Autoimmune rheumatic diseases (AIRDs) are known for their systemic presentations and multi-organ involvement. Numerous infectious diseases, particularly mycobacterial, fungal and indolent bacterial infections endemic to specific geographic regions, present with varied signs and symptoms of multi-system involvement and can mimic AIRDs. Thus, differentiating infection from an AIRD is critical to resolve competing treatment approaches. This case report demonstrates the need for clinicians to actively look for, and rule out, infections in patients with systemic manifestations and/or suspected AIRD.
Case Presentation
A 23-year-old man presented to our clinic in India with a three-year history of numbness and paresthesia in both feet and his left forearm without any motor weakness; a 1.5-year history of intermittent, moderate-grade fever (101°F) associated with chills; symmetrical, additive, inflammatory polyarthritis involving both the large and small joints of his arms and legs; early morning stiffness of around 30 minutes’ duration; and a six-month history of recurrent episodes of reddish painful swelling of his ears, nose and periorbital region (see Figure 1).
On an outpatient basis, he was initially diagnosed with relapsing polychondritis on the basis of recurrent episodes of auricular chondritis; nasal chondritis; seronegative, non-erosive, inflammatory polyarthritis; and peripheral neuropathy, with a nerve conduction study suggestive of bilateral tibial nerve axonopathy. No erosions were detectable on hand X-rays; power Doppler ultrasound of his hands and wrists revealed synovial proliferation and vascularity, but no erosions.
The patient was prescribed low-dose glucocorticoids and methotrexate, to which he had partial response. Eventually, his symptoms worsened and fevers became more frequent. He was admitted for evaluation.
On admission, the patient reported a history of painful lumps in his left inguinal region; weight loss of around 8 kg in a year-and-a-half, with a loss of appetite; episodic, painful erythematous nodular lesions over his legs; and episodic redness and pain in his right scrotum.
Examination revealed tender nerve thickening involving the ulnar, common peroneal and right greater auricular nerves on both sides (see Figure 2), with loss of pain, touch and temperature sensations in the distribution of ulnar and common peroneal nerves on both sides, and an absent left supinator jerk, right knee jerk and left ankle jerk. Joint position sense was preserved.
He also had multiple hypopigmented, normoaesthetic macules over his neck, trunk and both upper arms (see Figures 3 & 4); tender inguinal lymphadenopathy (2 cm, firm, mobile); erythematous tender nodules over his left shin and left antecubital fossa suggestive of erythema nodosum; and a tender joint count of 36/68 and a swollen joint count of 4/66 in both his knees and ankles.