Brain (Cerebral) Involvement in Scleroderma

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Systemic Scleroderma
—Vascular Headache
—What is Vertigo?
Localized Scleroderma
Personal Stories
Related Links

Overview of Brain Involvement in Scleroderma

There are two main types of scleroderma: localized (such as morphea and linear) and systemic (such as CREST, limited, diffuse, and overlap.) (Also see What is Scleroderma?, Types of Scleroderma and Symptoms of Scleroderma)

Localized scleroderma, particularly linear scleroderma, has recently been associated with both focal and progressive brain lesions, progressive brain atrophy, epilepsy, neurological symptoms, intracerebral inflammatory processes, migraine headaches, and trigeminal neuralgia. Therefore, neuroimaging studies should be considered in all patients with linear scleroderma, and certainly when neurological symptoms occur. See Localized Scleroderma Brain Involvement.


Cerebritis is inflammation of the brain and can be caused by infection or inflammation from disease. Although more known in Lupus sufferers, it can affect patients with Scleroderma in the same way.

A presentation of cerebritis secondary to granulomatosis with polyangiitis (GPA) (wegener). GPA is a rare and treatable differential diagnosis for confused patients with acute or subacute neurological features and unusual MRI findings. PubMed, Case Rep Neurol Med.

(Case Reports): Indicating the dilemma in diagnosing lupus cerebritis. Lupus Cerebritis should be included in the provisional diagnosis of a female patient of reproductive age group, who presents with complicated neurological manifestations and with no clear-cut clinical, pathological, or image finding. PubMed, J Family Med Prim Care. (Also see Diagnosis of Lupus)

Systemic Scleroderma Brain Involvement

Systemic scleroderma is associated with brain (cerebral) problems, including poor circulation in the brain and neuropsychiatric problems including cognitive impairment.

Cognitive Dysfunction in Autoimmune Rheumatic Diseases. The control of systemic inflammation together with tailored behavioural cognitive therapies may benefit these patients. PubMed, Arthritis Res Ther, 2020 Apr 15;22(1):78.

Demyelinating syndrome in systemic sclerosis and neuromyelitis optica (NMO). The finding suggests that patients presenting with acute neurological manifestations get tested for NMO-IgG/AQP-4 antibodies. PubMed, BMC Neurol, 2019 Oct 14;19(1):234.

Effects of autonomic dysfunction (AD) on exercise tolerance in systemic sclerosis (SSc) patients without clinical and instrumental evidence of cardiac and pulmonary involvement. In SSc patients without cardiopulmonary involvement AD is associated with better exercise tolerance and cardiac function during physical effort. PubMed, Clin Exp Rheumatol, 05/04/2018. (Also see Cardiac (Heart) Involvement)

Olfactory function in systemic lupus erythematosus (SLE) and systemic sclerosis (SSc). A longitudinal study and review of the literature. Both SLE and SSc patients with longstanding disease had significant reduction in all stages of the Sniffin' Sticks test (TDI) that maintained stable over a 2–year period. PubMed, Autoimmun Rev, 02/11/2018. (Also see Lupus in Overlap)

(Case Report) Cerebral sinus thrombosis in scleroderma: a case report. We discuss a scleroderma patient with high titter of anticardiolipin antibody who developed to cerebral sinus thrombosis and cerebellum infarction. PubMed, Acta Med Iran.

Neuromuscular manifestations of systemic sclerosis (SSc) (scleroderma). Central nervous system (CNS) involvement due to SSc is rare, but complications of other organ damage may have neurologic manifestations. UpToDate.

(Case Report) Relapsing and Progressive Tumefactive Demyelinating Form of Central Nervous System Involvement in a Patient with Progressive Systemic Sclerosis. Conventional MRI was very useful to detect the CNS lesions, and diffusion tensor imaging and MR spectroscopy could be helpful to evaluate the severity and the change of white matter involvement. J Korean Soc Radiol.

Cognitive and cerebral hemodynamic impairment in scleroderma patients. These findings show the presence of reduced performances of executive functions in scleroderma patients. The associated alteration of cerebrovascular reactivity (CVR) in the absence of other apparent causes of cerebrovascular impairment suggests that cognitive problems may be related to an alteration in cerebral perfusion regulation specifically linked to the disease. European Journal of Neurology. (Also see Vascular Involvement)

What is a Vascular Headache?

Vascular Disease and Systemic Scleroderma. The vascular system is our circulatory system. It consists of all the vessels that carry blood and lymph through the body, to and from organs. Vessels include arteries, veins, and capillaries. Any condition that affects the vascular system, all of in part, is considered a vascular disease. ISN.

Vascular in Scleroderma
Types in Scleroderma
In Other CTDs
Personal Stories
Related Information

What is Vertigo?

What is Vertigo? Vertigo is a symptom, not a disease. The term vertigo refers to the sensation of spinning or whirling that occurs as a result of a disturbance in balance (equilibrium). It also may be used to describe feelings of dizziness, lightheadedness, faintness, and unsteadiness. The sensation of movement is called subjective vertigo and the perception of movement in surrounding objects is called objective vertigo. Healthline.

Overview of Localized Scleroderma Brain Involvement

Localized scleroderma has also recently been associated with brain involvement, particularly with linear scleroderma and the type known as scleroderma en coup de sabre or Parry Romberg's syndrome. Findings include focal and progressive brain lesions, progressive brain atrophy, epilepsy, neurological symptoms, intracerebral inflammatory processes, migraine headaches, and trigeminal neuralgia. Therefore, neuroimaging studies should be considered in all patients with linear scleroderma, and certainly when neurological symptoms occur.

Localized Scleroderma: Linear/En Coup de Sabre Linear Scleroderma can appear as a long streak resembling a deep sabre wound — often called "en coup de sabre." ISN.

Also see: Linear Scleroderma, En Coup de Sabre and Parry Romberg's Syndrome ISN.

Media and Personal Stories: Cognitive, Headaches and Vestibular Disorders

Personal Stories. There are many possible causes of cognitive problems, brain fog, headaches, migraines, dizziness, balance problems and vertigo. Just because these stories are listed here does not mean their particular symptoms are caused by scleroderma or by vascular complications of scleroderma or other illnesses. ISN.

Antiphospholipid Syndrome ISN.

Autoimmune Ear Disease ISN.

Cardiac Involvement ISN.

Chronic Fatigue Syndrome/Fibromyalgia ISN.

Gastrointestinal Involvement Gastrointestinal symptoms occur in a variety of neuromuscular diseases that may affect the neural access from the brain down to the peripheral nerve. ISN.

Scleroderma and Hypertension ISN.

Multiple Sclerosis ISN.

Neurological Involvement in Systemic Sclerosis ISN.

Raynaud's Raynaud's is an extreme spasm of blood vessels in response to cold or stress. The fingers and/or toes become white and/or blue, and may become red on re-warming. ISN.

Vasculitis is a general term for a group of diseases that involve inflammation in blood vessels. ISN.

Go to Brain Involvement: Stories

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