Systemic sclerosis (SSc), or scleroderma, is a multisystem disease process that can result in significant end-organ damage if left undiagnosed or untreated. While some manifestations are well-known and widely researched, other presentations of SSc, including the presentation of our patient, require further investigation. Though many non-pulmonary and non-dermatologic manifestations lack widespread recognition, such presentations are important to recognize clinically in order to adequately investigate and appropriately treat. Fibrotic changes affect not only the skin but also the myocardial conduction system which can result in chronic systolic heart failure and significant conduction delays. This report is a case of newly diagnosed scleroderma that presented with worsening dyspnea and activity intolerance who was discovered to have new onset prolonged PR interval, right bundle branch block, and left anterior fascicular block. After a comprehensive workup, the patient was diagnosed with scleroderma and underwent treatment by a multidisciplinary team.

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Numerous pulmonary conditions, such as aspiration pneumonia and acute respiratory distress syndrome (ARDS), may result from aspiration of gastric or oropharyngeal contents passing into the lower respiratory tract. ARDS is a type of diffuse lung injury that is distinguished by the abrupt onset of extensive pulmonary inflammation accompanied by the failure of multiple organ systems. Systemic sclerosis is an uncommon connective tissue disorder that presents with skin thickening, the etiology of which remains unknown. Esophageal luminal dilatation is observed in the distal third of the esophagus in most cases of systemic sclerosis. This dilatation is primarily attributed to the greater abundance of smooth muscle fibers in this area. Here, we present the case of a 70-year-old female patient who was diagnosed clinically with diffuse systemic sclerosis and fulfilled the 2013 European League Against Rheumatism/American College of Rheumatology classification criteria. She had esophageal dilatation, with an esophageal luminal diameter measured at the upper, middle, and lower thoracic esophagus of 2.5 cm, 2.5 cm, and 3.5 cm, respectively. The patient was admitted to the intensive care unit (ICU) due to ARDS from aspiration pneumonia. Our patient\'s complicated condition at the time of ICU admission with ARDS secondary to aspiration pneumonia was primarily due to esophageal dilatation and reflux. Aggressive anti-reflux pharmacotherapy and bed elevation may be beneficial in preventing pulmonary injury caused by aspiration. Esophageal complications are common in such patients and can have a substantial impact on the prognosis and quality of life. Regular medical attention is necessary to identify and manage any potential issues.

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.

BACKGROUND: Systemic scleroderma (SSc) is an insidious autoimmune connective tissue disorder with multiorgan involvement. Renal involvement is one of the important causes of morbidity and mortality in scleroderma; however, nephrotic syndrome is reported rarely in association with SSc. We present a patient with SSc who developed focal segmental glomerulosclerosis (FSGS) as a complication of scleroderma.
METHODS: A 59 year old Caucasian female patient, with a known history of diffuse systemic sclerosis from 8 years, presented to our clinic with symptoms of anasarca and weight gain. Her physical examination was unremarkable except for periorbital and extremity edema. Her biochemistry assessment revealed decreased serum albumin levels and elevated serum creatinine levels. A renal biopsy was performed, which showed histopathological patterns of FSGS type of nephrotic syndrome. After administration of high doses of steroid and rituximab in the course of her treatment for 6 months, her symptoms and proteinuria were improved without the occurrence of scleroderma renal crises.
CONCLUSIONS: SSc is a complex multisystemic autoimmune disorder. SRC is the most prominent renal involvement in SSc, but other renal pathologies may also occur. Each patient should be precisely investigated since managing these renal conditions can differ significantly. Nephrotic syndrome is a rare complication of SSc, which could be managed with prompt diagnosis and steroid administration.

Fibroadenoma (FA) of the breast is a benign fibroepithelial lesion rarely showing atypical epithelial overgrowth. We present the case of a 50-year-old Japanese woman with sclerotic FA with atypical ductal hyperplasia (ADH)/ductal carcinoma in situ (DCIS). A small mass was detected during clinical examination in the upper lateral area of the left breast. Hematoxylin and eosin stain section of a breast needle core biopsy specimen showed trabecular growth of atypical epithelial cells without distinct myoepithelial lining in the sclerotic stroma. Initial pathological diagnosis of the biopsy specimen was invasive carcinoma of no special type. The surgical specimens included a well-bordered nodular lesion with similar histological findings to that of the biopsy specimen, but, the myoepithelial lining was highlighted by cytokeratin 5 (CK5) immunohistochemistry. The tumor cells were diffusely ER-positive and completely negative for CK5 in immunohistochemical staining. Final diagnosis based on the results of immunohistochemical staining and consultation between two breast pathology specialists was the lesion as sclerosing FA with ADH/DCIS. Awareness of the unique histological subtype of FA is important to avoid pathological misdiagnosis and clinical overtreatment.

Systemic sclerosis, an autoimmune disease characterized by fibrosis and vasculopathy of the skin and other multiple organs has been associated with an increased risk of malignancy. We present the case of a 74-year-old woman who had diffused cutaneous systemic sclerosis and uterine cervical cancer. The patient was initially diagnosed with stage IIB squamous cell carcinoma and concurrent chemoradiotherapy was planned. However, cisplatin could not be administered due to acute renal failure, so the patient was treated solely with radiotherapy. However, complications of systemic sclerosis progressed rapidly, and the patient died 63 days later from pulmonary edema. An autopsy later revealed that uterine cervix had primary signet ring cell carcinoma. We suspected that this patient had a combination of signet ring cell carcinoma and squamous cell carcinoma, with squamous cell carcinoma disappearing after radiotherapy. This case highlighted the importance of systemic management for cancers associated with systemic sclerosis.

A 42-year-old female with a past medical history significant for scleroderma and extensive tobacco use presented with a dry cough and pleuritic chest pain. Further workup was significant for leukocytosis, macrocytic anemia, left lower lung mass, bilateral supraclavicular, hilar, and mediastinal lymphadenopathy. After a comprehensive rheumatologic workup was completed, the patient was found to have strongly positive antinuclear antibody (ANA) and negative scleroderma-specific antibodies with fluorescent ANA indicating a nucleolar pattern. We present a case of paraneoplastic scleroderma in the setting of lung adenocarcinoma which emphasizes the bidirectional relationship that exists between malignancy and rheumatic diseases.

Systemic sclerosis (SSc) is a complex disease characterized by vascular injury with endothelial cell and platelet activation, immune dysregulation with inflammatory cytokines and fibroblast activation. The Raynaud phenomenon and puffy hands and fingers are common early manifestations of the disease that have a negative impact on patients\' quality of life. Vasodilators such as calcium channel blockers, PDE5 inhibitors, and prostacyclin analogs are recommended treatments, but they often have side effects and are not always effective. Ozone is an oxygen donor, an immunomodulator, an inducer of antioxidant enzymes and the endothelial nitric oxide synthase, a metabolic booster, and a stem cell activator. I report the case of a scleroderma patient treated effectively with autohemotherapy with ozone and a clear reduction of Raynaud\'s episodes and resolution of the edema of the hands. Furthermore, the capillaroscopic evaluation showed a rapid modification of the microcirculation which remained unchanged for months. Ozone therapy is effective to treat the Raynaud phenomenon and hand edema and should be considered, at least, as a complementary therapy to the standard of care, especially in patients who are unresponsive or with frequent adverse drug reactions. Further studies will be needed to confirm the efficacy of ozone therapy in scleroderma vasculopathy.

Myasthenia gravis (MG) is an autoimmune illness that causes neuromuscular junctions to be damaged by anti-acetylcholine receptor antibodies. It is a very rare condition that is more common among women. Fatigable fluctuating diplopia or ptosis is the characteristic early appearance of this condition. Dysphagia or dysphonia may be present in rare cases. This illness can affect any group of skeletal muscles, including those in the neck and upper limbs. It can also affect the muscles that help you breathe, which can lead to breathing failure. We present a case of a 20-year-old female diagnosed with mixed connective tissue disease presenting with acute respiratory failure as the initial presentation of MG. Clinicians have to have a high index of suspicion for myasthenia when patients arrive with fatigable muscle weakness. This will cut down on the amount of money spent on investigations and the risk of morbidity.