Differentiating Polymyalgia rheumatica from other diseases

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ahmed Elsaiey, MBBCH [2]

Overview

Polymyalgia rheumatica (PMR) must be differentiated from other conditions such as late onset rheumatoid arthritis, polymyositis, dermatomyositis, fibromyalgia, and remitting seronegative symmetrical synovitis with pitting edema.

Differentiating Polymyalgia Rheumatica from other Diseases

PMR must be differentiated from the following conditions:

• Rheumatoid arthritis: PMR and late onset rheumatoid arthritis (RA) can initially present with similar clinical features like synovitis. These patients are treated initially as PMR with glucocorticoids. RA treatment is started when there is no improvement or when it evolves into characteristic RA or when there is a persistently raised plasma viscosity.

• Polymyositis and dermatomyositis: Patients with dermatomyositis or polymyositis present with tenderness and weakness of proximal muscles, while PMR patients present with pain and stiffness. This differentiation between these entities may be difficult in elderly patients. Proper history, complete physical examination, ESR, creatine kinase levels and muscle biopsy help in establishing the proper diagnosis.

• Malignancy (such as myeloma): Patients with malignancy sometimes present with PMR like symptoms and have poor response to steroid therapy. This is in fact paraneoplastic syndrome presenting as PMR.

• Fibromyalgia: Fibromyalgia occurs in age groups 20-50 years. Patients have characteristic tender points. Unlike PMR, the active phase proteins and ESR are normal.

• Hyperparathyroidism: Hyperparathyroidism presents with proximal stiffness and bone pain with elevated parathyroid hormone levels and often raised calcium levels without elevation of ESR levels.

• Chronic infection (subacute bacterial endocarditis (SBE)): Rheumatologic symptoms seen in infective endocarditis can present as a clinical picture suggestive of polymyalgia rheumatica hindering the correct diagnosis.

• Hypothyroidism: Patients with hypothyroidism muscle and joint pain and weakness similar to PMR. Delayed relaxation of deep tendon reflexes is seen in hypothyroidism with elevated TSH levels and low T4 levels.

• Remitting seronegative symmetrical synovitis with pitting edema (RS3PE): RS3PE presents with symmetrical synovitis and pitting edema, usually in patients over 50 years of age and lack rheumatoid factor. Unlike PMR, the symptoms commonly manifest distally.

Differentiating polymyalgia rheumatica on the basis of fatigue and chronic pain

Disease	Differentiating signs and symptoms	Diagnostic findings
Polymyalgia rheumatica	Older at onset Generalized stiffness	Elevated ESR or CRP Response to corticosteroids
Fibromyalgia	Symptoms have been present at a similar level for at least 3 months. Chronic musculoskeletal pain with multiple tender points Stiffness, numbness, and fatigue Headaches Sleep disorder	All lab tests are normal
Rheumatoid arthritis	Multiple joint swelling Morning stiffness Rheumatoid nodules	Positive RF or anti-cyclic citrullinated protein (CCP) antibody Elevated ESR or CRP
SLE	Maculopapular rash Multi-system involvement	Positive anti-Smith antibodies
Chronic fatigue syndrome	Fatigue plus 4 of the following symptoms: Short-term memory loss Sore throat Tender lymph nodes in the neck or armpit Muscle pain Joint pain without swelling or redness Headaches Insomnia Malaise	Diagnosis of exclusions Symptoms must present for more than 6 months
Spondyloarthritis	Axial skeletal pain and stiffness Restricted spinal motion	Elevated ESR or CRP Negative RF Bamboo spine on x-ray
Osteoarthritis	Localized joint pain Restricted to affect joints Older at onset	Degenerative joint changes on x-ray
Hypothyroidism	Systemic symptoms such as weight gain, constipation, dry skin Muscular aching Prominent fatigue	Elevated TSH Low free T4
Polymyositis and dermatomyositis	Pelvic and shoulder girdle muscle weakness Rash	Muscle biopsy confirms the diagnosis Elevated CPK enzyme
Neuropathy	Numbness and tingling Paresthesia	Abnormal EMG

Differentiating polymyalgia rheumatica from other causes of inflammatory myopathy

Organ system	Disease	Symptoms												History	Physical Examination	Diagnosis
		Age of onset	Muscle weakness	Fever	Myalgia	Contractures	Gait abnormality	Neuropathy	Atrophy	Stiffness	Myoglobinuria	Other features	Laboratory Findings			Creatine Kinase	Muscle Biopsy	Electromyogram
Inflammatory/ Rheumatologic	Polymyalgia Rheumatica	50s	Diffuse	+	+	−	−	−	−	+	−	Weight loss	History of joints stiffness, worse in the morning	Restricted shoulder motion	↑↑ ESR ↑↑ CRP	Normal	Normal	Normal
	Dermatomyositis	40s−50s Can affect children	Proximal	+	+	−	−	−	−	+	−	Rash Dyspnea Weight loss Cough	Viral infections Cancer	Heliotrope rash on face and hands Telangiectasia Erythema Mechanic's hands Gottron's sign ( violaceous scaly eruption )	↑↑ ESR ↑↑ CRP	↑↑	Perimysial mononuclear infiltrate	Myopathic
	Polymyositis	> 18 years	Proximal	+	+	−	−	−	−	+	−	Similar to dermatomyositis without mucous and skin involvement	N/A	N/A	↑↑ ESR ↑↑ CRP	↑↑	Endomysial mononuclear infiltrate Patchy necrosis
	Inclusion body myositis	50s	Proximal & distal	−	−	−	−	−	−	−	−	Dysphagia Asymmetric weakness	Retrovirus (most common)	N/A	Antibodies to cytoplasmic 5'−nucleotidase	↑↑	Inflammatory cells Invading muscle cells Vacuolar degeneration Inclusions or plaques	Neurogenic
	Fibromyalgia	40−50s	Generalized	−	−	−	−	+	−	−	−	Anxiety or depression features Fatigue Sleep disturbance Numbness Muscle spasms	History of depression	Tenderness in the soft tissue anatomical location	Normal	Normal	Normal	Normal
Organ system	Disease	Age of onset	Muscle weakness	Fever	Myalgia	Contractures	Gait abnormality	Neuropathy	Atrophy	Stiffness	Myoglobinuria	Other features	History	Physical Examination	Laboratory Findings	Creatine Kinase	Muscle Biopsy	Electromyogram