Discussion of the case
The 20-year-old female was having polyarthritis for the past 7 years and recently she developed deformities involving small joints of the foot and hands. The patient also reported the occurrence of persisting backache, low-grade fever, an increase in pain intensity, and weight loss from the past 6 months. Her previous history revealed the incidence of tubercular psoas abscess around 8 years before the current visit. Steroid and anti-cardiac failure drugs were used for managing congestive cardiac failure and pancytopenia occurred around 6 months back. Schober's test reported was 3 cm stretch indicating restricted mobility of the spine. The previous investigation revealed that the patient had anemia, pancytopenia, and normal LFT. The urine report showed the presence of 2+ protein, RBCs, and pus cells. The patient's investigation demonstrated positive ANA, RNP/smith nuclear ribosomal small P protein, and SSA antibodies.
The differential diagnosis to be considered in the present case would be connective tissue diseases especially systemic lupus erythematosus, aplastic anemia with ANA positivity, chronic renal failure with secondary bone marrow failure, and viral infections like HIV and hepatitis C infection. The patients fulfilled the newer SLE criteria. However, based on the previous criteria, the patient can be classified as having incomplete lupus concerning chronic renal failure, creatinine was within normal limits, and she had positive ANA and profiles file. Based on the urinary test results, lupus nephritis can be considered the possible cause of renal disease.
The presence of moderate pancytopenia indicates possible aplastic anemia along with positive ANA. Further guidelines on work-up for aplastic anemia have been provided by Marsh et al. There are reported cases of SLE presenting with aplastic anemia. In the current case, the occurrence of chronic renal failure and secondary bone marrow failure, and a positive ANA profile suggest tests that lupus is the possible cause of renal disease. However, the investigation reveals that the level of creatinine was normal before the patient's admission.
Chronic viral infections especially HIV, and hepatitis C and B can mimic these clinical presentations. Careful exclusion of diagnosis is required. The recent SLICC classification criteria have improved the sensitivity of SLE diagnosis (Table 01).
Table 1. EULAR/ACR Classification Criteria for Systemic Lupus Erythematosus
As per the criteria 2019, the present case can be classified as definite SLE, since the patient is fulfilling 4 clinical criteria and 2 laboratory or immunological criteria. They are the presence of leukopenia, thrombocytopenia, polyarthritis, and proteinuria; immunology tests: ANA positivity and anti-Sm positivity. But the patient does not fit into this classification according to the previous criteria (1982). In the 1982 criteria, pancytopenia was represented as a single criterion, whereas recent 2019 criteria segregate the same into three separate criteria. A few odd issues in the patient had deforming arthritis. Elevated CRP (21.3) and normal levels of complements were noted. RF was negative and the X-ray of the hand was normal. Based on these analyses, the diagnosis should be considered as SLE with probable lupus nephritis.
The probable management includes the assessment of renal involvement. The presence of proteinuria and active sediments suggest renal involvement. Conducting renal biopsy can be deferred because of low platelet kidney count.
The treatment decision depends on the renal involvement. The disease should be managed as lupus nephritis based on further analyses.
References
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2. 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.
3. Marsh JCW, Ball SE, Darbyshire P, Gordon-Smith EC, Keidan AJ, Martin A, et al. Guidelines for the diagnosis and management of acquired aplastic anemia. Br J Haematol. 2003 Dec;123(5):782–801.
4. Tan EM, Cohen AS, Fries JF, Masi AT, McShane DJ, Rothfield NF, et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1982 Nov;25(11):1271–7.
