Rheumatic Manifestations of Systemic Diseases: a Practical Approach

! Systemic diseases may present with rheumatological complaints Rheumatic manifestations of systemic diseases: ! Musculoskeletal manifestations and autoimmune phenomena may A practical approach develop during the course of a systemic disease ! Some systemic diseases are associated with rheumatological conditions Faith L Chia and vice versa Consultant, Department of Rheumatology, Allergy and Immunology Tan Tock Seng Hospital Joint pain Vasculitis Proximal weakness Generalised pain Pregnancy

Does this patient have an inflammatory arthritis? Does this patient have an inflammatory arthritis?

A 78 year old lady presents complaining of painful wrists and hands for the A 66 year old lady had paraesthesia in her last 4 months. The pain is persistent and is associated with warmth and hand that progressed to joint discomfort swelling. NSAIDs and tramadol provide only partial relief. She also has LOA and stiffness over the last 3 years. She and has lost 5-6 kg. can’t straighten her fingers fully. There is no improvement with activity, and using her hands makes the symptoms worse.

She also has hypertension, hyperlipidaemia and DM type 2 for 15 years on medical treatment. No family history of note. FBC, CRP and ESR are normal. RF positive.

Hypertrophic Diabetic Pulmonary cheiroarthropathy Osteoarthropathy

Joint pain: Systemic causes Joint pain (or not)

• Be wary of symptoms that are not in keeping with inflammatory joint pain

• Positive RF = Rheumatoid arthritis

• Patients who complain of “joint pain” may actually have periarticular or soft tissue problems

Articular pain Non-articular pain Swelling Erythema Localisation If inflammatory: No erythema or swelling Stiffness >1h Worse with movement Improvement with activity Accompanied by systemic symptoms Rheumatic manifestations of diabetes mellitus Does this patient have a systemic vasculitis?

A 42 year old man was admitted with abrupt onset of fever, chills and rigors associated with myalgia for 6 days. Treated as for a CAP but with no improvement. He then developed this rash and joint pain over his hands and feet.

He was toxic with T40oC and had synovitis of the PIPJs. Palpable purpura and splinter haemorrhages seen.TW 32K CRP >200. UFEME microscopic haematuria and protein 1+ Infective endocarditis

Does this patient have a systemic vasculitis? Vasculitis!

A 78 year old lady was admitted with digital ischaemia over the last 2 weeks. • Systemic diseases can mimic vasculitis or cause a true secondary vasculitis She was a chronic smoker but was unaware of any other medical problems. • High swinging fevers should alert to other causes

• Many infectious agents and drugs can cause positive ANCA testing (usually p-ANCA but negative anti-MPO or anti-PR3)

Cryoglobulinaemia secondary to HBV infection

Vasculitis and mimics: systemic causes Does this patient have an inflammatory myopathy?

A 42 year old housewife is referred for muscle pain and weakness for 6 Infective causes Drugs Malignancy Others months associated with early morning stiffness lasting 10 minutes. CK done at her GP was 380. The symptoms get worse after the end of the day. She also has significant fatigue but has put on weight and not lost weight ‣ Infective ‣ Allergic reactions ‣ Intravascular ‣ DIVC recently. endocarditis ‣ Vasospasm lymphoma/NHL ‣ TTP ‣ Syphilis ‣ Ergot ‣ Leukaemias ‣ Cholesteral Examination reveals very mild ‣ Tuberculous ‣ Cocaine (mimics ‣ Paraneoplastic emboli proximal weakness. She has ‣ Mycobacteria WG) vasculitis ‣ Cardiac myxoma multiple trigger fingers but no ‣ Leprosy ‣ Congenital synovitis. She had asteatotic (Lucio’s causes eczema over her legs. When phenomenon) ‣ fibromuscular taking her blood pressure, it ‣ Viral infections dysplasia is noted she’s bradycardic ‣ Hep B/C, HIV ‣ Marfan’s ‣ Mycotic syndrome aneurysms ‣ Scurvy Hypothyroidism Inflammatory myopathies Proximal weakness: Systemic causes

Cushing’s syndrome • Determining the distribution of weakness is key to generating appropriate Hyperthyroidism differentials Hypothyroidism Hyperparathyroidism • There are many causes for raised CK

Hypokalaemia Steroids • High spiking fevers or asymmetrical myositis should alert you to the Hyperkalaemia Statins possibility of infective causes Alcohol ART

Becker’s muscular dystrophy Limb-girdle syndrome Myotonic dystrophies type 1 Glycogen storage diseases Lipid storage disorders Mitochondrial disorders

Generalised pain Rheumatic manifestations of Pregnancy

• Infections: Acute (viruses) and Chronic (Lyme, Hep C) • Musculoskeletal: ligamentous laxity, oedema, back pain, tenosynovitis

• Endocrinopathies (Hypothyroidism, Addison’s • Cutaneous: hyperpigmentation, alopecia (TE), pruritus, pruritic urticarial disease, hyperparathyroidism) papules and plaques of pregnancy, EN

• Myopathies • Haematologic: anaemia, thrombocytopaenia, increased risk of DVT • Vitamin D deficiency • Renal: microscopic haematuria, proteinuria • Metabolic bone diseases (osteomalacia), renal osteodystrophy • Neurologic: nerve entrapment, chorea gravidarum • Malignancy Fibromyalgia is not just

• Sleep disorders/Depression “rheumatism”

Approach to a patient Summary

• A thorough and meticulous history and examination is essential • Many systemic diseases have rheumatic manifestations

• Baseline labs: FBC, U/E/Cr, LFT, CRP or ESR • Autoimmune diseases are actually quite rare

• Differentiation is essential as treatment can be diametrically opposite

Joint pain Vasculitis Muscle weakness Generalised pain • Keep an open mind during evaluation Aspirate joint Cultures Muscle enzymes If tendinitis; consider Urinalysis Thyroid function Calcium/albumin doing glucose, Hep B/C serologies EMG Vitamin D thyroid function 2DE Biopsy Thyroid function

• Fishing expeditions with serologies are rarely helpful References

• Markenson JA. Rheumatic manifestations of endocrine diseases. Current Opinion in Rheumatol 2010; 22: 64

• Molloy E, Langford CA. Vasculitis Mimics. Current Opinion in Rheumatol 2008; 20:29

• Ravindran V, Anoop P. Rheumatologic manifestations of benign and malignant haematological disorders. Clin Rheumatol 2011; 30:1143

• Cacoub P, Terrier B. Hepatitis B-related autoimmune manifestations. Rheum Dis Clin N Am 2009; 35: 125

• Sammaritano LR. Rheumatologic manifestations of pregnancy. Rheum Dis Clin N Am 2010; 36: 729