Systemic sclerosis: Clinical manifestations, anesthetic and orthopedic considerations in a patient

Abstract

HighlightsSystemic sclerosis is a very rare progressive multisystem autoimmune disorder affecting multiple internal organs.Presents a challenge to the physician, anesthetist and operating surgeon involved in medical care of these patients.A multidisciplinary approach should be followed with all of these patients to avoid catastrophic results.We intend to highlight the medical, anesthetic and surgical challenges faced by our team in the management process.We combined the case report with detail review of this rare disease from text books and recent literature.

Computed tomography trachea volumetry in patients with scleroderma: Association with clinical and functional findings

“…Consistent with our findings, Kim et al [ 48 ] found an association between the fibrosis score detected by CT using a computer-assisted method and the mRSS of patients with scleroderma both at baseline and during a 12-month follow-up period. This reinforces the idea that the measure of eccentricity may be a marker of the severity of the process of collagen hyalinization and abnormalities in the elastic tissues that surround the neck and may extend to the intrathoracic structures of patients with scleroderma [ 42 ]. Considering that future directions for the management of patients with scleroderma, including epigenetic modulation and antifibrotic or biological therapy, are being discussed [ 49 , 50 ], we think that the parameters provided by the measurement of tracheal geometry can contribute to the evaluation of the outcomes.…”

“…In scleroderma, cutaneous involvement occurs due to the increased thickness and hardness of the skin, which leads to shrinking of the skin in deeper structures; although cutaneous involvement is usually most prominent on the face and hands, these abnormalities may extend to the upper chest [ 42 , 43 ]. Some studies have shown that subclinical involvement of the upper chest is detectable by high-frequency ultrasound even with normal palpation [ 44 , 45 ].…”

Clinical utility of ozone therapy for musculoskeletal disorders

“…Systemic sclerosis is a chronic tissue disease characterized by vascular abnormalities in the joints, internal organs, and fibrosis. 56 The etiology and clinical manifestations of scleroderma are still not understood; this is why it is difficult to treat systemic sclerosis. 56 The inflammatory processes can be limited by O 3 's potential in reducing the proliferation of neutrophils and mastocytes, increasing concentration of prostacyclin 6-keto-prostaglandin F1α (6-keto-PGF1α), impeding the release of acute phase proteins, and decreasing the concentration of prostacyclin (PGF)2α resulting from the oxygen radicals on arachidonic acid.…”

“… 56 The etiology and clinical manifestations of scleroderma are still not understood; this is why it is difficult to treat systemic sclerosis. 56 The inflammatory processes can be limited by O 3 's potential in reducing the proliferation of neutrophils and mastocytes, increasing concentration of prostacyclin 6-keto-prostaglandin F1α (6-keto-PGF1α), impeding the release of acute phase proteins, and decreasing the concentration of prostacyclin (PGF)2α resulting from the oxygen radicals on arachidonic acid. 57 The results from this study have proved to slow down the progression of the illness by limiting the activation of the immune system.…”

Chronic Intestinal Pseudo-Obstruction in Systemic Sclerosis: An Uncommon Presentation

“…SSc is a complex multisystem autoimmune disease that is described pathologically by variable fibrosis of the skin and internal organs, and extensive vasculopathy [ 5 ]. The disease is frequently complicated by small and large bowel involvement, characterized by reduced peristalsis and hypomotility with resulting stasis, intestinal dilatation, and, occasionally, CIPO.…”