If the cervical spine is involved, particular attention is warranted during pregnancy. Subluxation is common, and pregnancy, at least theoretically, predisposes to this because of joint laxity. Importantly, there are anesthesia concerns during endotracheal intubation.
Following pregnancy in women with rheumatoid arthritis and its juvenile form, contraceptive counseling may include combination oral contraceptives. These are a logical choice because of their efectiveness and their potential to improve disease (Farr, 2010). That said, all methods of contraception are appropriate.
This is a chronic multisystem connective tissue disorder of unknown etiology. It is characterized by microvascular damage, immune system activation leading to inlammation, and excessive deposition of collagen in the skin and often in the lungs, heart, gastrointestinal tract, and kidneys. It is uncommon, displays a 5-to-1 female dominance, and typically afects those aged 30 to 50 years (Meier, 2012; Varga, 2015).
This strong prevalence of scleroderma in women and its greater incidence in the years following childbirth give credence to the hypothesis that microchimerism is involved as discussed earlier (p. 1139) (Lambert, 2010). Artlett and coworkers (1998) demonstrated Y-chromosomal DNA in almost half of women with systemic sclerosis compared with only 4 percent of controls. Rak and colleagues (2009b) identiied male microchimerism in peripheral blood mononuclear cells more frequently in women with limited versus diuse scleroderma-20 versus 5 percent.
The hallmark is overproduction of normal collagen. In the more benign form-Limited cutaneous systemic sclerosis-progression is slow. With diuse cutaneous systemic sclerosis, skin thickening progresses rapidly, and skin fibrosis is followed by gastrointestinal tract ibrosis, especially the distal esophagus (Varga, 2015). Pulmonary interstitial fibrosis along with vascular changes may cause pulmonary hypertension, which develops in 15 percent of patients. Antinuclear antibodies are found in 95 percent of patients, and immunoincompetence often develops.
Raynaud phenomenon, which includes cold-induced epi sodic digital ischemia, is seen in 95 percent of patients, and there may also be swelling of the distal extremities and face.
Half of patients have symptoms from esophageal involve ment, especially fullness and epigastric burning pain. Pulmo nary involvement is frequent and causes dyspnea. The 10-year cumulative survival rate is 70 percent in those with pulmo nary fibrosis, and pulmonary arterial hypertension is the main cause of death Goven, 2010; Varga, 2015). Women with lim ited cutaneous disease such as the CEST syndrome-falcinosis,
Baynaud phenomenon, .sophageal involvement, .clerodacyy, and .elangiectasia-have milder disease.
Overlap syndrome refers to systemic sclerosis with features of other connective tissue disorders. Mixed connective tissue disease is a term used for the syndrome involving features of SLE, systemic sclerosis, polymyositis, rheumatoid arthritis, and high titers of anti-ribonucleoprotein (RNP) antibodies (see Table 59-1). he disorder is also termed undierentiated connective tissue disease (Spinillo, 2008).
Although systemic sclerosis is incurable, treatment directed improve function. Renal involvement and hypertension are often comorbid. At times, ACE inhibitors may be required for blood pressure control despite their known teratogenicity. Scleroderma renal crisis develops in up to a fourth of these patients and is characterized by obliterative vasculopathy of the renal cortical arteries. his leads to renal failure and malignant hypertension.