Overview: Identifying and Managing 3 Scleroderma Emergencies


Thanks to the rampant spread of COVID-19, many events, symposiums, and clinics moved online. One was the 2020 ACR State-of-the-Art Clinical Symposium, in which participants discuss diagnostic information, treatments, and other issues pertaining to rheumatology. This year, Dr. Francesco Boin, MD, presented on how to identify and manage three scleroderma-related emergencies: critical digital ischemia, scleroderma renal crisis, and intestinal pseudo-obstruction. According to The Rheumatologist, understanding this can better patient care and thus improve patient outcomes.


Scleroderma is a group of rare diseases characterized by the stiffening/hardening of skin and connective tissues throughout the body. Though researchers know it is caused by excess collagen production, they are still unsure as to what causes that excess. Currently, scleroderma is split into 2 subsets:

  • Localized, a mild form in which only a few patches of skin or muscles are impacted.
  • Systemic, a more severe form which can widely affected the skin, lungs, kidneys, heart, blood vessels, muscles, joints, stomach, and bowels.

In some cases, scleroderma presents abnormally. As a result, while some experience hard and tight skin patches, others may experience symptoms such as:

  • A decline in kidney, heart, and lung function
  • Acid reflux
  • Esophageal damage
  • Problems with nutrient absorption
  • Raynaud’s phenomenon
  • Numbness and pain in the fingers and toes

Learn more about scleroderma here.

Identifying and Managing Rheumatological Emergencies

According to Dr. Boin, there are three main types of scleroderma-related emergencies: critical digital ischemia, scleroderma renal crisis, and intestinal pseudo-obstruction. In better understanding these issues, doctors will be better poised to improve patient health.

Critical Digital Ischemia

According to an article in Seminars in Arthritis and Rheumatism, critical digital ischemia is:

a rare, but serious complication of systemic sclerosis (SSc) [that] causes pain at rest and which compromises tissue viability as a result of impaired perfusion/oxygenation.

In layman’s terms, critical digital ischemia occurs when your digits (fingers and toes) aren’t getting enough blood supply. As a result, they become painful. Critical digital ischemia won’t respond to calcium channel blockers or other medications in the same way that Raynaud’s phenomenon might.

In addition to digital pain, patients may experience damaged blood vessels and arteries, as well as a change in digit color. According to Dr. Boin, “rest, warmth, and pain control with analgesics” is crucial to treatment. However, if the ischemia continues, patients may require surgery.

Although scleroderma renal crisis is not a common occurrence, it is a dangerous one. In fact, says Consultant360, this emergent situation is fatal in 10% of cases. SRC is:

characterised by new-onset malignant hypertension and progressive acute renal failure, often with associated microangiopathic haemolytic anaemia and thrombocytopenia.

Those with SRC may experience high blood pressure that can cause organ damage, renal failure, anemia, and low platelet counts. Although SRC presents as scleroderma’s first manifestation around 20% of the time, it may also appear as hypertension without renal failure, or vice versa.

Generally, SRC is serious enough that it requires hospitalization. Once diagnosed, patients should receive ACE inhibitors, calcium channel blockers, and other medication designed to lower their blood pressure.

Intestinal Pseudo-Obstruction

Similar to paralytic ileus, in which intestinal obstruction results from muscle paralysis, patients with intestinal pseudo-obstruction experience difficulty moving food through the digestive tract. While symptoms mimic that of an obstruction or blockage, patients with IP have no intestinal obstructions. Says the NIH:

The condition may arise from abnormalities of the gastrointestinal muscles themselves (myogenic) or from problems with the nerves that control the muscle contractions (neurogenic).

Patients with this emergent condition may experience:

  • Loss of appetite
  • Anorexia
  • Diarrhea
  • Unintended weight loss
  • Pain and bloating
  • Nausea and vomiting

Patients should not be treated with opiates, as they are a risk factor for IP. After reaching a diagnosis, treatments can / should include:

  • Surgery (in severe cases)
  • Nutritional support and diet assistance
  • Antibiotics
  • Bowl rest
  • IV-administered hydration

The 2021 ACR State-of-the-Art Clinical Symposium will take place in March in New Orleans. Interested? Click here to learn more.

Jessica Lynn

Jessica Lynn

Jessica Lynn has an educational background in writing and marketing. She firmly believes in the power of writing in amplifying voices, and looks forward to doing so for the rare disease community.

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