Discussion of case
The female patient 38 years of age had alopecia areata 6 months before the development of synovitis and photosensitivity. On evaluation, the development of sensory symptoms was found to be due to mononeuritis multiplex. The patient was positive for ANA, anti-RNP, Sm, SSA a, and Ro52. Her urine routine was normal. Based on the clinical presentation, the patient can be classified as a definite case of SLE. The disease progression predominantly involves the skin and musculoskeletal/peripheral nervous system in the form of vasculitis. There was no renal/CNS involvement. Infections are other diagnostic possibilities that could be considered.
The patient satisfies the EULAR criteria for diagnosing SLE.1 The EULAR/ACR Classification Criteria for Systemic Lupus Erythematosus are given below in table 1.
Table 1. EULAR Classification Criteria for Systemic Lupus Erythematosus1
Note -§ = additional criteria within the same domain will not be counted; * = in an assay with 90% specificity against relevant disease controls. Anti-β2GPI = anti–β2-glycoprotein I; anti-dsDNA = anti-double-stranded DNA
The patient had photosensitivity, polyarthritis (6), fever (2), non-scaring Alopecia (2) and positive serological test, and low complement (4) which suggest SLE as per the criteria. The other possibility to exclude is vasculitis.
In the present case, it is more important to focus on the customization of the treatment protocol to manage the specific clinical scenario. The patient's primary clinical features (musculoskeletal symptoms and antibody profile) are more suggestive of benign SLE. However, given the development of vasculitis presenting as mono neuritis multiplex, it is necessary to consider associated APLA syndrome as an alternative possibility. But the investigation for APLA was negative. Low C3 levels indicate that the disease process is more of immune complex vasculitis.
Such patients need to be managed initially with prednisolone 1 mg/kg body weight, along with HCQ and a steroid-sparing immunosuppressive agent. In the absence of renal and CNS involvement, the treatment should be initiated with HCQ and azathioprine. HCQ of 25 mg per day should be escalated to 100 mg per day in divided doses. The steroid should be gradually tapered.
Final Diagnosis
SLE with mono neuritis multiplex
Follow-up
Significant improvement in muscle weakness was noted and the patient was maintained on 100 mg of azathioprine and HCQ.
Learning points
- The treatment protocol in SLE is an organ- dependant
- The initial presentation may be benign, but the involvement of critical organs should raise suspicion of SLE.
Reference
- Aringer M, Costenbader KH, Daikh DI, et al. 2019 EULAR/ACR Classification Criteria for Systemic Lupus Erythematosus. Arthritis Rheumatol. 2019;71(9):1400-1412. doi:10.1002/art.40930.
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