UNASSIGNED: Scleroderma is a multisystemic disorder characterised by inflammatory and vascular anomalies, and excess fibrosis. Progressive systemic sclerosis (PSS) mainly progresses with skin, joint, lung, heart, and kidney involvement. Involvement of cerebral vessels is rare in both localised scleroderma and PSS. Transient ischemic attack and stroke are rare complications of scleroderma.
UNASSIGNED: We present a 60-year-old stroke patient with localised scleroderma presenting with impaired speech, forgetting words, and occasional temporary memory loss.
UNASSIGNED: In the case we present, no pathology was found in the clinical and laboratory tests performed in terms of ischemic risk factors. Skin findings included contracture, skin biopsy results, and antibody positivity related to scleroderma. Given the current pathogenesis of scleroderma, the patient was suspected of having a stroke.

We present the case of a 44 year old woman with systemic sclerosis who presented with intense abdominal pain without signs of peritonitis. An abdominal computed tomography showed generalized intestinal dilation, intestinal pneumatosis and an extensive pneumoperitoneum. A diagnostic laparoscopy was performed but no perforation nor gastrointestinal leakage were found. Spontaneous pneumoperitoneum in patients with systemic sclerosis without visceral perforation is an extremely rare complication. Physicians must have a low threshold of suspicion for this entity when a patient with systemic sclerosis presents with spontaneous pneumoperitoneum in the absence of peritoneal signs.
Presentamos el caso de una mujer de 44 años con diagnóstico de esclerosis sistémica, quien presentó dolor abdominal intenso sin datos de irritación peritoneal. Una tomografía computarizada de abdomen mostró dilatación generalizada de asas intestinales, neumatosis intestinal y neumoperitoneo extenso, por lo cual se realizó una laparoscopía diagnóstica, sin encontrar sitio de perforación. El neumoperitoneo espontáneo en pacientes con esclerodermia sin evidencia de perforación visceral es una complicación extremadamente rara. El médico deberá mantener un alto índice de sospecha para esta condición ante un paciente con esclerosis sistémica que se presente con un neumoperitoneo espontáneo sin datos de irritación peritoneal.

Cryptogenic organising pneumonia (COP) is a form of idiopathic diffuse interstitial lung disease (ILD) that develops in response to a variety of unknown irritants. An essential component of the development of organising pneumonia (OP) is damage to type II pneumocytes and the alveolar basement membrane. An autoimmune illness called systemic sclerosis (SSc) has a significant death rate from cardiopulmonary involvement such as pulmonary hypertension and ILD. Arthritis is an autoimmune disorder, in which the patients experience extra-articular symptoms such as ILD during the course of their disease, and COP frequently coexists with these conditions. It is exceedingly uncommon for OP to occur as the initial sign of arthritis, and its clinical characteristics are still unclear. Scleroderma and inflammatory polyarthritis related to COP are presented in this report.

UNASSIGNED: This case highlights the need for further research to explore a potential link between systemic sclerosis and fistula development, and the importance of raising awareness among clinicians about this possibility for timely appropriate management.
UNASSIGNED: The primary diagnosis in this case is systemic sclerosis (scleroderma) in a 47-year-old female patient with various clinical manifestations, including vesicovaginal and vesicorectal fistulas. The medical interventions and diagnostic workup involved an electrocardiogram, cardiac echocardiogram, pulmonary function tests, beta blockers, aspirin, inhaled corticosteroids, albuterol, endoscopy, biopsy, treatment for Helicobacter pylori infection, fluconazole for candida esophagitis, endoscopic dilation for achalasia, anticholinergic therapy for mixed urinary incontinence, gabapentin for neuropathic symptoms, analgesia for knee pain, and psychiatric treatment with selective serotonin reuptake inhibitors. The outcomes described in the case report include the diagnosis of systemic sclerosis, the identification of vesicovaginal and vesicorectal fistulas, the patient\'s medical history and symptoms over the years, and various treatments and management strategies.

We present a case series of four patients with systemic sclerosis and skeletal myopathy. While idiopathic inflammatory myopathies, or myositis, are thought to be the most common type of muscle disease seen in systemic sclerosis, we highlight four cases where unique clinical findings and careful assessment ruled out myositis mimics. Key diagnostic tools that can be helpful for clinicians to diagnose a neuromuscular disease are also detailed in this report.

OBJECTIVE: The World Trade Center (WTC) attack in New York resulted in a dust plume containing silica, hydrocarbons, and asbestos. Autoimmune disorders have been reported among those with WTC site exposure. The characteristics of individuals developing systemic sclerosis (SSc) have not been previously described. The purpose of this study was to describe the features of patients with SSc with WTC exposure.
METHODS: Data were collected from 11 patients with SSc or SSc spectrum conditions who reported exposure to the WTC site. Seven patients completed an exposure assessment.
RESULTS: Of the 11 patients, the majority (n = 8) were female. The median (range) for age at diagnosis was 46 (36-75) years, time between exposure and first non-Raynaud phenomenon SSc symptom was 8 (1-19) years, and time between exposure and diagnosis was 11 (2-18) years. Fifty-five percent had SSc onset > 5 years from WTC exposure. Five patients had limited cutaneous SSc, 3 patients had diffuse cutaneous SSc, 1 patient with SSc features met criteria for mixed connective tissue disease (CTD), and 2 patients had undifferentiated CTD with features of SSc. Four patients had overlapping features with other CTDs. Interstitial lung disease (ILD) was present in 10 patients. Five of 11 patients had a history of tobacco use. Seven of 7 patients who completed the questionnaire reported other hazardous exposures outside of WTC. Of these, only 2 patients reported personal protective equipment use.
CONCLUSIONS: A high frequency of ILD and overlap features were observed among patients with SSc with WTC exposure. Future studies are needed to characterize this association.

Autologous hematopoietic stem cell transplantation (aHSCT) represents an effective treatment option in patients with severe forms of systemic sclerosis (SSc) by resetting the immune system. Nevertheless, secondary autoimmune disorders and progressive disease after aHSCT might necessitate renewed immunosuppressive treatments. This is particularly challenging when organ dysfunction, i.e., end-stage kidney failure, is present. In this case report, we present the unique case of a 43-year-old female patient with rapidly progressive diffuse systemic sclerosis who underwent aHSCT despite end-stage renal failure as consequence of SSc-renal crisis. Therefore, conditioning chemotherapy was performed with melphalan instead of cyclophosphamide with no occurrence of severe adverse events during the aplastic period and thereafter. After aHSCT, early disease progression of the skin occurred and was successfully treated with secukinumab. Thereby, to the best of our knowledge, we report the first case of successful aHSCT in a SSc-patient with end-stage kidney failure and also the first successful use of an IL-17 inhibitor to treat early disease progression after aHSCT.

Microstomia presents a challenge for the patient and dental provider. This report describes a partial digital workflow for the fabrication of a mandibular complete denture for a patient with microstomia. Computer-aided design and computer-aided manufacturing technology was utilized to 3D print a sectional custom tray with a unique design. The sectional custom tray was used to make a conventional border molded impression of the edentulous arch to fabricate a flexible complete mandibular denture for a 58-year-old female patient with scleroderma and microstomia. This treatment resulted in a successful prosthetic outcome and high patient satisfaction.

UNASSIGNED: Systemic sclerosis (SSc) is divided into three subtypes: limited cutaneous SSc (lcSSc), diffuse cutaneous SSc (dcSSc), and systemic sclerosis sine scleroderma (ssSSc). ssSSc is a rare subtype of SSc that presents with internal organ manifestations but no cutaneous findings.
UNASSIGNED: We report the case of a 58-year-old patient with a history of pulmonary hypertension who presented with symptoms of fatigue, inflammatory polyarthritis, and joint swelling. Following a comprehensive clinical examination and laboratory tests, the patient was diagnosed with ssSSc.
UNASSIGNED: Due to its atypical clinical course, we present this case report, which commenced with idiopathic pulmonary hypertension. Subsequently, after 7 months, the patient presented complaints of polyarthritis with positive antinuclear antibodies. Raynaud\'s phenomenon was identified 2 months later during the rheumatology clinic examination. Typically, the clinical course encompasses all three features simultaneously, without any gap between them.
UNASSIGNED: Diagnosis of ssSSc remains challenging, and it is essential to consider this disease form in all cases involving unexplained fibrotic involvement of the internal organs.

Immune checkpoint inhibitors are increasingly being used to treat various malignancies. Despite their efficacy, they are known to potentially cause immune-related adverse effects, including dermatological manifestations. A rare cutaneous immune-related adverse effect is scleroderma, which has been reported to occur with anti-programmed cell death-1 (PD-1) agents such as pembrolizumab and nivolumab. This may present with skin tightening and hardening at any point during or after immunotherapy. We present the case of a 54-year-old Caucasian woman who, following 16 doses of pembrolizumab for breast cancer, developed clinical features of scleroderma confirmed on histology. She was initially treated with oral corticosteroids, followed by oral psoralen-UVA, with poor response, but eventually improved with methotrexate. A literature review revealed 12 other cases of scleroderma following pembrolizumab treatment and 6 cases of scleroderma following nivolumab treatment. Males and females were both affected, and their ages ranged from 33 to 81 years. Scleroderma developed at different stages of pembrolizumab or nivolumab therapy. Although scleroderma is not commonly drug-induced, anti-PD-1 agents may be a rare cause and it is important to elicit an accurate drug history, including immunotherapy, in such cases.