Guidelines for Rheumatology Referral

Revised September 25, 1996
© 1996 American College of Rheumatology

Robin K. Dore, MD, Phillip J. Clements, MD, Robert I. Fox, MD, Daniel E. Furst, MD, Herbert Kaplan, MD, Rodanthi C. Kitridou, MD

Printing of these guidelines supported by a grant from Wyeth-Ayerst.

Introduction

1. Patients with an Uncertain Diagnosis
2. Patients with a Presumed or Confirmed Diagnosis

A. Inflammatory Arthritis

714.0 Rheumatoid arthritis
714.3 Juvenile rheumatoid arthritis (adults with hx of)
99.3 Reactive arthritis (including Reiter's syndrome)
696.0 Psoriatic arthritis
718. Arthritis assoc with inflammatory bowel disease
720.0 Ankylosing spondylitis
88.81 Lyme disease
711. Infectious arthritis

B. Connective Tissue Disease

710.0 Lupus
710.1 Scleroderma
710.2 Sjögren's syndrome
710.3 Dermatomyositis
710.4 Polymyositis
710.8 Mixed connective tissue disease (MCTD)
710.9 Unspecified connective tissue disease (UCTD)
725. Polymyalgia rheumatica
279.8 Antiphospholipid antibody syndrome

C. Systemic Vasculitis

287.0 Henoch Schonlein Purpura
446.0 Polyarteritis nodosa (PAN)
446.4 Wegener's granulomatosis
446.5 Giant cell arteritis
446.7 Takayasu arteritis
287.0 Churg-Strauss syndrome
273.2 Cryoglobulinemia
711.2 Behcet's syndrome
446.20 Hypersensitivity vasculitis

D. Uncommon Rheumatic Diseases

277.3 Amyloidosis
275.0 Hemochromatosis
733.99 Relapsing polychondritis
713.7 Sarcoidosis

E. Osteoarthritis

F. Regional Musculoskeletal Disorders

722.xx Degenerative disk disease
Radiculopathy
Spinal stenosis
726.xx Bursitis/tendinitis
727.xx Tenosynovitis
728.5 Hypermobility syndrome
Regional pain syndromes
Repetitive use syndromes

G. Fibromyalgia/Myofascial Pain

H. Metabolic Bone Disease

733.xx Osteoporosis
731.0 Paget's disease
713.0 Endocrine arthropathy
733.7 Reflex sympathetic dystrophy
588.0 Renal osteodystrophy
268.2 Osteomalacia

I. Crystal Induced Arthropathy

274.9 Gout
712.2 CPPD disease
712.8 Hydroxyapatite deposition disease
271.8 Calcium oxalate deposition disease

J. Children with Rheumatic Diseases

K. Pregnant Women with Rheumatic Diseases

Appendix 1: Rheumatologist's Role in Patient Care

Introduction

There are over one hundred different rheumatic diseases.
While the diagnosis and management of some rheumatic diseases does not always require specialized care, there are numerous rheumatic diseases which are systemic, complex and life-threatening. There are many others which involve the risk of deformity or disability and require immediate and aggressive therapy, often with agents which may have significant toxicity.
Even where there is an established diagnosis, management of patients with rheumatic diseases is often difficult.
The referral of a patient to a rheumatologist for a consultation is often prompted by one or more of the following factors:

- Diagnostic uncertainty
- Uncontrolled symptoms
- Increasing disability or deformity
- Disease complications
- Management uncertainty
- Consideration of immunosuppressive therapy
- Proposed surgical intervention
- Medication complications
- Patient request for specialist opinion

The objective of a rheumatology consultation is to make or confirm a diagnosis and to provide a prognosis and treatment recommendations.
Since findings on physical examination may take precedence over laboratory findings in the establishment of a diagnosis and a treatment plan, the importance of a skilled examination cannot be overemphasized.
Establishing a prognosis and recommending treatment require experience in assessing the severity, activity and progression of disease. This process may involve one or more visits following the consultation.
Long term management of the rheumatic patient not only involves monitoring and treating disease and medication complications but also counseling skills and the ability to work with, and often coordinate, a team of health care providers, including physical therapists, occupational therapists, nurses, mental health professionals and other physicians.
The goal of the Guidelines for Rheumatology Referral is to help physicians provide quality medical services. The Guidelines are intended to provide a general understanding of the reasons for involving a rheumatologist in patient care and to identify some circumstances when a referral to a rheumatologist is appropriate.
The Guidelines were developed by the authors with input from primary care providers, internists, orthopedists and patient groups. Multiple preliminary drafts and the final product were reviewed by primary care physicians, patient groups and rheumatologists from across the country. There is no evidence-based data available regarding appropriate rheumatology referral, and the Guidelines were based upon consensus of the above physicians and patient groups. The physicians involved in the writing and editing of the Guidelines represent academic, private practice, research and managed care settings.
The Guidelines are directed to two sets of users. The first set of users is of course those physicians who desire to refer a patient to a rheumatologist. The Guidelines will hopefully identify when it is appropriate to refer a patient to a rheumatologist and simplify and expedite the referral process.
The second set of users includes those who, prospectively or retrospectively, review physician decisions to refer care to rheumatologists. The Guidelines should help these users evaluate the medical necessity of specialist care and help them promptly elicit any additional information which may be required to conclude a review.
The patients who are referred to rheumatologists fall into two broad categories - those whose diagnosis is uncertain but a rheumatic disease is a possible cause - and those who carry a diagnosis or presumed diagnosis.
For patients with an uncertain diagnosis, the Guidelines offer a brief list of signs or symptoms which suggest the need for a rheumatology referral.
For patients with a diagnosis or presumed diagnosis, the reasons for a rheumatology referral have been consolidated into five categories:

- Diagnosis
- Uncontrolled Disease
- Disease Complications
- Medication Complications
- Management

Some reasons for referral falling within the category are listed below the category. The Guidelines are not intended to be exclusive. All diseases and all appropriate reasons for a referral are not listed.
The rheumatologist's role in patient care is not solely consultative. (See Appendix 1, The Rheumatologist's Role in Patient Care). Rheumatologists provide exclusive or concurrent care for many of their patients' rheumatic diseases, including monitoring of disease status, assessing results of treatment and monitoring for potential treatment side effects. The reasons for referral may also justify returning care of a previously stable patient to a rheumatologist for reconsultation and ongoing care; e.g., a patient experiences a disease flare, disease complications or medication complications.
The Guidelines are respectfully submitted to physicians and others in health care with the belief that they promote quality health care for our patients.

Guidelines

1. Patients with Uncertain Diagnosis

- Normal laboratory findings but local or generalized pain and swelling.
- Abnormal laboratory findings but symptoms and/or
examination do not fit criteria for any specific rheumatic disease.
- Patient's complaints are out of proportion to findings on
laboratory or physical examination.
- Unexplained symptoms/physical findings such as rash, fever, arthritis, anemia, weakness, weight loss, fatigue or anorexia.

2. Patients with a Presumed or Confirmed Diagnosis

A. Inflammatory Arthritis

Diagnosis

Establish or confirm diagnosis; e.g., differentiate erosive osteoarthritis and inflammatory arthritis, differentiate crystal induced arthropathy and seronegative spondyloarthropathy.

Uncontrolled Disease

- Disease onset with significant pain, stiffness or swelling
requiring immediate/aggressive therapy.
- Pain, stiffness or swelling which does not respond to NSAID therapy within 3-4 months after disease onset.
- Previously stable disease becomes active.
- Erosions appear or progress on x-ray at any time in the course of the disease.
- Functional deterioration affecting quality of life.
- Rapid disease progression (physical signs of) e.g., nodules, new onset of deformities, subluxation or loss of motion in one or more joints.

Disease Complications

- Cardiac involvement causing chest pain and/or pericardial
effusions.
- Eye involvement presenting as dry, red and/or painful eyes, especially if unilateral.
- Renal disease presenting as pedal edema, proteinuria.
- Pulmonary involvement causing SOB, cough, nodules on chest x-ray or pleural effusions/infiltrates.
- Vasculitis, cutaneous or systemic, causing rash, skin ulcer ations, neuropathy.

Medication Complications

Treatment is effective but drug toxicity or intolerance occurs; e.g., steroid myopathy, osteoporosis, multiple recurrent infections, pneumonitis, bone marrow suppression.

Management

- Corticosteroid therapy (chronic) is required to control
disease.
- Immunosuppressive drug therapy is considered to control
disease or to taper corticosteroids.
- Joint injections, particularly when prior joint injections have not provided sufficient relief or an injection of a small or
difficult to access joint is involved, e.g., finger, wrist, elbow, ankle.
- Long-term treatment plan/goals for patients with chronic, long-standing disease must be established.
- Physical/occupational therapy recommendations, including appropriate use and duration of PT/OT.
- Pre/Post surgery medication modifications/coordination.
- Surgical opinion - opinion/second opinion regarding the
timing of and need for surgical intervention.

B. Connective Tissue Disease

Diagnosis

- Establish or confirm diagnosis.
- Differentiate sicca syndrome and Sjögren's syndrome.
- Evaluate recurrent fetal losses or unexplained thromboses.
- Interpret serologic laboratory tests.

Uncontrolled Disease

- Previously stable disease becomes active.
- Elevated CPK, ESR on corticosteroid therapy.
- Normal ESR but symptoms persist.
- Pleurisy or arthritis not controlled by NSAIDs.
- Skin tightening, rash, not controlled by topical therapy.
- Signs/symptoms (other) persist despite therapy, e.g., Raynaud's, digital ulcers, muscle weakness, dry eyes and/or mouth.
Uncontrolled Disease

Persistent rheumatic symptoms, sleep disturbance, fatigue, pain, despite therapy including anti-depressants and exercise.

Medication Complications

Treatment is effective but drug toxicity or intolerance occurs.

Management

- Establish a treatment plan.
- Family/patient counseling, including reassurance that condition is not life-threatening.
- Pain management program recommendations.
- Physical/occupational therapy recommendations, including appropriate use and duration of PT/OT.
Disease Complications

- Ankylosis causing loss of motion.
- Cardiac involvement causing pericarditis/myocarditis,
pericardial effusion.
- Hematologic involvement causing anemia, neutropenia, thrombocytopenia, ITP.
- Eye disease with recurrent corneal ulcerations or change in vision.
- Fetal losses or thromboses occur while on therapy such as ASA or anticoagulation.
- GI involvement causing motility disorders or abdominal pain.
- Lung involvement causing shortness of breath, cough.
- Lymph node involvement causing lymphadenopathy,
lymphoma or pseudolymphoma.
- Malignant hypertension (scleroderma and others).
- Peripheral/central nervous system disease; e.g., confusion, disorientation, neuropathy, paresthesias, seizures, TIAs, CVAs.
- Renal insufficiency/nephrotic syndrome/ glomerulonephritis/renal tubular acidosis causing fluid retention, decreased urine output.
- Vasculitis, cutaneous or systemic.

Medication Complications

Treatment is effective but drug toxicity or intolerance occurs; e.g., steroid myopathy, osteoporosis, multiple recurrent infections.

Management

- Establish a treatment plan.
- Anticoagulation therapy, e.g., ASA, heparin or Coumadin in presence of antiphospholipid antibodies.
- Corticosteroid therapy (chronic) is required to control disease.
- Immunosuppressive drug therapy is considered to control disease or to taper corticosteroids.
- Apheresis, IV immunoglobulin or other non-DMARD therapy is considered.
- Physical/occupational therapy recommendations, including appropriate use and duration of PT/OT.
- Pre/Post surgery medication modifications/coordination.

C. Systemic Vasculitis

287.0 Henoch Schonlein purpura
446.0 Polyarteritis nodosa (PAN)
446.4 Wegener's granulomatosis
446.5 Giant cell arteritis
446.7 Takayasu arteritis
287.0 Churg-Strauss syndrome
273.2 Cryoglobulinemia
711.2 Behcet's syndrome
446.20 Hypersensitivity vasculitis
Diagnosis

Establish or confirm diagnosis; presenting symptoms may include fever, weight loss, malaise, rash, arthritis, renal insufficiency, chronic sinusitis, unilateral headache, cough and/or SOB.

Uncontrolled Disease

Progressive systemic involvement despite therapy.

Disease Complications

- Eye involvement, e.g., iritis, uveitis.
- Lung involvement causing shortness of breath, cough, pulmonary hemorrhage, infiltrates, nodules or cavities.
- Mesenteric infarction/perforation causing abdominal pain, distention.
- Renal insufficiency/glomerulonephritis.
- Peripheral/central nervous system disease; e.g., confusion, disorientation, paresthesias, seizures, TIAs, CVAs.

Medication Complications

Treatment is effective but drug toxicity or intolerance occurs; e.g., bone marrow suppression, opportunistic infections, hemorrhagic cystitis (cyclophosphamide induced), steroid myopathy, osteoporosis.

Management

- Establish a treatment plan.
- Apheresis, IV immunoglobulin or other non-DMARD therapy is considered.
- Corticosteroid therapy (chronic) is required to control disease.
- Immunosuppressive drug therapy is considered to control disease or to taper corticosteroids.
- These diseases should in almost all instances be managed by subspecialists (e.g., rheumatologists, nephrologists, pulmonologists); concurrent care may be provided by other physicians.

D. Uncommon Rheumatic Diseases

277.3 Amyloidosis
275.0 Hemochromatosis
733.99 Relapsing polychondritis
713.7 Sarcoidosis

Diagnosis

- Establish or confirm diagnosis; e.g., differentiate between
infectious arthritis and sarcoidosis.
- Interpret laboratory tests; e.g., angiotensin converting enzyme.
- Determine need for tissue biopsy.
Uncontrolled Disease

- Previously stable disease becomes active.
- Signs/symptoms persist despite therapy; e.g., dry eyes and/or mouth, rash, arthritis.

Disease Complications

- Cardiac involvement causing cardiomyopathy, pericarditis.
- Eye involvement with uveitis.
- Hematologic involvement causing hyperviscosity, bone
marrow suppression.
- Lung involvement causing shortness of breath, cough,
nodules, cavities or infiltrates.
- Lymph node involvement causing lymphadenopathy.
- Peripheral/central nervous system disease; e.g., entrapment neuropathy, paresthesias, vasomotor instability.
- Renal insufficiency/nephrotic syndrome causing peripheral edema, decreased urine output.
- Vasculitis, cutaneous or systemic.

Medication Complications

Treatment is effective but drug toxicity or intolerance occurs; e.g., steroid myopathy, osteoporosis, opportunistic infections.

Management

E. Osteoarthritis

Diagnosis

Establish or confirm diagnosis; e.g., differentiate erosive osteoarthritis and inflammatory arthritis, differentiate osteoarthritis and crystal induced arthropathy.

Uncontrolled Disease

Single or multiple joint involvement which does not respond to NSAID therapy.

Medication Complications

NSAID treatment is effective but drug toxicity or intolerance occurs.
Management

- Joint injections, particularly when prior joint injections have not provided sufficient relief or an injection of a small or difficult to access joint is involved, e.g., finger, wrist, elbow, ankle.
- Physical/occupational therapy recommendations, including appropriate use and duration of PT/OT.
- Pre/Post surgery medication modifications/coordination.
- Surgical opinion - opinion/second opinion regarding the timing of and need for surgical intervention.

F. Regional Musculoskeletal Disorders

722.xx Degenerative disk disease
Radiculopathy
Spinal stenosis
726.xx Bursitis/tendinitis
727.xx Tenosynovitis
728.5 Hypermobility syndrome
Regional pain syndromes
Repetitive use syndromes

Diagnosis

Differentiate between a regional musculoskeletal problem and a generalized systemic disorder.

Uncontrolled Disease

Functionally compromising or unresponsive to primary treatment.

Medication Complications

NSAID treatment is effective but drug toxicity or intolerance occurs.

Management

- Periarticular injections, particularly when prior joint injections have not provided sufficient relief.
- Physical/occupational therapy recommendations, including appropriate use and duration of PT/OT.
- Surgical opinion - opinion/second opinion regarding the timing of and need for surgical intervention.

G. Fibromyalgia/Myofascial Pain

Diagnosis

Differentiate articular disease, soft tissue rheumatism and systemic inflammatory disease (e.g., rule out lupus in patients with positive ANA and lacking other diagnostic criteria).

H. Metabolic Bone Disease

733.xx Osteoporosis
731.0 Paget's disease
713.0 Endocrine arthropathy
733.7 Reflex sympathetic dystrophy (RSDS)
588.0 Renal osteodystrophy
268.2 Osteomalacia

Diagnosis

- Establish or confirm diagnosis.
- Interpret metabolic laboratory tests.
- Evaluate bone density studies and nuclear medicine bone scans.
- Determine need for bone biopsy.

Uncontrolled Disease

- Corticosteroid therapy (chronic) is necessary to control life threatening or disabling primary disease.
- Elevated alkaline phosphatase in Paget's disease despite therapy.
- Functional deterioration affects quality of life.
- Osteopenia despite hormone replacement therapy (HRT) or other therapy.
- Osteopenia when HRT contraindicated or not desired.
- Persistent pain and/or swelling despite therapy in RSDS.

Disease Complications

- Chronic pain.
- Fractures.

Medication Complications

Treatment is effective but drug toxicity or intolerance occurs.

Management

- Establish a treatment plan.
- Evaluate and treat fractures caused by these diseases.

I. Crystal Induced Arthropathy

274.9 Gout
712.2 CPPD disease
712.8 Hydroxyapatite deposition disease
271.8 Calcium oxalate deposition disease

Diagnosis

- Differentiate polyarticular crystal disease and inflammatory arthritis.
- Establish diagnosis by joint aspiration and
crystal identification.
- Interpretation of abnormal laboratory values (calcium, uric acid, phosphorus).

Uncontrolled Disease

- Recurrent episodes despite appropriate therapy.
- Pain, stiffness and swelling which does not respond to NSAID therapy.

Disease Complications

- Polyarticular gout.
- Tophaceous gout.

Medication Complications

Treatment is effective but drug toxicity or intolerance occurs.

Management

Hypouricemic drug therapy is considered.

J. Children With Rheumatic Diseases

Diagnosis, treatment recommendations and concurrent care with pediatrician or family practitioner.

K. Pregnant Women with Rheumatic
Diseases

Diagnosis, treatment recommendations and concurrent care with obstetrician or family practitioner during pregnancy and three months post partum.

Acknowledgments

The authors would like to acknowledge the useful analysis by Drs. Dieppe and Paine based on their experience in the United Kingdom1 and the comments and suggestions of the following individuals:

Teresa J. Brady PhD, Joseph Golbus MD, Robert S. Katz MD, Douglas E. Roberts MD, Arthur L. Weaver MD, Robert F. Willkens MD

on drafts or portions of drafts of the Guidelines.

The Guidelines are not an exclusive list of circumstances in which a referral is appropriate. At the same time, the Guidelines do not require a referral in each of the circumstances indicated. The decision to refer is left to the judgment of the primary care physician or other health care provider.
It is hoped that those who use the Guidelines will offer comments and suggestions. It is also hoped that those organizations which adopt the Guidelines will notify the authors of their adoption and make an effort to evaluate their usefulness.

1Dieppe, P. and Paine, T., Referral Guidelines for General Practitioners - Which Patients with Limb Joint Arthritis Should be Sent to a Rheumatologist?, Reports on Rheumatic Diseases, Jan 1994 No. 1, Arthritis and Rheumatism Council (UK). The reasons for referring patients to a rheumatologist are essentially those identified by Drs. Dieppe and Paine.

Appendix 1:
Rheumatologist's Role in Patient Care

The objective of a rheumatology consultation is to make or confirm a diagnosis and, in those cases as well as where a diagnosis has been previously established, to provide a prognosis and treatment recommendations. This process may involve one or more visits following the consultation. Subsequently, the rheumatologist acting as a specialist with respect to the rheumatic disease might provide ongoing care, concurrent care, periodic re-evaluation, PRN care (such as for flare intervention) or no care at all, as described below. Rheumatologists are almost all trained as internists, though a small number of rheumatologists, who treat exclusively children, are trained as pediatricians. Some rheumatologists and pediatric rheumatologists may offer their patients primary care services in addition to providing necessary rheumatology specialty care.

Diagnosis: Make the diagnosis or confirm the diagnosis of the referring physician.

Prognosis and Treatment: Establish a prognosis, make treatment recommendations, initiate treatment and educate the patient.

Consultation Only: Return care to the referring physician who will continue care and will be responsible for monitoring the effectiveness and toxicity of the treatment.

Ongoing Care: Assume primary care for the treatment of the rheumatic disorder and be responsible for monitoring the effectiveness and toxicity of the treatment.

Concurrent Care: Share care for the rheumatic disease with the referring physician. The rheumatologist and the referring physician see the patient at mutually agreeable intervals. Each will monitor the effectiveness and toxicity of the treatments at their respective visits. They will determine whether laboratory tests arising from monitoring by the referring physician will be provided to the rheumatologist.

PRN Care: See the patient on an "as needed" basis, such as for exacerbation (e.g., a flare) in disease activity.

Follow-up Consultation: See the patient for re-evaluation after a given period of time; the rheumatologist will not monitor the patient during the interim.

Joint Injections/Aspirations: The rheumatologist may be requested to inject/aspirate a joint.

Nonarticular Injections: The rheumatologist may be requested to inject tendons, bursae or trigger points.

Pregnancy: The rheumatologist may be consulted in the case of pregnancy of a patient with a rheumatic disease to coordinate any necessary modification in patient medications.

Surgery: The rheumatologist may be consulted prior to and after any surgery to coordinate any necessary modification in patient medications, e.g., NSAIDs, corticosteroids, immunosuppressive therapy.

Surgical Opinions: The rheumatologist may be consulted prior to any orthopedic/neurosurgical procedure on a patient with rheumatic disease regarding the timing of and necessity for the surgery as well as to coordinate any necessary modification in patient medications.

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