The First Case of Rowell Syndrome with Lupus Nephritis and Lobar Pneumonia in a Male Child Reported in Pakistan

Abstract

Rowell syndrome (RS) is a rare entity that presents with lupus erythematosus (LE), erythema multiforme (EM) like lesions and characteristic immunological findings including positive rheumatoid factor (RF), speckled antinuclear antibody (ANA) and positive anti-Ro or anti-La antibodies in the serum. Only a few cases have been reported in the literature, mostly in middle-aged women. Our case is about an 11-year-old male child who had a history of pustular rashes over the whole body with scattered targetoid lesions for the past few months and later developed respiratory distress and swelling of the body. He was extensively investigated and confirmed on specific positive immunochemistry markers as RS along with lobar pneumonia (LP) and lupus nephritis. The co-existence of lobar pneumonia in our patient was a unique feature that has not been previously reported.

Systemic lupus erythematosus associated with erythema multiforme: A rare case report of Rowell's syndrome

“…3 The majority of the reported cases showed a satisfactory response to corticosteroids with azathioprine, antimalarial drugs such as chloroquine or hydroxychloroquine, dapsone, or cyclosporine. 1,11,13 Studies have shown that Belimumab, a human monoclonal antibody directed against the B lymphocyte stimulator, is often considered effective in recalcitrant cases of SLE, although data regarding its use in cutaneous SLE is limited. 1 The response to treatment can be assessed using the levels of certain au-dsDNA, anti-Nucl, anti-His, and anti-C1q), among which anti-dsDNA performs the best when assessing disease activity, as suggested by the researchers Shang et al 3 Our patient was subsequently managed with hydroxychloroquine (200 mg once daily), prednisolone (1 mg/kg/day), steroid ointment, a proton pump inhibitor, and sunscreen cream after the diagnosis.…”

“…However, the diagnosis becomes challenging when DLE coexists with SLE 3 . The majority of the reported cases showed a satisfactory response to corticosteroids with azathioprine, antimalarial drugs such as chloroquine or hydroxychloroquine, dapsone, or cyclosporine 1,11,13 . Studies have shown that Belimumab, a human monoclonal antibody directed against the B lymphocyte stimulator, is often considered effective in recalcitrant cases of SLE, although data regarding its use in cutaneous SLE is limited 1 .…”

“…12 RS is an uncommon presentation of lupus erythematosus with erythema multiforme-like lesions associated with specific serological changes, including positive rheumatoid factor (RF), speckled ANA, positive rheumatoid factor, or anti-La antibodies in the serum. 8,12,13 The speckled pattern of ANA is the most consistent diagnostic feature of Rowell's syndrome. Anti-La antibodies and rheumatoid factor seem to be less consistent features.…”

Rowell syndrome complicated with macrophage activation syndrome in a child

“…5 Interestingly, many patients reported in the literature are from India. 5–15 Unlike adults, pediatric patients with RS had a higher frequency of SLE, which may suggest that RS is part of a more serious disease in this population. 5–15…”

“…MAS is increasingly recognized as a feared complication of autoimmune diseases; 7 this has led to the adaptation of the diagnostic criteria for hemophagocytic syndrome published by the Histiocyte Society in 2004, seeking to achieve an earlier diagnosis of MAS in SLE, systemic juvenile idiopathic arthritis, Kawasaki disease, and lymphoma. 8,16–18 A validated score has been developed to diagnose reactive HLH termed HScore. 19 Parodi et al.…”