Dr. Boin noted that atypical presentations of scleroderma renal crisis are possible as well. For example, patients in the early stage may have hypertension without renal failure or, in other cases, may manifest renal failure without hypertensive crisis. Less overt signs of malignant hypertension, such as asymptomatic pericardial effusion or arrhythmias, and signs of thrombotic microangiopathy can be present even in the absence of renal failure or hypertension.
When renal crisis is suspected, baseline laboratory studies, such as complete blood count, basic metabolic panel and urinalysis, must be collected for comparison to repeat testing as the disease progresses.
In most cases, hospitalization is required, and treatment with an angiotensin-converting enzyme (ACE) inhibitor should be started immediately, with the addition of angiotensin receptor blockers (ARBs), calcium channel blockers and any other antihypertensive medications needed to lower blood pressure by up to 20 mmHg per 24 hours. Other vasodilators, such as endothelin receptor antagonists and prostacyclin antagonists, may also be considered.
Dr. Boin advocates for renal biopsy in most cases to confirm the diagnosis, measure damage and gauge prognosis. This may be important if advance treatments, such as eculizumab or plasma exchange (when thrombotic thrombocytopenic purpura [TTP] or atypical hemolytic uremic syndrome [HUS] are present) are to be considered.
Intestinal Pseudo-Obstruction
For the final scleroderma-associated emergency, Dr. Boin discussed intestinal pseudo-obstruction. This clinical syndrome is characterized by obstructive symptoms despite the absence of a mechanical etiology. For patients with scleroderma, this disorder is due to malfunction of intestinal propulsion.2
Several clinical features often accompany the onset of intestinal pseudo-obstruction, including an established history of gastrointestinal dysmotility, appearance of dysphagia, loss of appetite, abdominal bloating and diarrhea, and weight loss. As the condition progresses, patients often experience regurgitation of food, nausea and vomiting, abdominal pain and distension, absent or metallic bowel sounds, and profound anorexia.
The clinical evaluation of patients with intestinal pseudo-obstruction may raise concern for an acute surgical abdomen. The exam and imaging studies must be carefully reviewed by all members of the medical team in such cases (i.e., surgery, gastroenterology and rheumatology).
Intestinal pseudo-obstruction has multiple risk factors, and Dr. Boin specifically pointed out that opiate use is high on this list. Whenever possible, opiates should be avoided in patients with scleroderma. Management should involve strict bowel rest (with nothing by mouth), nasogastric decompression, intravenous hydration and correction of any electrolyte abnormalities. Antibiotics, such as rifaximin or ciprofloxacin, may be indicated to help alleviate features of small bacterial intestinal overgrowth (SIBO). Prokinetic agents are often helpful.
