Abhra Chandra Chowdhury

IL-17 and IFN-γ producing NK and γδ-T cells are preferentially expanded in synovial fluid of patients with reactive arthritis and undifferentiated spondyloarthritis

The IL-17/1L-23 axis is important in the pathogenesis of spondyloarthropathy. Innate cells produce IL-17 in addition to Th17 cells. We studied the frequencies of natural killer (NK) (total, CD56bright, CD56dim, perforin+ and granzyme+), NK-T, γδ-T, and IFN-γ+, IL-17+ NK and γδ-T cells in peripheral blood (PB) and synovial fluid (SF) of ReA/uSpA patients. PB from 45 patients and paired SF from 39 patients were studied, together with PB from 18 healthy controls (HC). The frequency of γδ-T cells was decreased (p<0.05) while IL-17 producing NK and γδ-T cells were increased (p<0.05) in PB of patients as compared to HC. In SF, CD56bright NK cells were increased (p<0.001) but had reduced expression of perforin and granzyme (p<0.0001) as compared to PB. Frequency of IL-17+, IFN-γ+ NK and γδ-T cells was higher in SF as compared to PB (p<0.05). We suggest that innate cells by producing pro-inflammatory cytokines may contribute to pathogenesis.

Methotrexate-induced pancytopenia: a case series of 46 patients

Methotrexate (MTX) has the potential to cause serious adverse reactions and even mortality. We analyzed the predisposing factors and outcome in patients with MTX‐induced pancytopenia admitted into our unit from 1996 to 2015.

Toxic epidermal necrolysis due to therapy with cyclophosphamide and mesna. A case report of a patient with seronegative rheumatoid arthritis and rheumatoid vasculitis.

Rheumatoid vasculitis usually occurs on the background of seropositive rheumatoid arthritis, although in rare cases the patients can be seronegative. We report a woman with seronegative rheumatoid arthritis with rheumatoid vasculitis who developed toxic epidermal necrolysis involving most of her body surface area, while on therapy with intravenous cyclophosphamide and mesna. After withdrawal of suspected offending agents, administration of intravenous immunoglobulin, and supportive therapy, she had a favorable outcome. Such an occurrence is rare and serves to educate about a potentially life-threatening adverse event associated with a commonly used immunosuppressive agent.ZusammenfassungEine rheumatoide Vaskulitis tritt meist vor dem Hintergrund einer seropositiven rheumatoiden Arthritis auf, selten auch bei Seronegativität. Berichtet wird über eine Patientin mit seronegativer rheumatoider Arthritis, die unter Therapie mit i.v. Cyclophosphamid und Mesna eine toxische epidermale Nekrolyse im Bereich von nahezu der gesamten Körperoberfläche entwickelte. Nach Sistieren der im Verdacht stehenden Substanzen, i.v. Gabe von Immunglobulinen und Implementierung einer supportiven Therapie kam es zu einem günstigen Verlauf und Outcome. Die Schilderung dieses selten vorkommenden Ereignisses soll für eine potenziell lebensbedrohliche Nebenwirkung in Verbindung mit einem häufig eingesetzten Immunsuppressivum sensibilisieren.

Toxic epidermal necrolysis due to therapy with cyclophosphamide and mesna

Rheumatoid vasculitis usually occurs on the background of seropositive rheumatoid arthritis, although in rare cases the patients can be seronegative. We report a woman with seronegative rheumatoid arthritis with rheumatoid vasculitis who developed toxic epidermal necrolysis involving most of her body surface area, while on therapy with intravenous cyclophosphamide and mesna. After withdrawal of suspected offending agents, administration of intravenous immunoglobulin, and supportive therapy, she had a favorable outcome. Such an occurrence is rare and serves to educate about a potentially life-threatening adverse event associated with a commonly used immunosuppressive agent.ZusammenfassungEine rheumatoide Vaskulitis tritt meist vor dem Hintergrund einer seropositiven rheumatoiden Arthritis auf, selten auch bei Seronegativität. Berichtet wird über eine Patientin mit seronegativer rheumatoider Arthritis, die unter Therapie mit i.v. Cyclophosphamid und Mesna eine toxische epidermale Nekrolyse im Bereich von nahezu der gesamten Körperoberfläche entwickelte. Nach Sistieren der im Verdacht stehenden Substanzen, i.v. Gabe von Immunglobulinen und Implementierung einer supportiven Therapie kam es zu einem günstigen Verlauf und Outcome. Die Schilderung dieses selten vorkommenden Ereignisses soll für eine potenziell lebensbedrohliche Nebenwirkung in Verbindung mit einem häufig eingesetzten Immunsuppressivum sensibilisieren.

Pulmonary co-infection with Nocardia and Aspergillus in a patient with adult-onset Still's disease receiving steroids and tacrolimus

Patients on immunosuppression are at risk of unusual infections. We present a man diagnosed to have adult-onset Stills disease who, on high-dose oral steroid and tacrolimus, developed a cavitating pneumonia due to co-infection with Aspergillus flavus and Nocardia. Timely diagnosis and institution of appropriate therapy resulted in a favourable clinical outcome. Such co-infection in a patient receiving tacrolimus is rare in the published literature. This case serves to emphasise the need to be vigilant for unusual infections in patients who are immunosuppressed, either due to drugs or underlying disease condition.

Lepra Reaction with Lucio Phenomenon Mimicking Cutaneous Vasculitis

Leprosy is a disease typically found in the tropics. Patients with leprosy can have varying presentation with constitutional symptoms, joint pains, skin nodules, and rarely a vasculitis-like picture with skin ulcers and neuropathy. We present a young lady who presented with the rare manifestation of skin infarcts mimicking cutaneous vasculitis, diagnosed on histopathology to have Lucio phenomenon on a background of lepromatous leprosy. With increasing migration and widespread use of biologic response modifiers, clinicians all over the world need to be aware of various presentations of leprosy as well as needing to keep an open mind while considering the differential diagnoses of vasculitis.

Differences between adult and pediatric onset Henoch‐Schonlein purpura from North India

Henoch‐Schönlein purpura (HSP), a primary vasculitis, characterized by purpura, abdominal pain, arthritis and renal involvement, is predominantly a disease of childhood. However, rarely it can occur in adults in whom it is believed to be a more severe form with poor renal outcomes. We aimed to answer if the age of onset affected the clinical spectrum and renal outcomes of the disease in a north Indian population. Hence, we studied the differences in clinical spectrum and renal outcomes between adult‐onset HSP and childhood‐onset HSP.

Behcet’s disease without oral ulcers presenting with erythema nodosum and deep venous thrombosis

We present a young male with recurrent erythema nodosum and recent deep vein thrombosis with scrotal ulcers but no oral ulcers. He was diagnosed as having Behcets disease (BD) and subsequently responded to immunosuppressants and anticoagulation. This case highlights that up to 2% patients with BD may not have oral ulcers. Timely institution of therapy in our patient resulted in a favorable outcome.

Refractory polymyositis undergoing remission following antitubercular therapy

Opportunistic infections pose a significant problem in patients receiving immunosuppressive therapy for autoimmune diseases. We present a case of a woman with polymyositis refractory to high-dose steroid and methotrexate, as a consequence of which she developed miliary tuberculosis. Her myositis went into remission after initiation of antitubercular therapy, in spite of bringing down the intensity of immunosuppression. This is the first reported case of myositis undergoing remission after treating intercurrent infection, and illustrates the complex relationship between autoimmune disease and host response to infection.

Multiple myeloma masquerading as severe seropositive rheumatoid arthritis with subcutaneous nodules and mononeuritis multiplex

Multiple myeloma can rarely mimic seronegative rheumatoid arthritis (RA). We report a 55‐year‐old woman who presented with longstanding deforming polyarthritis with extensive subcutaneous nodules, tenosynovitis, anti‐cyclic citrullinated peptide positivity and mononeuritis multiplex. Even though the clinical picture was consistent with seropositive RA, the absence of bone erosion or joint space narrowing on hand and knee radiographs led us to question the diagnosis of RA. Further investigation revealed a diagnosis of multiple myeloma with cutaneous amyloid deposits, based on serum immunofixation, bone marrow aspiration and biopsy of a subcutaneous nodule. The only clue to suspect myeloma from the basic investigations and clinical examination was mild hypercalcemia. This case serves to reiterate the need to maintain a heightened suspicion for other diagnoses even when RA appears most likely.