Rheumatologic Issues in Women and Rehabilitation

This article discusses rheumatologic conditions in women including rheumatoid arthritis, lupus, Sjogren’s syndrome, inflammatory myopathies, systemic sclerosis, and polymyalgia rheumatica. These conditions, often affecting muscles, joints, and other organ systems, require early diagnosis and multidisciplinary management. Treatment includes medications, braces, therapy services, education, and lifestyle modifications including energy conservation techniques. These conditions can also impact pregnancy and require close monitoring and careful disease control. It also highlights the benefits of pulmonary rehabilitation that can be helpful for patients with chronic respiratory disease secondary to their rheumatologic conditions.

Key points

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Rheumatologic conditions frequently affect women more commonly than men, including most connective tissue diseases such as rheumatoid arthritis and lupus.
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Rheumatoid arthritis presents with symmetric joint inflammation leading to joint damage and deformities if not treated early. Ankle and foot involvement is commonly overlooked.
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Systemic lupus erythematosus primarily affects young women and can involve various organ systems, leading to significant morbidity and mortality.
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Inflammatory myopathies are more common in women and can present with progressive proximal muscle weakness and extramuscular manifestations, requiring immunosuppressive therapy and rehabilitation.
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Systemic sclerosis predominantly affects women and can lead to skin and visceral organ fibrosis, with significant impacts on quality of life and survival.

Abbreviations

ADLs	activities of daily living
ANA	antinuclear antibody
CTDs	connective tissue disorders
DM	dermatomyositis
DMARDs	disease-modifying antirheumatic drugs
EMG	electromyography
HRQoL	health-related quality of life
IgG	immunoglobulin G
IL	interleukin
ILD	interstitial lung disease
JIA	juvenile idiopathic arthritis
lcSSc	limited cutaneous systemic sclerosis
MCP	metacarpophalangeal
OA	osteoarthritis
OT	occupational therapy
PIP	proximal interphalangeal
PM	polymyositis
PMR	polymyalgia rheumatica
PR	pulmonary rehabilitation
pSS	primary Sjogren’s syndrome
PT	physical therapy
RA	rheumatoid arthritis
RF	rheumatoid factor
ROM	range of motion
SLE	systemic lupus erythematosus
SSc	systemic sclerosis

Introduction

Rheumatologic conditions affect the joints, muscles, and connective tissues, frequently impairing musculoskeletal function and impacting functional mobility and activities of daily living (ADLs). Beyond the local effects causing muscle weakness, joint pain, stiffness, swelling, and instability, many of these conditions may have more systemic involvement with negative effects on the central nervous system and cardiopulmonary system, which increases risk for further reduced overall function and quality of life. These conditions can affect individuals of any gender; however, certain conditions tend to be more prevalent in female individuals or present differently in women. Appropriate diagnosis and treatment plans in conjunction with effective rehabilitation management with an emphasis on understanding the unique challenges faced by women with rheumatologic conditions are important to promote optimal health.

Background

Rheumatic disorders, including rheumatoid arthritis (RA) and other connective tissue disorders (CTDs), affect women more commonly than men. Younger women are at 3 times higher risk of developing more severe and deforming RA than men. Up to 80% of patients who develop CTDs such as scleroderma are women in their prime adult years and beyond. Another similar CTD is dermatomyositis (DM), where the prevalence is twice as high in women of childbearing age. Patients with these disorders have higher risk of developing lung disease, including interstitial lung disease (ILD). ^,

Many patients with rheumatism have systemic inflammation, as well as other clinical features including fatigue, stiffness, muscle weakness, joint changes causing pain, instability, loss of range of motion (ROM) leading to contracture development, and emotional issues that may negatively influence their general wellness and cause disability. These patients benefit from early intervention and management, including rehabilitation and exercise. These have demonstrated improved muscle and joint function, bone strength, aerobic and cardiopulmonary function, and improved health-related quality of life (HRQoL) measures, all of which improve self-esteem. There is evidence to support the beneficial effects of exercise through myogenic and vascular mechanisms on sleep, pain, fatigue, and bowel flora. ^,

Discussion

We will review rheumatologic conditions that have a greater incidence or more unique presentation in women. We will discuss features of the disease conditions that may include presentation, diagnosis, management, and rehabilitation to reduce disease burden or progression of disease, improve pain, maintain function, and optimize quality of life.

Rheumatoid Arthritis

RA most commonly affects the small joints but can also affect intermediate and large joints, particularly in juvenile idiopathic arthritis (JIA). RA affects women more commonly than men in adult onset (3:1 female:male) and in all juvenile onset forms except for enthesitis-related JIA (1.5:1 male:female) and systemic JIA (1:1 male:female). Population studies in Minnesota show a rising trend in the incidence of RA in women more recently ( Fig. 1 ). Autoantibody production may predate clinical arthritis by up to 5 years or more, but the transition from this preclinical stage to early and then chronic arthritis, in terms of synovial inflammation and joint damage, can be rapid. Beginning treatment within 12 weeks of clinical onset has significant benefit in achieving remission. Later time to diagnosis, later start of disease-modifying antirheumatic drugs (DMARDs) and seropositivity (positive rheumatoid factor [RF] and anticyclic citrullinated peptide antibodies) are associated with more difficult to treat disease and increased risk of joint damage, deformity, and extra-articular manifestations.

The hallmark of RA is symmetric joint inflammation (pain, stiffness, swelling, and redness) that is frequently worse in the morning and improves after greater than 1 hour of activity. Periarticular erosions at the wrists, metacarpophalangeal (MCP) joints, proximal interphalangeal (PIP) joints, and metatarsal heads can aid in diagnosis as compared to sites more prevalent in gout or erosive osteoarthritis but are not pathognomonic. Carpal tunnel syndrome is common at diagnosis or with active disease, due to inflammation in the wrist. Classic RA hand deformities include radial deviation at the wrist, volar subluxation and ulnar deviation at MCP joints, and swan neck and boutonniere deformities of fingers. Wrist hand orthoses and finger orthoses and splints ( Fig. 2 ) can be used to relieve pain, stabilize joints, and improve function. The swan neck ring splint prevents PIP joint hyperextension, and the boutonniere ring splint prevents PIP joint flexion. Some women with RA may prefer ring splints that resemble jewelry where they are made of metal (silver or gold) and may have a decorative pattern or include gemstones.

Up to 89% of patients with RA will experience ankle or foot symptoms. Despite the importance of the feet in patient-reported outcomes and observed joint damage and disability, disease-specific measures such as the Disease Activity Score (of 28 joints) and Clinical Disease Activity Index do not include the foot joints! Metatarsophalangeal joints are often affected early. Tenosynovitis, rheumatoid nodules, and inflamed bursae are common, and atrophy of fat pads can occur. Cartilage loss and changes in weight-bearing joints in the foot ultimately leads to valgus and hindfoot deformity posteriorly, and fibular deviation anteriorly. Tarsometatarsal joint subluxation can result in a “rocker-bottom” foot deformity similar to Charcot joint. Observation for characteristic deformities such as metatarsal head subluxation, hallux valgus deformity, hammertoes and toe clawing, metatarsal squeeze testing, and assessment for rheumatoid nodules or Morton’s neuroma along the plantar aspect, should be included in physician evaluation of women with RA, especially those that report foot pain or issues.

Insoles, foot orthoses, and shoe modifications including additional depth (to accommodate custom-molded foot orthotics), high toe box (for hammer toes), and wide toe box (for bunion/hallux valgus) can make a dramatic difference for quality of life by reducing pain, improving gait mechanics, and avoiding the need for corticosteroid injections, which carry a risk of tendon rupture and fat pad necrosis. ^, Shoe modifications include inner sole excavation relief (usually under metatarsal heads), metatarsal pads (placed proximal to the metatarsal heads and transfers load to the metatarsal shaft and relieves stress/pressure at metatarsal heads); Fig. 3 , toe crests (relieves pressure at distal end and plantar surface for hammertoes), and foot orthoses. A rocker bottom shoe may also be beneficial to reduce pain and improve gait mechanics ( Fig. 4 ).

Systemic or intra-articular glucocorticoids may be helpful with acute inflammation but due to their toxicity profile, chronic use should be avoided. Therapeutic treatment options continue to expand for RA and include conventional synthetic DMARDs such as methotrexate, oral small molecules such as janus kinase inhibitor (JAK) inhibitors, and biologic medications targeting tumor necrosis factor, interleukin (IL)-6r, IL-1r, and B cell marker (CD20).

Acutely inflamed joints should be rested with relative rest. Nighttime splints and immobilizers, allowing for local rest of an inflamed joint, can be helpful in the short term; however, complete immobilization as was popular prior to the introduction of DMARDs is counterproductive and causes decline in muscle strength. Twice daily full and slow passive ROM should be done to prevent soft tissue contracture. Isometric strengthening is recommended as this causes the least amount of periarticular bone destruction, pain, and joint inflammation especially during an acute flare; additionally, it helps to maintain and restore strength by generating muscle tension with minimal work, fatigue, and stress. Isotonic strengthening exercises are recommended after achieving remission. Aquatic therapy can improve pain while providing joint and muscle support due to buoyancy of water.

Physical modalities for inflamed joints include therapeutic cold or cryotherapy to reduce pain and swelling (but avoid in patients with Raynaud phenomenon). Therapeutic heat should be avoided in acutely inflamed joints as it increases collagenase enzyme activity that causes increased joint destruction; however, after acute inflammation improves, superficial heat including moist heat, paraffin wax ( Fig. 5 ), and fluidotherapy can decrease pain and increase collagen extensibility for symptom management. Orthoses for distal upper and lower extremity joints can also be helpful to provide pain relief, decrease joint motion (joint stabilization), reduce weight through joint, facilitate relative rest, decrease inflammation, and support improved biomechanics to allow for better mobility and ADLs (see Fig. 2 ). Assistive and adaptive devices can be recommended and utilized to improve function and compensate for impairments allowing for increased independence ( Fig. 6 ).

As with most CTDs, pregnancy outcomes in RA are improved with better disease control. Contraception and the care of pregnant women with RA and other rheumatic disease must be discussed and tailored to the individual patient with an emphasis on safety and efficacy. Best pregnancy outcomes will result if the pregnancy is planned so that safer medications can be used during the preconception period, during pregnancy, and while breastfeeding. Poorly controlled rheumatic diseases are associated with poor pregnancy outcomes.

Postpartum, patients with RA self-report disability in physical domains of parenting such as carrying, hygiene, feeding, getting up and down, and household shopping. Difficulty with ADLs in the first trimester and presence of erosive disease increase the risk of high parenting disability postpartum.

Systemic Lupus Erythematosus

Systemic lupus erythematosus (SLE [lupus]) is a pleomorphic autoimmune disease that primarily affects young women of reproductive age. In the United States, an estimated 200,000 persons are affected, with a female:male ratio of at least 9:1, and it is more common in persons of African origin than Northern European origin. In addition to the classic malar rash, patients may experience constitutional, hematologic, mucocutaneous, musculoskeletal, renal, and central nervous system manifestations. Classification criteria require a positive antinuclear antibody (ANA) of at least 1:80, in addition to clinical and immunologic criteria.

Jaccoud’s arthropathy is a nonerosive, fully reducible arthropathy that mimics RA in appearance (MCP dorsal subluxation and ulnar deviation, swan neck and boutonniere deformities, and thumb interphalangeal joint hyperextension), but does not cause joint destruction as it involves ligamentous and capsular laxity with periarticular inflammation. Persons with Jaccoud’s changes do not typically experience limitations in ADLs or ROM ( Fig. 7 ). Nonsteroidal anti-inflammatory drugs, low-dose corticosteroids, conventional DMARDs such as methotrexate, or biologic medications such as belimumab may be needed for joint pain or stiffness that does not respond to hydroxychloroquine alone. Work disability and unemployment are unfortunately common, though influenced by both physical and neuropsychiatric symptoms.