A combined Clinico Radiological case from Dr Joe Thomas (Rheumatologist)
This is a combined case with Dr Joe Thomas on a case of Polyarteritis Nodosa that presented with Myositis and Myopericarditis. Dr Thomas is a senior consultant Rheumatologist who is also joining us in presenting the Spine Arthropathy and Spondyloarthropathy course.
- 20-year-old student presented with polyarthritis.
 - ESR 38, CRP 11, ANA(IF) speckled 2 +, ANA profile showed ds DNA 2 + and SCL 70 1+, dsDNA positive by elisa (461).
 - Diagnosed as SLE and was on treatment with HCQS, steroids, methotrexate.
 - Persistent joint pain with fever and rising inflammatory markers (ESR 90, CRP121) despite treatment.
 - PET CT : Inflammatory arthritis with enthesitis.
 - Managed with pulse steroid and rituximab 500mg.
- Readmitted in view of persistent fever and generalized myalgia in functional class 4.
 - Inflammatory markers- ESR 120, CRP 201.
 - Work up for infection and malignancy were negative (including bone marrow).
 - Steroids and IVIG were started.
 - Developed left lower limb numbness with gait abnormality and severe retrosternal chest pain.
- Admitted in ICUÂ in view of severe chest pain (requiring opioids to control pain).
 - Very high inflammatory markers, Trop I- 740.0 ng/L (N<9) and repeat work up for Lupus was negative.
 - ECHOCARDIOGRAM: Global LV hypokinesia with severe left ventricular systolic dysfunction.
 - Diagnosis at this stage was severe myopericarditis secondary to autoimmune disease.
- USG guided Muscle biopsy done suggestive of inflammatory myositis.
 - Surprisingly CPK and other work up for myositis was negative.
 - There was worsening mononeuritis multiplex.
 - In spite of repeat pulse steroids, her chest pain, cardiac function and cardiac enzymes worsened.
Polyarteritis Nodosa With Myopericarditis.
- In this case clinical phenotype was that of PAN rather than lupus inspite of initial autoantibody profile, (main clue was >CRP 100).
 - In a patient with myalgia/no muscle weakness and MRI suggestive of myositis, with normal CPK, think of PAN.
 - Work up for DADA2 and another genetic syndrome were negative. The patient stabilized with combination of medications (Steroids + IVIG + Tofacitnib + Methotrexate).
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