Voices of Scleroderma Volume 3
Contents:
Patients who have SSc may be classified in distinct clinical subsets that have characteristic patterns of skin and internal organ involvement and survival rates. In this article, we discuss the classification and diagnosis of SSc, the clinical manifestations—ranging from Raynaud phenomenon to skin, GI, lung, kidney, and heart involvement—the cause and pathogenesis, and evaluation.
We also describe therapies for the disease and its complications. The 2 main forms of SSc that affect adults are limited cutaneous lcSSc and diffuse cutaneous dcSSc Table 1 ; classification is determined by the extent and pattern of skin involvement. Facial involvement may be seen in either form.
However, many patients do not fit neatly into this dichotomous pattern. Up to 7 clinical subsets may be identified according to the presence of distinct serum autoantibodies; 4 subsets may be identified on the basis of gene expression patterns in skin. Although skin tightening often is of greatest concern to patients, internal organ dysfunction is common in the limited and diffuse forms and is potentially life-threatening.
A small subset of patients have scleroderma sine scleroderma and lack skin tightening but experience characteristic internal organ dysfunction. Localized forms of scleroderma affect mostly children and are not associated with significant internal organ complications see Table 1.
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In addition, scleroderma mimics that are associated with skin induration but lack internal organ involvement need to be considered in establishing the diagnosis of SSc. Skin induration in the absence of Raynaud phenomenon, or sparing the fingers, is unlikely to be SSc.
Signs and symptoms related to gastroesophageal reflux disease GERD may bring patients with undiagnosed SSc to medical attention; initial symptoms may include chronic dry cough, hoarse voice, heartburn, pulmonary symptoms eg, dyspnea on exertion or shortness of breath , arthralgias and diffusely swollen hands, and skin problems eg, dry, itchy, hyperpigmented or hypopigmented skin.
Misdiagnosis of undifferentiated polyarthropathy or carpal tunnel syndrome in patients who have early SSc is not uncommon. A diagnosis of Raynaud phenomenon is made when a patient provides a history of acral skin color changes precipitated by cold or emotional stress. In contrast to primary Raynaud syndrome, which typically presents during adolescence and does not lead to ischemic complications, secondary Raynaud phenomenon occurs later and often is complicated by ischemic tissue damage. Physical findings include reversible cyanosis and manifestations of ischemic damage, such as digital pitting, abnormal nail fold capillaroscopy, ischemic ulcers, and pterygium inversus unguis Figure 1.
Scleroderma, meaning hard skin, is the clinical hallmark of SSc.
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Skin changes usually begin on the fingers and hands; early swelling often is attributed to arthritis. Gradually, skin on the fingers sclerodactyly , hands, and forearms becomes indurated.
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Over time, the process may stabilize lcSSc or continue to progress centripetally to include the up-per arms, chest, neck, abdomen, and trunk dcSSc. Pigmentary changes are common and may be accompanied by intense pruritus and hair loss.
Physical findings include flexion contractures of the proximal interphalangeal joints; thickened, tight, and shiny skin on involved areas; and reduced oral aperture and deepening of perioral facial folds radial furrowing Figure 2. Telangiectasias often occur on the face, buccal mucosa, chest, and hands. The GI tract is the second most frequently involved organ in patients who have SSc; the entire tract from mouth to anus may be affected. Considerable pathology may be caused by esophageal and intestinal dysmotility, as well as GERD, even in asymptomatic patients. Esophageal disease is especially common in patients who have the CREST calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia variant of lcSSc.
Because esophageal disease may be asymptomatic, acid suppression therapy should be started in all patients; in theory, such therapy may prevent stricture formation, aspiration pneumonitis, and Barrett esophagus. However, the precise role of acid suppression therapy in halting the progression of esophageal or pulmonary disease has not been established. Dysmotility of the intestinal tract may result in pseudo-obstruction and small-bowel bacterial overgrowth with vitamin B12 and folate deficiency, and malabsorption.
Endoscopic laser coagulation and obliteration of vascular ectasia decrease the risk of rebleeding. Chronic constipation is common and may result in intestinal impaction. A diagnosis of intestinal pseudo-obstruction often is made at the time of laparotomy, although nonsurgical treatments—including bowel rest, antibiotics, and judicious use of promotility agents—often are effective. Smooth muscle dysfunction in the anus causes fecal incontinence and is a common problem.
Less common GI manifestations of SSc include asymptomatic pneumatosis cystoides intestinalis and wide-mouthed saccular diverticula of the colon. The diagnosis of ILD often is made with screening high-resolution CT HRCT scans of the chest that demonstrate reticular opacification of the lung base or ground-glass opacification.
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More advanced ILD is associated with radiographic findings of honeycombing, traction bronchiectasis, and bilateral subpleural fibrosis most prominent in the lower lung zones. Unilateral or upper lobe abnormalities that are seen on HRCT scans may suggest infection or malignancy and may require evaluation by bronchoscopy, bronchoalveolar lavage BAL and, in some cases, open lung biopsy. D L CO ratio lower than 1: Patients with both limited and diffuse forms of SSc are at risk for PAH, which may develop insidiously, in spite of having stable skin disease.
Risk factors for severe PAH include limited cutaneous disease, older age, and elevated pulmonary artery pressures at initial evaluation. D L CO ratio greater than 1. However, they are useful screening tools when performed serially as part of regular clinical assessments. The only way to directly measure pulmonary artery pressures and evaluate response to treatment is right heart catheterization. PAH is present when mean pulmonary artery pressure, as measured at right heart catheterization, exceeds 25 mm Hg.
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Left heart catheterization may be performed concomitantly with right heart catheterization to rule out coronary artery disease and left ventricular diastolic dysfunction, which can cause pulmonary venous hypertension. SRC is characterized by a sudden onset of malignant hypertension that may be accompanied by chest pain with decompensated congestive heart failure CHF and papilledema.
Laboratory studies show proteinuria, microangiopathic anemia, microscopic hematuria, and progressive oliguric renal failure. The pathogenesis of SRC is attributed to endothelial cell injury that leads to intimal thickening in small renal arteries as well as platelet aggregation.
Luminal narrowing decreases renal perfusion, which further activates the renin-angiotensin system.
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Sinikka Kurri what this? Shipping times range from 2 weeks to 6 months, depending on the countries. If your story was submitted for Volume 3, please email Judith if your shipping address has changed since then. It is simply an unbeatable book for scleroderma patients, caregivers, and their friends, families and doctors! Laszlo Czirjak on Scleroderma-Like Disorders , Francisco Castellanos , and Carol Langenfeld —plus over intriguing patient and caregiver and stories in remembrance, from 16 countries!
Order your copies today! She is the author of three fiction novels. Her latest novel, Mind Blindness , was released in July Judith volunteered to preserve the sclero.