The contemporary management of systemic sclerosis

Abstract

Systemic sclerosis (SSc) is a rare autoimmune connective tissue disease characterized by vascular dysfunction, fibrosis, inflammation and autoantibodies. The pathophysiology of SSc is not completely understood, and many patients acquire organ or tissue damage despite advances in treatment. Current treatments target affected organs with modest improvements. Areas covered: This review evaluates several treatment strategies for SSc based on involved organs including skin, pulmonary, cardiac, renal, musculoskeletal, and gastrointestinal. Currently, pulmonary hypertension and interstitial lung disease are the primary causes of increased mortality. We will outline an approach to treatment of SSc based on disease manifestations and current evidence. Expert commentary: This complex disease is currently treated with therapies developed for similar indications such as for vascular manifestations of SSc using idiopathic pulmonary arterial hypertension treatments. Future directions in this field may include combination and maintenance therapy that is currently used in other autoimmune diseases, and tailoring these treatments according to the patients' phenotype. This will hopefully increase the efficacy of available treatments and decrease mortality from SSc.

Neurological, cardiac, musculoskeletal, and renal manifestations of scleroderma along with insights into its genetics, pathophysiology, diagnostic, and therapeutic updates

“…The prognosis of SSc myositis is fatal if cardiac or lung involvement as they cause severe functional glitches as stated above, sans organ acquaintance is not problematic to patients 42 . Unlike skeletal, myositis responds well to corticosteroids, glucocorticoids, methotrexate, rituximab, IVIG, and immunosuppressive therapy only for inflammation sans necrosis 49–51 …”

“…Various presentations of kidney pathology in SSc are hypertensive SRC, normotensive SRC, isolated reduced glomerular filtration rate, myeloperoxidase anti‐neutrophil cytoplasmic antibody‐glomerulonephritis (MPO‐ANCA GN), reduced renal function reserve and disparate renal vascular resistance indices 104 . The basic pathway of renal crisis is thrombotic micro‐angiopathy (hemolytic anemia), acute pulmonary edema, malignant hypertension >150/90 mmHg (hyperreninemia), and progressive acute kidney injury, 30,105 50% of patients have worsening renal function 51 . Biopsy findings indicate the renal plasma flow measurements that vascular intimal proliferation leads to typical onion bulb‐like lesions characterized by vessel narrowing and resting renal blood flow (RBF) 84,106,107 …”

“… 42 Unlike skeletal, myositis responds well to corticosteroids, glucocorticoids, methotrexate, rituximab, IVIG, and immunosuppressive therapy only for inflammation sans necrosis. 49 , 50 , 51 …”

“…42 Unlike skeletal, myositis responds well to corticosteroids, glucocorticoids, methotrexate, rituximab, IVIG, and immunosuppressive therapy only for inflammation sans necrosis. [49][50][51] 4.2.3 | Tendon involvement TFR, tenosynovitis, and tendon rupture are major pathologies; the prevalence rate is minimal, with just 11% in SSc; antibodies involved are anti-topo I, anti-centromere, anti-scl70, anti-RNAP III. The areas highly involved are ankles, anterior and posterior tibial, peroneal, and Achilles, followed by extensor or flexor tendons of fingers, knees, wrists, hand, elbows, triceps and shoulders, scapular, trochanteric, PIPs, MCPs, toe rubs.…”

Gut Disease in Systemic Sclerosis—New Approaches to Common Problems

“…Symptoms of GIT involvement can be the presenting feature in 10% of SSc patients, and occurs during disease course in up to 95% of SSc patients (1). In addition to the enormous morbidity associated with SSc-related GIT involvement, there is a 6-12% mortality attributed to end-stage GIT manifestations in SSc patients (2,3) While there are some recent advances in certain aspects of SSc management, predominantly with a focus on tailoring treatment to the individual patient phenotype, there are challenges to studying GIT involvement in SSc (4). In particular, GIT involvement can be heterogeneous in clinical presentation and disease course.…”