BERLIN – A delay in undertaking plasma exchange may predict a poorer outcome after a first attack of neuromyelitis optica spectrum disorder, while antibodies to myelin oligodendrocyte glycoprotein (MOG) appear to predict a more positive outcome.

Michele G. Sullivan/MDedge News

“We saw that for each day of delay in plasma exchange, the Expanded Disability Status Scale [EDSS] at 6 months increased by about 0.028 points, indicating a worse prognosis,” Maxime Guillaume, MD, said at the annual congress of the European Committee for Treatment and Research in Multiples Sclerosis.

However, said Dr. Guillaume, a resident at Rouen University Hospital, France, steroids are still a reasonable first-line therapy as long as they are discontinued quickly if they don’t appear to be helping. Plasma exchange is most effective if administered less than 2 weeks after symptom onset.

His study examined 6-month outcomes among 214 attacks in 188 patients; some patients had several first attacks in different areas. Response was defined in two ways. First, patients were clinically classified as having a good response, a bad response, or no response to treatment. The second definition was based on the EDSS. Good response was an EDSS decrease of at least 2 points for an initial score of 3 or higher, or a decrease of 1 point if the initial score was less than 3. Poor response was an EDSS that decreased without reaching these thresholds.

The cohort was largely female, with a mean age of 38 years. Most (55%) were positive for antibodies against aquaporin-4. Anti-MOG antibodies were present in 30%. A total of 7.5% were negative for both antibodies, and the remainder had an undetermined serotype.

The clinical presentations varied. Most frequently, patients presented with myelitis only (44%). This was followed by optic neuritis only (34%), both myelitis and optic neuritis (8%), and myelitis plus brainstem involvement (5%). Other clinical manifestations were acute demyelinating encephalomyelitis, and encephalitis alone.

The most common treatment was methylprednisolone (73%), followed by plasma exchange (25%), which occurred at a median of 9 days after symptom onset.

Outcomes varied according to the definition of response. By clinical characteristics, there was a complete response in 41, a partial response in 122, and no response in 51. By change in EDSS, 136 had a good response and 27 a partial response; 51 were still considered nonresponders.

Dr. Guillaume conducted a multivariate analysis to determine predictive factors. In both definitions, anti-MOG antibodies nearly quadrupled the chance of a good treatment response, and delaying plasma exchange was associated with a significantly increased chance of a poor response. When judged by the clinical response definition, multiple lines of treatment also were associated with a poor response. This, he said, was another reflection of plasma exchange delay.

Dr. Guillaume had no financial disclosures.

[email protected]

SOURCE: Guillaume M. et al. ECTRIMS 2018, Abstract 211.

BERLIN – A delay in undertaking plasma exchange may predict a poorer outcome after a first attack of neuromyelitis optica spectrum disorder, while antibodies to myelin oligodendrocyte glycoprotein (MOG) appear to predict a more positive outcome.

Michele G. Sullivan/MDedge News

“We saw that for each day of delay in plasma exchange, the Expanded Disability Status Scale [EDSS] at 6 months increased by about 0.028 points, indicating a worse prognosis,” Maxime Guillaume, MD, said at the annual congress of the European Committee for Treatment and Research in Multiples Sclerosis.

However, said Dr. Guillaume, a resident at Rouen University Hospital, France, steroids are still a reasonable first-line therapy as long as they are discontinued quickly if they don’t appear to be helping. Plasma exchange is most effective if administered less than 2 weeks after symptom onset.

His study examined 6-month outcomes among 214 attacks in 188 patients; some patients had several first attacks in different areas. Response was defined in two ways. First, patients were clinically classified as having a good response, a bad response, or no response to treatment. The second definition was based on the EDSS. Good response was an EDSS decrease of at least 2 points for an initial score of 3 or higher, or a decrease of 1 point if the initial score was less than 3. Poor response was an EDSS that decreased without reaching these thresholds.

The cohort was largely female, with a mean age of 38 years. Most (55%) were positive for antibodies against aquaporin-4. Anti-MOG antibodies were present in 30%. A total of 7.5% were negative for both antibodies, and the remainder had an undetermined serotype.

The clinical presentations varied. Most frequently, patients presented with myelitis only (44%). This was followed by optic neuritis only (34%), both myelitis and optic neuritis (8%), and myelitis plus brainstem involvement (5%). Other clinical manifestations were acute demyelinating encephalomyelitis, and encephalitis alone.

The most common treatment was methylprednisolone (73%), followed by plasma exchange (25%), which occurred at a median of 9 days after symptom onset.

Outcomes varied according to the definition of response. By clinical characteristics, there was a complete response in 41, a partial response in 122, and no response in 51. By change in EDSS, 136 had a good response and 27 a partial response; 51 were still considered nonresponders.

Dr. Guillaume conducted a multivariate analysis to determine predictive factors. In both definitions, anti-MOG antibodies nearly quadrupled the chance of a good treatment response, and delaying plasma exchange was associated with a significantly increased chance of a poor response. When judged by the clinical response definition, multiple lines of treatment also were associated with a poor response. This, he said, was another reflection of plasma exchange delay.

Dr. Guillaume had no financial disclosures.

[email protected]

SOURCE: Guillaume M. et al. ECTRIMS 2018, Abstract 211.

BERLIN – A delay in undertaking plasma exchange may predict a poorer outcome after a first attack of neuromyelitis optica spectrum disorder, while antibodies to myelin oligodendrocyte glycoprotein (MOG) appear to predict a more positive outcome.

Michele G. Sullivan/MDedge News

“We saw that for each day of delay in plasma exchange, the Expanded Disability Status Scale [EDSS] at 6 months increased by about 0.028 points, indicating a worse prognosis,” Maxime Guillaume, MD, said at the annual congress of the European Committee for Treatment and Research in Multiples Sclerosis.

However, said Dr. Guillaume, a resident at Rouen University Hospital, France, steroids are still a reasonable first-line therapy as long as they are discontinued quickly if they don’t appear to be helping. Plasma exchange is most effective if administered less than 2 weeks after symptom onset.

His study examined 6-month outcomes among 214 attacks in 188 patients; some patients had several first attacks in different areas. Response was defined in two ways. First, patients were clinically classified as having a good response, a bad response, or no response to treatment. The second definition was based on the EDSS. Good response was an EDSS decrease of at least 2 points for an initial score of 3 or higher, or a decrease of 1 point if the initial score was less than 3. Poor response was an EDSS that decreased without reaching these thresholds.

The cohort was largely female, with a mean age of 38 years. Most (55%) were positive for antibodies against aquaporin-4. Anti-MOG antibodies were present in 30%. A total of 7.5% were negative for both antibodies, and the remainder had an undetermined serotype.

The clinical presentations varied. Most frequently, patients presented with myelitis only (44%). This was followed by optic neuritis only (34%), both myelitis and optic neuritis (8%), and myelitis plus brainstem involvement (5%). Other clinical manifestations were acute demyelinating encephalomyelitis, and encephalitis alone.

The most common treatment was methylprednisolone (73%), followed by plasma exchange (25%), which occurred at a median of 9 days after symptom onset.

Outcomes varied according to the definition of response. By clinical characteristics, there was a complete response in 41, a partial response in 122, and no response in 51. By change in EDSS, 136 had a good response and 27 a partial response; 51 were still considered nonresponders.

Dr. Guillaume conducted a multivariate analysis to determine predictive factors. In both definitions, anti-MOG antibodies nearly quadrupled the chance of a good treatment response, and delaying plasma exchange was associated with a significantly increased chance of a poor response. When judged by the clinical response definition, multiple lines of treatment also were associated with a poor response. This, he said, was another reflection of plasma exchange delay.

Dr. Guillaume had no financial disclosures.

[email protected]

SOURCE: Guillaume M. et al. ECTRIMS 2018, Abstract 211.

The Diagnosis: Self-healing Langerhans Cell Histiocytosis

Histopathologic examination showed an infiltrate of mononuclear cells with indented nuclei admixed with a variable dermal inflammatory infiltrate. Immunohistochemistry demonstrated cells that were strongly positive for CD1a (Figure, A) and langerin (Figure, B) antigens as well as S-100 protein (Figure, C), which was consistent with Langerhans cell histiocytosis (LCH).

Histiocytoses are a heterogeneous group of disorders in which the infiltrating cells belong to the mononuclear phagocyte system.^1,2 Langerhans cell histiocytosis is the most common dendritic cell-related histiocytosis, occurring in approximately 5 per 1 million children annually, giving it an incidence comparable to pediatric Hodgkin lymphoma and acute myeloid leukemia.^1,2

Historically, there has been much debate about the pathogenesis of the disease.² Until recently it was unknown whether LCH was primarily a neoplastic or an inflammatory disorder. Although the condition initially was thought to have a reactive etiology,¹ more recent evidence suggests a clonal neoplastic process. Langerhans cell histiocytosis lesions are clonal and display malignancy-associated mechanisms such as immune evasion. Genome sequencing has revealed several mutations in precursor myeloid cells that result in the common downstream hyperactivation of the mitogen-activated protein kinase signaling pathway that regulates cell proliferation and differentiation.¹

Langerhans cell histiocytosis displays a wide spectrum of clinical phenotypes, which historically were subclassified as eosinophilic granulomas (localized lesions in bone), Hand-Schüller-Christian disease (multiple organ involvement with the classic triad of skull defects, diabetes insipidus, and exophthalmos), and Letterer-Siwe disease (visceral lesions involving multiple organs).³ However, in 1997 the Reclassification Working Group of the Histiocyte Society redefined LCH as single-system single site (SS-s) LCH, single-system multisite LCH, and multisystem LCH.⁴

In SS-s LCH, the most common site is bone (82%), followed by the skin (12%).⁵ Skin SS-s LCH classically presents as multiple skin lesions at birth without systemic manifestations; the lesions spontaneously involute within a few months.⁶ Less commonly, skin SS-s LCH can present as a single lesion. Berger et al⁷ described 4 neonates with unilesional skin SS-s LCH. Since then, more than 30 cases have been reported in the literature,⁸ and we report herein another unilesional self-healing LCH.

The morphology of skin lesions in self-healing LCH is highly variable, with the most common being multiple erythematous crusted papules (50%), followed by eczematous scaly lesions resembling seborrheic dermatitis in intertriginous areas (37.5%).^3,6 Unilesional self-healing LCH typically presents as an ulcerated or crusted nodule or papule on the trunk. This variability results in a large differential diagnosis. Self-healing LCH is easily mistaken for infectious processes including neonatal herpes simplex and varicella-zoster virus infection.⁹ Often, the dermatology department is consulted to rule out LCH when the asymptomatic neonate does not respond to parenteral acyclovir.

Less commonly, the magenta-colored papulonodules of self-healing LCH can mimic blueberry muffin rash and mandate a workup for intrauterine infections, especially cytomegalovirus, rubella, and blood dyscrasia.¹⁰ Other noninfectious processes in the differential of self-healing LCH include congenital infantile hemangioma, neonatal lupus erythematosus, seborrheic dermatitis (cradle cap), pyogenic granuloma, and psoriasis.^3,10 Definitive diagnosis requires histopathology.

Because unilesional self-healing LCH has an excellent prognosis and usually resolves on its own, therapy is unnecessary.^3,8 One large retrospective study (N=146) found that of all patients with skin lesions, 56% were managed with biopsy only.⁵ Other options include watchful waiting and topical corticosteroids. If the skin lesions are large, ulcerated, and/or painful, alkylating antitumor agents have been used. For extensive cutaneous disease, systemic corticosteroids combined with chemotherapy and psoralen plus UVA can be effective.⁶

The primary concern in the management of self-healing LCH is that the solitary skin lesion may be the harbinger of an aggressive disorder that can progress to systemic disease.⁵ Moreover, recurrent visceral or disseminated disease may occur months to years after resolution of solitary skin lesions.⁹ Studies have shown that localized and disseminated disease cannot be differentiated on the basis of clinical findings, histology, immunohistochemistry, or biomarkers.^3,11 As a result, an evaluation for systemic disease should be performed at the time of diagnosis for cutaneous LCH.^3,9 Minimum baseline studies recommended by the Writing Group of the Histiocyte Society include a complete blood cell count, liver function tests, coagulation studies, chest radiography, skeletal surveys, and urine osmolality testing.¹² Periodic clinical follow-up is recommended for all variants of LCH.⁹

Our case was diagnosed as self-healing LCH based on histologic findings. No treatment was required, and at 3-month follow-up the infant was asymptomatic without recurrence and was meeting all developmental milestones.

The Diagnosis: Self-healing Langerhans Cell Histiocytosis

Histopathologic examination showed an infiltrate of mononuclear cells with indented nuclei admixed with a variable dermal inflammatory infiltrate. Immunohistochemistry demonstrated cells that were strongly positive for CD1a (Figure, A) and langerin (Figure, B) antigens as well as S-100 protein (Figure, C), which was consistent with Langerhans cell histiocytosis (LCH).

Histiocytoses are a heterogeneous group of disorders in which the infiltrating cells belong to the mononuclear phagocyte system.^1,2 Langerhans cell histiocytosis is the most common dendritic cell-related histiocytosis, occurring in approximately 5 per 1 million children annually, giving it an incidence comparable to pediatric Hodgkin lymphoma and acute myeloid leukemia.^1,2

Historically, there has been much debate about the pathogenesis of the disease.² Until recently it was unknown whether LCH was primarily a neoplastic or an inflammatory disorder. Although the condition initially was thought to have a reactive etiology,¹ more recent evidence suggests a clonal neoplastic process. Langerhans cell histiocytosis lesions are clonal and display malignancy-associated mechanisms such as immune evasion. Genome sequencing has revealed several mutations in precursor myeloid cells that result in the common downstream hyperactivation of the mitogen-activated protein kinase signaling pathway that regulates cell proliferation and differentiation.¹

Langerhans cell histiocytosis displays a wide spectrum of clinical phenotypes, which historically were subclassified as eosinophilic granulomas (localized lesions in bone), Hand-Schüller-Christian disease (multiple organ involvement with the classic triad of skull defects, diabetes insipidus, and exophthalmos), and Letterer-Siwe disease (visceral lesions involving multiple organs).³ However, in 1997 the Reclassification Working Group of the Histiocyte Society redefined LCH as single-system single site (SS-s) LCH, single-system multisite LCH, and multisystem LCH.⁴

In SS-s LCH, the most common site is bone (82%), followed by the skin (12%).⁵ Skin SS-s LCH classically presents as multiple skin lesions at birth without systemic manifestations; the lesions spontaneously involute within a few months.⁶ Less commonly, skin SS-s LCH can present as a single lesion. Berger et al⁷ described 4 neonates with unilesional skin SS-s LCH. Since then, more than 30 cases have been reported in the literature,⁸ and we report herein another unilesional self-healing LCH.

The morphology of skin lesions in self-healing LCH is highly variable, with the most common being multiple erythematous crusted papules (50%), followed by eczematous scaly lesions resembling seborrheic dermatitis in intertriginous areas (37.5%).^3,6 Unilesional self-healing LCH typically presents as an ulcerated or crusted nodule or papule on the trunk. This variability results in a large differential diagnosis. Self-healing LCH is easily mistaken for infectious processes including neonatal herpes simplex and varicella-zoster virus infection.⁹ Often, the dermatology department is consulted to rule out LCH when the asymptomatic neonate does not respond to parenteral acyclovir.

Less commonly, the magenta-colored papulonodules of self-healing LCH can mimic blueberry muffin rash and mandate a workup for intrauterine infections, especially cytomegalovirus, rubella, and blood dyscrasia.¹⁰ Other noninfectious processes in the differential of self-healing LCH include congenital infantile hemangioma, neonatal lupus erythematosus, seborrheic dermatitis (cradle cap), pyogenic granuloma, and psoriasis.^3,10 Definitive diagnosis requires histopathology.

Because unilesional self-healing LCH has an excellent prognosis and usually resolves on its own, therapy is unnecessary.^3,8 One large retrospective study (N=146) found that of all patients with skin lesions, 56% were managed with biopsy only.⁵ Other options include watchful waiting and topical corticosteroids. If the skin lesions are large, ulcerated, and/or painful, alkylating antitumor agents have been used. For extensive cutaneous disease, systemic corticosteroids combined with chemotherapy and psoralen plus UVA can be effective.⁶

The primary concern in the management of self-healing LCH is that the solitary skin lesion may be the harbinger of an aggressive disorder that can progress to systemic disease.⁵ Moreover, recurrent visceral or disseminated disease may occur months to years after resolution of solitary skin lesions.⁹ Studies have shown that localized and disseminated disease cannot be differentiated on the basis of clinical findings, histology, immunohistochemistry, or biomarkers.^3,11 As a result, an evaluation for systemic disease should be performed at the time of diagnosis for cutaneous LCH.^3,9 Minimum baseline studies recommended by the Writing Group of the Histiocyte Society include a complete blood cell count, liver function tests, coagulation studies, chest radiography, skeletal surveys, and urine osmolality testing.¹² Periodic clinical follow-up is recommended for all variants of LCH.⁹

Our case was diagnosed as self-healing LCH based on histologic findings. No treatment was required, and at 3-month follow-up the infant was asymptomatic without recurrence and was meeting all developmental milestones.

The Diagnosis: Self-healing Langerhans Cell Histiocytosis

Histopathologic examination showed an infiltrate of mononuclear cells with indented nuclei admixed with a variable dermal inflammatory infiltrate. Immunohistochemistry demonstrated cells that were strongly positive for CD1a (Figure, A) and langerin (Figure, B) antigens as well as S-100 protein (Figure, C), which was consistent with Langerhans cell histiocytosis (LCH).

Histiocytoses are a heterogeneous group of disorders in which the infiltrating cells belong to the mononuclear phagocyte system.^1,2 Langerhans cell histiocytosis is the most common dendritic cell-related histiocytosis, occurring in approximately 5 per 1 million children annually, giving it an incidence comparable to pediatric Hodgkin lymphoma and acute myeloid leukemia.^1,2

Historically, there has been much debate about the pathogenesis of the disease.² Until recently it was unknown whether LCH was primarily a neoplastic or an inflammatory disorder. Although the condition initially was thought to have a reactive etiology,¹ more recent evidence suggests a clonal neoplastic process. Langerhans cell histiocytosis lesions are clonal and display malignancy-associated mechanisms such as immune evasion. Genome sequencing has revealed several mutations in precursor myeloid cells that result in the common downstream hyperactivation of the mitogen-activated protein kinase signaling pathway that regulates cell proliferation and differentiation.¹

Langerhans cell histiocytosis displays a wide spectrum of clinical phenotypes, which historically were subclassified as eosinophilic granulomas (localized lesions in bone), Hand-Schüller-Christian disease (multiple organ involvement with the classic triad of skull defects, diabetes insipidus, and exophthalmos), and Letterer-Siwe disease (visceral lesions involving multiple organs).³ However, in 1997 the Reclassification Working Group of the Histiocyte Society redefined LCH as single-system single site (SS-s) LCH, single-system multisite LCH, and multisystem LCH.⁴

In SS-s LCH, the most common site is bone (82%), followed by the skin (12%).⁵ Skin SS-s LCH classically presents as multiple skin lesions at birth without systemic manifestations; the lesions spontaneously involute within a few months.⁶ Less commonly, skin SS-s LCH can present as a single lesion. Berger et al⁷ described 4 neonates with unilesional skin SS-s LCH. Since then, more than 30 cases have been reported in the literature,⁸ and we report herein another unilesional self-healing LCH.

The morphology of skin lesions in self-healing LCH is highly variable, with the most common being multiple erythematous crusted papules (50%), followed by eczematous scaly lesions resembling seborrheic dermatitis in intertriginous areas (37.5%).^3,6 Unilesional self-healing LCH typically presents as an ulcerated or crusted nodule or papule on the trunk. This variability results in a large differential diagnosis. Self-healing LCH is easily mistaken for infectious processes including neonatal herpes simplex and varicella-zoster virus infection.⁹ Often, the dermatology department is consulted to rule out LCH when the asymptomatic neonate does not respond to parenteral acyclovir.

Less commonly, the magenta-colored papulonodules of self-healing LCH can mimic blueberry muffin rash and mandate a workup for intrauterine infections, especially cytomegalovirus, rubella, and blood dyscrasia.¹⁰ Other noninfectious processes in the differential of self-healing LCH include congenital infantile hemangioma, neonatal lupus erythematosus, seborrheic dermatitis (cradle cap), pyogenic granuloma, and psoriasis.^3,10 Definitive diagnosis requires histopathology.

Because unilesional self-healing LCH has an excellent prognosis and usually resolves on its own, therapy is unnecessary.^3,8 One large retrospective study (N=146) found that of all patients with skin lesions, 56% were managed with biopsy only.⁵ Other options include watchful waiting and topical corticosteroids. If the skin lesions are large, ulcerated, and/or painful, alkylating antitumor agents have been used. For extensive cutaneous disease, systemic corticosteroids combined with chemotherapy and psoralen plus UVA can be effective.⁶

The primary concern in the management of self-healing LCH is that the solitary skin lesion may be the harbinger of an aggressive disorder that can progress to systemic disease.⁵ Moreover, recurrent visceral or disseminated disease may occur months to years after resolution of solitary skin lesions.⁹ Studies have shown that localized and disseminated disease cannot be differentiated on the basis of clinical findings, histology, immunohistochemistry, or biomarkers.^3,11 As a result, an evaluation for systemic disease should be performed at the time of diagnosis for cutaneous LCH.^3,9 Minimum baseline studies recommended by the Writing Group of the Histiocyte Society include a complete blood cell count, liver function tests, coagulation studies, chest radiography, skeletal surveys, and urine osmolality testing.¹² Periodic clinical follow-up is recommended for all variants of LCH.⁹

Our case was diagnosed as self-healing LCH based on histologic findings. No treatment was required, and at 3-month follow-up the infant was asymptomatic without recurrence and was meeting all developmental milestones.

Symptoms of gastrointestinal involvement such as abdominal pain and vomiting may delay diagnosis of Kawasaki disease in pediatric patients.

NaiyanaDonraman/Thinkstock

“The clinical onset of Kawasaki disease with gastrointestinal involvement often leads to diagnostic and therapeutic delays – a risk factor for the development of coronary complications,” Claudia Colomba, MD, from the department of sciences for health promotion and mother and child care at the University of Palermo (Italy), and her colleagues wrote in the Journal of Pediatrics.

After caring for a boy aged 14 years at their center who presented with these symptoms, Dr. Colomba and her colleagues performed a search of the PubMed and Scopus databases and identified 33 articles with 48 total cases of Kawasaki disease with intestinal involvement between 1979 and 2017.

There were 40 cases of fever (82%), 34 cases of abdominal pain (69%), and 24 cases of vomiting (49%) at disease onset, with diarrhea occurring in 14 cases (29%) and jaundice in 1 case (2%), the researchers noted. Cardiac involvement occurred in 21 cases (43%). With regard to imaging, 38 cases of pseudo-obstruction (77%) were identified by plain radiograph, ultrasonography, and CT. Over half of the cases required surgery; of these 25 cases (51%), 8 cases involved a resection of the restricted loop and included a temporary colostomy (16%), 5 cases were exploratory laparotomy (10%), and there was 1 case with enterolysis (2%).

A total of 45 patients received medical treatment, with 12 patients (25%) receiving intravenous immunoglobulin and 18 (37%) receiving intravenous immunoglobulin plus aspirin. One patient had cyanosis and hand and foot gangrene. There were three patients who died, with massive liver necrosis identified during the autopsy of one patient. Of the other two who died, one did so 2 days after exploratory laparotomy and the other died because of Pseudomonas septic shock.

The researchers reported a good outcome in 28 patients (57%), which included 3 cases where there was no treatment.

“The diagnosis of Kawasaki disease should be considered in all children with fever, abdominal pain, and radiologic signs of pseudo-obstruction, even in the absence of typical symptoms and signs,” Dr. Colomba and her colleagues wrote. “A more comprehensive analysis including all clinical forms of Kawasaki disease would be useful to correlate intestinal involvement with worse outcomes for cardiac complications, as well as to clues to more rapid diagnosis and avoidance of unnecessary invasive procedures.”

The authors reported no relevant conflicts of interest.

SOURCE: Colomba C et al. J Pediatr. 2018 Jul 17. doi: 10.1016/j.jpeds.2018.06.034.

Symptoms of gastrointestinal involvement such as abdominal pain and vomiting may delay diagnosis of Kawasaki disease in pediatric patients.

NaiyanaDonraman/Thinkstock

“The clinical onset of Kawasaki disease with gastrointestinal involvement often leads to diagnostic and therapeutic delays – a risk factor for the development of coronary complications,” Claudia Colomba, MD, from the department of sciences for health promotion and mother and child care at the University of Palermo (Italy), and her colleagues wrote in the Journal of Pediatrics.

After caring for a boy aged 14 years at their center who presented with these symptoms, Dr. Colomba and her colleagues performed a search of the PubMed and Scopus databases and identified 33 articles with 48 total cases of Kawasaki disease with intestinal involvement between 1979 and 2017.

There were 40 cases of fever (82%), 34 cases of abdominal pain (69%), and 24 cases of vomiting (49%) at disease onset, with diarrhea occurring in 14 cases (29%) and jaundice in 1 case (2%), the researchers noted. Cardiac involvement occurred in 21 cases (43%). With regard to imaging, 38 cases of pseudo-obstruction (77%) were identified by plain radiograph, ultrasonography, and CT. Over half of the cases required surgery; of these 25 cases (51%), 8 cases involved a resection of the restricted loop and included a temporary colostomy (16%), 5 cases were exploratory laparotomy (10%), and there was 1 case with enterolysis (2%).

A total of 45 patients received medical treatment, with 12 patients (25%) receiving intravenous immunoglobulin and 18 (37%) receiving intravenous immunoglobulin plus aspirin. One patient had cyanosis and hand and foot gangrene. There were three patients who died, with massive liver necrosis identified during the autopsy of one patient. Of the other two who died, one did so 2 days after exploratory laparotomy and the other died because of Pseudomonas septic shock.

The researchers reported a good outcome in 28 patients (57%), which included 3 cases where there was no treatment.

“The diagnosis of Kawasaki disease should be considered in all children with fever, abdominal pain, and radiologic signs of pseudo-obstruction, even in the absence of typical symptoms and signs,” Dr. Colomba and her colleagues wrote. “A more comprehensive analysis including all clinical forms of Kawasaki disease would be useful to correlate intestinal involvement with worse outcomes for cardiac complications, as well as to clues to more rapid diagnosis and avoidance of unnecessary invasive procedures.”

The authors reported no relevant conflicts of interest.

SOURCE: Colomba C et al. J Pediatr. 2018 Jul 17. doi: 10.1016/j.jpeds.2018.06.034.

Symptoms of gastrointestinal involvement such as abdominal pain and vomiting may delay diagnosis of Kawasaki disease in pediatric patients.

NaiyanaDonraman/Thinkstock

“The clinical onset of Kawasaki disease with gastrointestinal involvement often leads to diagnostic and therapeutic delays – a risk factor for the development of coronary complications,” Claudia Colomba, MD, from the department of sciences for health promotion and mother and child care at the University of Palermo (Italy), and her colleagues wrote in the Journal of Pediatrics.

After caring for a boy aged 14 years at their center who presented with these symptoms, Dr. Colomba and her colleagues performed a search of the PubMed and Scopus databases and identified 33 articles with 48 total cases of Kawasaki disease with intestinal involvement between 1979 and 2017.

There were 40 cases of fever (82%), 34 cases of abdominal pain (69%), and 24 cases of vomiting (49%) at disease onset, with diarrhea occurring in 14 cases (29%) and jaundice in 1 case (2%), the researchers noted. Cardiac involvement occurred in 21 cases (43%). With regard to imaging, 38 cases of pseudo-obstruction (77%) were identified by plain radiograph, ultrasonography, and CT. Over half of the cases required surgery; of these 25 cases (51%), 8 cases involved a resection of the restricted loop and included a temporary colostomy (16%), 5 cases were exploratory laparotomy (10%), and there was 1 case with enterolysis (2%).

A total of 45 patients received medical treatment, with 12 patients (25%) receiving intravenous immunoglobulin and 18 (37%) receiving intravenous immunoglobulin plus aspirin. One patient had cyanosis and hand and foot gangrene. There were three patients who died, with massive liver necrosis identified during the autopsy of one patient. Of the other two who died, one did so 2 days after exploratory laparotomy and the other died because of Pseudomonas septic shock.

The researchers reported a good outcome in 28 patients (57%), which included 3 cases where there was no treatment.

“The diagnosis of Kawasaki disease should be considered in all children with fever, abdominal pain, and radiologic signs of pseudo-obstruction, even in the absence of typical symptoms and signs,” Dr. Colomba and her colleagues wrote. “A more comprehensive analysis including all clinical forms of Kawasaki disease would be useful to correlate intestinal involvement with worse outcomes for cardiac complications, as well as to clues to more rapid diagnosis and avoidance of unnecessary invasive procedures.”

The authors reported no relevant conflicts of interest.

SOURCE: Colomba C et al. J Pediatr. 2018 Jul 17. doi: 10.1016/j.jpeds.2018.06.034.