Discussion of Feb 2014 case
The elderly, 78 years male presented with a 7-year history of polyarthritis and experienced worsening symptoms for 3 months. The disease, which affected both small and large joints, caused limitation in movement of shoulders, knees, and hands. Although the patient was diabetic and hypertensive, he could achieve adequate control through medications. The patient had normal ESR and CRP when evaluated a month before, A gradual increase in the levels of ESR and CRP (114mm per hour and 77.9 mg/L respectively), when compared to the previous month was noticed during the current consultation. Work-up for RF, ANA, Anti-CCP, and viral markers (hepatitis B, C, and HIV) was negative. The neurological evaluation indicated bilateral lower limb peripheral neuropathy and minimal pericardial effusion.
Based on the aforementioned clinical findings, the probable diagnoses are:
Autoimmune and musculoskeletal disease
- RA with exacerbation of the disease
- OA with development of PMR or RA
- Vasculitis
- Reactive arthritis
Malignancy
- Paraneoplastic manifestation of a tumor
- Multiple myeloma
Infective joint disease
Rare probability of chikungunya or post-viral arthritis
Other causes
Renal/ metabolic
The current case fulfills the ACR/EULAR 2010 criteria for diagnosing RA1, with joint involvement scoring 5, elevated ESR and CRP scoring 1, and more than 6 weeks scoring 7. However, since both the antibody profiles are negative and the patient is aged, it is significant to consider the possibility of other diseases. The patient had the severe proximal joint disease and ESR was more than 100. When the 2012 revised EULAR/ACR criteria for polymyalgia rheumatic (PMR) are considered, the patient fulfilled all the essential criteria like age above 50 yrs, recent onset bilateral shoulder pain, and elevated ESR and CRP.2 He gets a score of 4 in non-ultrasound criteria, based on the presence of stiffness throughout the day and absence of anti-CCP antibodies (ACCPA) and RF. The unusual symptom noticed is the occurrence of peripheral arthritis in small joints of hands and feet. As per the patient history, the disease of the hand is more than 7 years old, and the X-ray demonstrated more osteoarthritis changes than RA. These findings suggest the possibility of having PMR than RA.
Table 1: The 2012 provisional classification criteria for polymyalgia rheumatic PMR classification criteria scoring algorithm—required criteria: age 50 years or older, bilateral shoulder aching and abnormal CRP and/or ESR* |
|
Points without the US (0–6) |
Points with US†(0–8) |
Morning stiffness duration >45 min |
2 |
2 |
Hip pain or limited range of motion |
1 |
1 |
Absence of RF or ACPA |
2 |
2 |
Absence of other joint involvement |
1 |
1 |
At least one shoulder with subdeltoid bursitis and/or biceps tenosynovitis and/or glenohumeral synovitis (either posterior or axillary) and at least one hip with synovitis and/or trochanteric bursitis |
Not applicable |
1 |
Both shoulders with subdeltoid bursitis, biceps tenosynovitis, or glenohumeral synovitis |
Not applicable |
1 |
*A score of 4 or more is categorized as PMR in the algorithm without the US and a score of 5 or more is categorized as PMR in the algorithm with the US. |
†Optional ultrasound criteria. |
ACPA: anticitrullinated protein antibody; CRP: C-reactive protein; ESR: erythrocyte sedimentation rate; PMR: polymyalgia rheumatic; RF: rheumatoid factor; US: ultrasound. |
Although the patient did not have any symptoms of neuropathy, the electro neuro myogram (ENMG) conducted before the current consultation raised the suspicion of benign peripheral polyneuropathy. The findings could be explained by the evidence suggesting the increased incidence of marginal axonal sensory neuropathy in elderly diabetic patients.3,4,5 or a part of his diabetes mellitus. However, one must consider the possibility and a workup for vasculitis can be considered for exclusion. However, the gradual clinical presentation precludes such a possibility.
Paraneoplastic syndrome with rheumatological manifestation should be considered in the present case and a basal screening for the same should be performed. The common presentations of the syndrome are explained by Racanelli et al.6 He fulfilled the criteria for diagnosing PMR and RA. The patient also demonstrated some of the unexplainable features of each disease. Hence a careful exclusion of malignancy and multiple myeloma is essential.
Elevation of ESR and CRP, and involvement of small and large joints is rather unusual in infective and post-infective arthritis. Additionally, the absence of metabolic features and the normal levels of creatinine and alkaline phosphatase suggested the reduced possibility of having metabolic rheumatological diseases.
Follow-up and diagnosis
The patient was initially managed with a small dose of steroid 0.5 mg / Kg body weight of steroid along with Naprosyn sodium. The response was dramatic, and the pain was reduced by more than 70%, thereby suggesting the possibility of having PMR. Work-up for malignancy was negative. The patient has further managed with weekly methotrexate therapy as a steroid-sparing drug since the patient is suffering from Diabetes.
Final Diagnosis
Polymyalgia rheumatica
Learning points
- PMR is a possible diagnosis in elderly patients
- A possibility of malignancy should be carefully excluded in such patients
- Background symptoms or age-related problems like osteoarthritis and polyneuropathy should be carefully monitored in such patients.
- Though the condition may fulfill two or three criteria, other clinical features should be considered for confirming the diagnosis.
References
- Kay J, Upchurch KS. ACR/EULAR 2010 rheumatoid arthritis classification criteria. Rheumatology. 2012;51(suppl_6):vi5-vi9.
- Dasgupta B, Cimmino MA, Maradit-Kremers H, Schmidt WA, Schirmer M, Salvarani C, et al. 2012 provisional classification criteria for polymyalgia rheumatica: a European League Against Rheumatism/American College of Rheumatology collaborative initiative. Ann Rheum Dis. 2012 Apr 1;71(4):484–92.
- Shaumberg HH, Spencer PS, Ochoa J. The aging human peripheral nervous system. In: Katzman R, Terry RD, editors. The neurology of aging. Philadelphia: Davis; 1983.
- Mold JW, Vesely SK, Keyl BA, Schenk JB, Roberts M. The prevalence, predictors, and consequences of peripheral sensory neuropathy in older patients. J Am Board Fam Pract. 2004 Oct;17(5):309–18.
- Jordan B, Cummings JL. Mental status, and neurologic examination in the elderly. In: Hazzard WR, Blass JP, Ettinger WH, Jr., et al, editors. Principles of geriatric medicine and gerontology. New York: McGraw-Hill; 1999.
- Racanelli V, Prete M, Minoia C, Favoino E, Perosa F. Rheumatic disorders as paraneoplastic syndromes. Autoimmun Rev. 2008 May;7(5):352–8.