|Year : 2015 | Volume
| Issue : 3 | Page : 195-199
Pandigital and subcutaneous chronic tophaceous gout with acute renal failure
J Shashibhushan, K Venugopal, Mudegoudara Lingaraja, Vishwanath Huggi, CP Patanjali
Department of General Medicine, Vijayanagara Institute of Medical Sciences, Bellary, Karnataka, India
|Date of Web Publication||16-Jul-2015|
Department of General Medicine, Vijayanagara Institute of Medical Sciences, Bellary - 583 104, Karnataka
Source of Support: None, Conflict of Interest: None
Gout (Podagra) is a disorder of purine metabolism characterized by the deposition of monosodium urate crystals in joints and connective tissue and risk of deposition in kidney interstitium. Although acute gouty arthritis is familiar for most physicians, chronic gouty arthritis, which affects small joints of the hands can be difficult to distinguish from other common interphalangeal arthropathies such as rheumatoid arthritis (RA), psoriatic arthritis, and erosive osteoarthritis because of very similar presentations. Here we describe a 60-year-old male diabetic patient with pandigital, extensive subcutaneous tophaceous gout presented with uremic encephalopathy and joint deformities. He had been treated mistakenly as RA for 10 years.
Keywords: Acute renal failure, monosodium urate crystals, rheumatoid arthritis, tophi
|How to cite this article:|
Shashibhushan J, Venugopal K, Lingaraja M, Huggi V, Patanjali C P. Pandigital and subcutaneous chronic tophaceous gout with acute renal failure. Int J Health Allied Sci 2015;4:195-9
|How to cite this URL:|
Shashibhushan J, Venugopal K, Lingaraja M, Huggi V, Patanjali C P. Pandigital and subcutaneous chronic tophaceous gout with acute renal failure. Int J Health Allied Sci [serial online] 2015 [cited 2020 Jul 7];4:195-9. Available from: http://www.ijhas.in/text.asp?2015/4/3/195/160900
| Introduction|| |
Gout with an incidence of 1-2% in developing countries, characterised by recurrent attacks of acute inflammatory arthritis and deposition of monosodium urate crystals (MSU) crystals in joints and subcutaneous tissues. The crystallization of uric acid, often related to relatively high levels in the blood. Under excretion of uric acid by the kidney is the primary cause of hyperuricemia in about 90% of patients, while the overproduction accounts to <10%.  The metatarsal-phalangeal joint at the base of the big toe is the most commonly affected. The deposition of MSU crystals produce masses commonly referred as tophi and usually thought to be a late manifestation of gout. Other than classical monoarticular arthritis, Gouty tophi may be found in the synovial membrane, periarticular ligaments, tendons, soft tissues, subcutaneous tissue, Achilles tendon and helix of the ear.  Few cases have been reported regarding the deposition of tophi in the axial skeleton, mitral valve and kidneys.  However, some patients develop chronic polyarthritis mimicking rheumatoid arthritis (RA).  Both disease entities have been reported to occur in the adult population to the extent of approximately 1%  with symmetric polyarthritis. The symmetrical presentation or positive rheumatoid factor (RF) can be seen in both the diseases. It may be associated with obesity, chronic kidney disease (CKD), hypertension, and diabetes.
Here, we report a case of chronic severe gout associated with diabetes and acute renal failure (ARF) with emphasis on clinical features and complications.
| Case report|| |
A 60-year-old male presented to emergency department with altered sensorium of 1-day duration. He had a past history of diabetes for 5 years and was on oral hypoglycemic drugs. On examination, his pulse rate was 88/min, blood pressure of 150/80 mmHg, SpO 2 was 93% at room air. Cardiovascular system examination was normal. Respiratory system examination revealed bilateral basal fine rales. Central nervous system examination revealed no focal neurological deficit but the patient was irritable. Physical examination revealed soft tissue swelling overall digits of hands, wrist [Figure 1], forearm, all digits of the foot, ankle and around knee joint [Figure 2]. These nodules did not show any signs of inflammation. There was an ulcer noted on the dorsum of the right foot with discoloration of the skin. He had flexion contractures of knee joint, hip joint, and fingers.
|Figure 1: Gouty tophi were noted over proximal interphalyngeal joint of all fingers, metacarpophalangeal joint of thumb and index finger|
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|Figure 2: (a) Ulcer and discoloration over dorsum of right foot with tophi over all toes and dorsum. (b) Huge soft tissue swelling was noted around knee joint with flexion deformity, and wasting of leg muscles|
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Laboratory workup revealed hemoglobin 11 g/dL, Leukocyte counts of 9,000/μL, platelet counts of 240 × 103/μL, blood urea of 167 mg/dL, creatinine of 6.9 mg/dL, random blood sugar was 140 mg%, and sodium, potassium, and chloride were 138, 6.2 and 108 mmol/L, respectively. Uric acid levels in blood and urine was normal. Patient was taken for hemodialysis and dialyzed for 4 h. Patient's consciousness improved gradually, and he was dialyzed continuously for 3 days thereafter every 3 rd day for 3 times. His renal parameters reverted to normal; patient was fully conscious and oriented on subsequent days. RF was negative and anti-cyclic citrullinated peptide (CCP) was 1.1 U/ml, and other laboratory investigations are given in [Table 1].
X-ray of both hands showed sub articular erosions with asymmetric soft tissue swelling and X-ray of feet showed the evidence tophi and joint destruction [Figure 3]. X-ray knee showed soft tissue swelling [Figure 4]. Fine needle aspiration cytology (FNAC) from soft tissue swelling of fingers demonstrated needle-shaped crystals [Figure 5]; histopathological examination of nodule removed from right forearm showed features of gout revealed presence of tophus [Figure 6].
|Figure 3: (a) X-ray anteroposterior (AP) view of foot: Black arrow showing destruction of 2nd metatarsal-phalangeal joint, red arrow showing tophi. (b) X-ray AP view of left hand: Black arrow showing tophi. Red arrow showing reduced joint space, sub periosteal erosion and punched-out lesion. (c) X-ray AP view of right hand showing tophi|
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|Figure 4: X-ray anteroposterior and lateral view of knee joint: Soft tissue swelling noted above patella and popliteal fossa|
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|Figure 5: Microphotography showing urate crystals in back ground of acute inflammatory cells (×10). Inside picture focused on urate crystals|
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|Figure 6: Microphotography showing gouty tophi surrounded by fibrocartilagenous tissue admixed with inflammatory cells comprised mainly of neutrophils (×10)|
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A diagnosis of pandigital chronic tophaceous gout was made, and the patient was treated with febuxostat 40 mg/day, which was increased to 80 mg/day. On follow-up after 15 days patient's renal parameters were normal with good control of glucose.
| Discussion|| |
Gout is a metabolic disorder characterized by hyperuricemia and deposition of MSU crystals within the periarticular tissues, resulting in recurrent painful arthritis. Males dominate the disease population, with only a 5% female prevalence. Secondary gout occurs in 10% of all cases and is the result of increased turnover of nucleic acid, drugs, or acquired defective renal excretion. Tophi appear as firm, pink nodules or fusiform swellings. The overlying skin may be yellow, erythematous or ulcerated. The lesion may drain clear fluid with flakes of urate or thick chalky material.
Complications of tophi include pain, soft tissue damage, ulceration, deformity, joint destruction, nerve compression syndromes such as carpal tunnel syndrome, and renal failure. Deposition of tophi in synovial or extra synovial site around the major joints leads to joint contractures is predominating in pseudogout than gout.  The renal involvement in gout is due to uric acid nephropathy (precipitation of uric acid in the renal tubules or collecting ducts), urate nephropathy (deposition of urate crystals in the renal interstitium and pyramids) and uric acid stones. Urate nephropathy may lead to CKD and end-stage renal disease. Crystal deposition causes increased tubular pressure, increased intra-renal pressure, and extrinsic compression of the small-diameter renal venous network. This causes an increase in renal vascular resistance and a fall in renal blood flow. The elevated tubular pressure and decreased renal blood flow cause a decline in glomerular filtration and can result in ARF.  Individuals who form uric acid stones tend to excrete less ammonium, which contributes directly to low urinary pH. In addition, persons with gout and those who form stones, in particular, have a reduced postprandial alkaline tide (alkaline urinary pH). , The current opinion is that gout per se does not result in overt renal failure, but it may occur with other associated comorbidities like hypertension, diabetes mellitus, and nephrolithiasis.  In our case, tophi were painless, multiple and pandigital. Ulceration noticed over dorsum of right foot, flexion contractures of fingers, huge swelling over knee joints with flexion contractures of knee and wasting of leg muscles.
Annemans et al.  identified a high incidence of comorbidities, such as obesity, diabetes and hypertension in gout. In some cases (CKD) it is clear that presence of comorbidities contributes to the progression of gout. Conversely, whether gout/hyperuricemia contributes to these comorbidities is not clear. In vitro and animal models, epidemiological studies suggest that gout may contribute to CKD, hypertension and cardiovascular disease. However, relation between gout and diabetes is not clear.
Gout is diagnosed either by clinical examination, elevated serum uric acid level or cytological examination of an effusion. Measurement of serum uric acid is of limited help in the diagnosis because, serum uric acid levels can be normal or low at the time of an acute attack, as inflammatory cytokines can be uricosuric and the uric acid level may be low or normal due to the uricosuric action of increased blood glucose levels in chronic gout.  However, Excretion of >800 mg of uric acid per 24 h on a regular diet suggests that causes of overproduction of purine. In our case, multiple samples of serum uric acid level and 24 h uric acid excretion were normal. Rao et al.  reported a case of gouty tophus with normal serum uric acid level.
Cytological diagnosis of gout is contingent on the identification of the characteristic crystals in joint fluid. With either untreated hyperuricemia or long standing gout characterized by multiple episodic bouts of arthritis, gouty tophi will form in the soft tissues. FNAC of gouty tophi is an easy alternative to synovial biopsy and joint fluid analysis. It is simpler, easier and less painful. As crystals are preserved in stained smears, they can be employed for polarization and confirmation of gout.  It shows typical needle-shaped crystal in light microscopy and negative birifringent when viewed under a polarized microscope.  Biopsy of the soft tissue swelling shows characteristic tophi in the background of inflammatory cells.  It should be differentiated from other crystalloid arthropathies like pseudogout and calcium apatite arthropathy, which shows rhomboid crystals and bipyramid crystals respectively. 
The early radiographic signs of gout are joint effusion and periarticular edema, caused by deposition of the nonopaque crystals within the synovial and cartilaginous tissues. Eventually, there is osseous erosion, manifested as "punched-out" lesions at the margins of the articular surfaces of the hands and feet. Although various clinical and radiological clues exist, gouty arthritis is especially confused with RA. Important radiographic features distinguishing gouty arthritis from RA are the presence of marginal erosion, joint space narrowing and marked periarticular osteopenia in the latter. In addition to the radiographic findings, the conjunction of both a negative RF and anti-CCP test made the diagnosis of RA unlikely in this patient.
Our patient had multiple, pandigital tophi which were painless, with complications like ulceration and joint contractures. The presence of diabetes in our patient may be an association with gout. Since patient's renal parameter reverted to complete normal after hemodialysis and it was also normal during follow-up. So ARF in this patient can be attributed to the disease, which was very unusual.
| Conclusion|| |
Multiple gouty tophi can be present all over the body; it's being painless is less common. Gout should be considered as one of the important differential diagnosis while evaluating periarticular swelling. Early recognition of the disease and prompt treatment of the disease will prevent the long-term complications like joint contractures and ARF.
| Acknoweldgments|| |
We would like to thank Dr. Gadwalkar Srikant R, professor and HOD, Dr. Ravi SL, professor department of medicine for his support. The authors would like to thank Dr. Shanti, professor of pathology and Dr. Chandrashekar, assistant professor, Department of Pathology, vims-Bellary, for his assistance in histopathological reporting and selecting appropriate images for the article.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]