should not conflict with systemic treatments. Before starting implant therapy, a thorough risk assessment is essential, though.

Systematic scleroderma is a rare chronic autoimmune disease of unknown aetiology. The aim of this study was to identify the prevalence of orofacial pathognomonic conditions in patients with systemic scleroderma using only randomised prospective studies that investigated the treatment of oral and maxillofacial changes, highlighted associations between the disease and Sjogren\'s syndrome, and/or analysed the effect of oral hygiene.
The literature was systematically reviewed based on Cochrane Library, EMBASE, PubMed, Scopus, and Web of Science articles published up to March 2020. The primary endpoint of this analysis was defined as an estimation of the prevalence of oral mucosal changes in different areas of the oral cavity (oral mucosa, tongue, lip, periodontal status, bones, and other regions) in patients suffering from scleroderma. Therefore, a systematic literature search (Cochrane Library, EMBASE, PubMed, Scopus, and Web of Science) was conducted and limited by the publication date (1950-03/2020) and the publication language (English). Extracted frequencies were pooled using methods for meta-analysis. In order to obtain the highest level of evidence, only prospective study reports were considered to be eligible.
After full-text screening, 14 (766 patients) out of 193 publications were eligible for the final analysis. Twelve studies produced reliable results in the final data sets. Calculation of the pooled effect estimate (random effects model) revealed a prevalence of 57.6% (95% CI: 40.8-72.9%) for the main area \"lip\". For the area \"oral mucosa\", a prevalence of 35.5% (95% CI: 15.7-62.0%) was calculated. The prevalence for \"other regions\" was only based on studies with salivary changes and was calculated to be 25.4% (95% CI: 14.2-41.3%).
The most pathognomonic conditions in the orofacial region in patients with systemic scleroderma affect the lips, oral mucosa, and salivary glands.

Autoimmune diseases are an important field for the development of bone marrow transplantation, or hematopoietic stem cell transplantation. In Europe alone, almost 3000 procedures have been registered so far. The Brazilian Society for Bone Marrow Transplantation (Sociedade Brasileira de Transplantes de Medula Óssea) organized consensus meetings for the Autoimmune Diseases Group, to review the available literature on hematopoietic stem cell transplantation for autoimmune diseases, aiming to gather data that support the procedure for these patients. Three autoimmune diseases for which there are evidence-based indications for hematopoietic stem cell transplantation are multiple sclerosis, systemic sclerosis and Crohn\'s disease. The professional stem cell transplant societies in America, Europe and Brazil (Sociedade Brasileira de Transplantes de Medula Óssea) currently consider hematopoietic stem cell transplantation as a therapeutic modality for these three autoimmune diseases. This article reviews the evidence available.

To investigate the validation status of laser Doppler flowmetry (LDF) in systemic sclerosis (SSc) according to the \'Outcome Measures in Rheumatologic Clinical Trials\' (OMERACT) filter.
The literature was systematically reviewed to identify all reports assessing the microcirculatory flow in SSc patients. The OMERACT filter -including the domains of truth, discrimination and feasibility- was applied and a quality assessment was done by the \'Good Methods Checklist\'. To ease the comparison between studies the results were grouped per dynamic test situation: basal, cold/heat challenge and occlusion.
The literature search resulted in 4332 hits. Based on title and abstract screening 243 hits were retained and of these, 52 full texts described an assessment by LDF in SSc patients. Finally, 18 studies passed the quality assessment and form the object of this review. The review reveals that expert consensus is lacking on the face and content validity of LDF in SSc. The construct validity of LDF, on the other hand is partially validated. Conflicting results exist on the discriminant capacity of LDF in distinguishing healthy from diseased patients, primary from secondary Raynaud\'s phenomenon and in differentiating between disease subsets. Yet, complementing an LDF-measurement with a heat challenge, as well as the evaluation of the post-occlusive hyperaemic response, has the potential to elicit a difference between healthy and diseased patients. Lastly, data on the feasibility of LDF in SSc is lacking in the identified literature.
This systematic review emphasizes the very preliminary validation status of LDF in the assessment of the microcirculatory flow in SSc.

Metastatic carcinoma to the vulva is rare, where the incidence is believed to be between 5% and 8%. However, malignant tumors have been described in 3-11% of systemic scleroderma (SSc) cases. We report the case of one patient, a 66-year-old postmenopausal woman, whose medical history was marked with rheumatic vascular disease (systemic scleroderma) since 1993 without muscular, renal, cardiac lesions or HTA (arterial hypertension) and without tobacco history. The woman presented with a new vulvar mass of the right labia in December 2011 that had progressively enlarged in size. CT scan of the abdominopelvic region demonstrated a lobular mass of the right labia with central necrosis, 7 cm on the wide axis, and the rectum and the vaginal wall were normal. No inguinal or iliac lymphadenopathy was noted. An outpatient excisional biopsy revealed a poorly differentiated malignant tumor suggestive of carcinoma. IHC: CK7+/CK20-, estrogen receptors-, AE 1 AE 3+, vimentine+, S100-, Desmina-, CD34-, KI 67: 20%. The thoracic scan revealed a large mass of 4 cm × 3 cm in the right lung base with right paratracheal lymphadenopathy 3 cm × 2 cm. A bronchoscopy revealed discrete stenosis of the mediastinal portion of the right bronchial tree. The bronchial biopsy also revealed poorly differentiated lung carcinoma, non-small cell, which was identical with the vulvar tumor.
CONCLUSIONS: The presence of the single lung lesion with only one lymphadenopathy paratracheal with pathological and immunohistochemical (IHC) profile similar to the vulvar lesion, and a particular IHC profile with CK7+ and CK20- was detected - that is more specific to the primitive pulmonary cancer, and the presence of only one sarcoma marker vementine+, desmine and actine-. Also the presence of KI 67: 20%, predicted the proliferative and great metastatic power of the lung tumor was observed. Additionally, lung cancer was the most frequent type and may develop in scleroderma as reported in most studies. This allows to conclude for primitive lung carcinoma revealed with vulvar metastasis after elimination of the possibility of vulvar sarcoma. The patient was treated by chemotherapy (Taxol/Platin) with partial response from the lung after 3 cycles and palliative radiotherapy in the vulva with a good response. This case described primary lung carcinoma associated with scleroderma, revealed by a vulvar metastasis, which may be related to the aggressiveness of lung cancer when the lung fibrosis follow-up is not performed well to detect early the development of lung tumors in the patient with systemic scleroderma.
Le carcinome métastatique de la vulve est rare, son incidence est estimé entre 5% et 8%. D’autre part les tumeurs malignes ont été décrite dans 3-11% de la sclérodermie systémique (ScS) cas. Nous rapportons le cas d’une patiente, 66 ans ménopausée, ses antécédents médicaux ont été marquée par une maladie vasculaire rhumatismale (sclérodermie systémique) depuis 1993 sans atteintes musculaires, rénales, cardiaques ou HTA (hypertension artérielle) et sans histoiretabagique. La patiente a représenté une masse vulvaire de la lèvre droite de la vulve en Décembre 2011, qui avait progressivement augmenté de taille. La tomodensitométrie abdomino-pelvienne a montré une masse lobulaire de la lèvre droite avec une nécrose centrale, 7 cm l’axe le plus large, le rectum et la paroi vaginale étaient normale. Aucune adénopathie inguinale ou iliaque a été noté. Une biopsie-exérèse ambulatoire a révélé une tumeur maligne peu différenciée suggérant un cancer. IHC: CK7+/CK20−, récepteurs des oestrogènes−, AE 1 AE 3+, vimentine+, S100−, Desmina−, CD34−, KI 67: 20%. Le scanner thoracique a révélé une grosse masse de 4 × 3 cm au niveau de la base du poumon droit avec lymphadénopathie paratrachéaux droite de 3 × 2 cm. Une bronchoscopie a révélé: une sténose de la partie médiastinale de l’arbre bronchique droit. Et la biopsie bronchique a révélé un carcinome du poumon peu différencié, non à petites cellules, ce qui était identique à la tumeur vulvaire.
La présence de la lésion pulmonaire unique avec un seul lymphadénopathie paratrachéal avec à l’anatomopathologie et immunohistochimie (IHC) un profil similaire à la lésion vulvaire, et le profil IHC particulier avec CK7+ et CK20− qui sont plus spécifiques au cancer primitif pulmonaire, et la présence d’un seul marqueur de sarcome vementine+, desmine et actine−. Aussi la présence de KI 67: 20%, qui prédit le grand pouvoir prolifératif et métastatique de la tumeur pulmonaire. En plus le cancer du poumon est le type de cancer le plus fréquent qui peut se développer chez les patients sclérodermiques dans la plupart des études. Ces arguments ont permis de conclure au carcinome primitif du poumon révélé par des métastases vulvaire après élimination de la possibilité de sarcome vulvaire. Traités par chimiothérapie (Taxol/Platin) avec une réponse partielle au niveau du poumon après 3 cycles et radiothérapie palliative de la vulve avec une bonne réponse. Ce cas décrit un carcinome primitif du poumon associé à une sclérodermie systémique, révélé par une métastase vulvaire, qui peut être lié à l’agressivité du cancer pulmonaire lorsque le bon suivi de la fibrose pulmonaire n’est pas effectué pour le dépistage précoce des tumeurs pulmonaires développées chez des patients suivis pour sclérodermie systémique.